Primary Intraosseous Carcinoma in the Pediatric Mandible

Primary Intraosseous Carcinoma in the Pediatric Mandible | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case report Primary Intraosseous Carcinoma in the Pediatric Mandible Hyun Jun Oh, Dong Whan Shin, Hoon Myoung, Hye-Jung Yoon, Soung Min Kim This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-195662/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Background: Primary intraosseous carcinoma (PIOC) is a rare malignant odontogenic tumor that predominantly occurs in males older than 50 years. PIOC can be misdiagnosed as odontogenic cyst because it occasionally shows well-defined border on radiography. In this study, a case of a 14-year-old female with PIOC who was misdiagnosed with odontogenic cyst clinically is reported along with a literature review of pediatric PIOC cases. Case Presentation: A 14-year-old female patient presented with painful swelling on the mandibular right premolar area. There was a radiolucent lesion with a well-defined border in panoramic view. She was diagnosed with odontogenic cyst, and the cystic mass was enucleated with extraction. However, the biopsy result was consistent with PIOC. After cancer work-up, she underwent partial mandibulectomy, selective neck dissection, and reconstruction with a fibular free flap. Although the surgical resection margins were clear, local recurrence and lung metastasis occurred four months after surgery. She underwent concurrent chemo-radiation therapy, but the prognosis was poor. Conclusions: PIOC should be diagnosed differentially from odontogenic cyst even in pediatric populations. Surgery Oncology odontogenic cyst primary intraosseous carcinoma pediatric mandible Figures Figure 1 Figure 2 Figure 3 Figure 4 Background Primary intraosseous carcinoma (PIOC) is a rare and infrequently reported malignant odontogenic tumor. In 2005, the World health organization (WHO) divided PIOC into three subcategories according to histogenesis [1]. However, in 2017, WHO reclassified it as a single entity after leaving out unsubstantiated references to histogenesis in 2017 [2]. Approximately 260 cases have been reported [3, 4]. PIOC is more common in males and usually occurs in people 50 years and older [3, 5-11]. PIOC has occurred extremely rarely in pediatric populations: prior to this case, only nine cases had been reported in the English literature [12-20]. PIOC is misdiagnosed frequently as odontogenic cyst because it occasionally shows well-defined borders in panoramic view or on computed tomography (CT) [21-24]. In this study, a PIOC case of a 14-year-old female patient who was misdiagnosed with odontogenic cyst is discussed along with a literature review of pediatric PIOC cases. Case presentation A 14-year-old female patient was referred from a local dental clinic to the Department of Oral and Maxillofacial Surgery at Seoul National University Dental Hospital, Seoul, Korea. She presented with painful swelling on the mandibular right premolar area and complained of intermittent bleeding when she brushed her teeth. A panoramic view showed a radiolucent lesion with a well-defined border (Fig 1a). Adjacent tooth displacement and external root resorption were noted. There was a radiopaque focus in the upper area of the lesion. The patient was diagnosed with odontogenic cyst, and the cystic mass was enucleated with extraction of the right first premolar under local anesthesia. Histopathology of the surgical specimen revealed high cellularity and a sheet-like growth pattern with epithelial islands (Figs. 2a,2b). Microscopic examination showed round monotonous shaped tumor cells with high nucleus-cytoplasm ratio and bizarre mitosis (Figs. 2c,2d). On immunohistochemical staining, CK-7 and CK-14 showed focal positive. CK-18, CK-PAN, and Vimentin were positive. Moreover, cells were 40-60% positive for Ki-67. However, CD99, Desmin, S-100 protein, and NSE were negative. A final diagnosis was consistent with PIOC. Enhanced CT, magnetic resonance imaging (MRI), positron emission tomography-CT (PET-CT), bone scintigraphy and neck ultrasonography were performed. In enhanced CT, a periosteal reaction was observed on the buccal side (Fig. 3a). In MRI, diffuse enhancement of the soft tissue was observed at the adjacent buccal area (Fig. 3b). There were no significant lymph nodes. In PET-CT, a soft tissue lesion on the right premolar area and a borderline-sized lymph node at the right level IB were observed (Fig. 3c). There was no distant metastasis. In bone scintigraphy, increased uptake in the right mandible was observed (Fig. 3d). In neck ultrasonography, no significant cervical lymph node enlargement was observed. Hand-wrist radiograph and lower extremity angiography were performed for reconstruction surgery with the fibular free flap. In hand-wrist radiograph, that patient’s skeletal maturation was estimated as 15-16 years old. On leg angiography, anterior tibial, posterior tibial, and peroneal arteries were intact. One month after enucleation surgery, the patient showed abnormal healing (Fig. 4a). Surgical resection and reconstruction with a fibula free flap were prepared. The required bone length was 73 mm, and a resin stent was prepared (Fig. 4b). She underwent partial mandibulectomy from the right retromolar area to the left central incisor and selective neck dissection (right levels I, II, III) under general anesthesia. The mandibular and neck masses were removed en bloc (Fig. 4c) with simultaneous reconstruction using the microvascular fibula free flap (Fig. 4d). The surgical resection margin was clear, and no metastatic cervical lymph nodes were found in the dissected mass. Perineural or vascular invasion was not seen. However, there was involvement of the underlying bone. The patient’s healing was uneventful. However, local recurrence and lung metastasis occurred four months after surgery. She underwent concurrent chemo-radiation therapy (CCRT) for one month; however, a recurred lesion on the right mandibular ramus did not decrease (Fig. 1b). She was lost to follow-up after her visit one month after the end of CCRT. Discussion According to the previous case studies, nine PIOCs have been reported in pediatric populations [12-20]. A summary of all 10 pediatric cases, including this case, is provided in Table 1 and 2. PIOC is more common in adult males [3,5-11]; however, seven of the total 10 pediatric cases were female. PIOC occurs more often in the mandible than maxilla [3,5-11], seen in seven of the pediatric patients. The most common symptom was swelling, followed by pain [3,5,7]. The symptoms of the pediatric patients were consistent. PIOC can be misdiagnosed as odontogenic cyst because it occasionally presents with a well-defined border in panoramic view or CT [21-24]. Differential diagnosis of PIOC from odontogenic cyst is important because the surgical approach is different. Kaffe et al. [21] reported that 61% of PIOC cases presented as a unilocular radiolucent lesion. In our review of pediatric cases, six of 10 cases showed unilocular radiolucency. Radiologic borders that were defined but non-corticated were reported to occur in 57% of the PIOC cases and the remaining 43% had diffuse borders. In cases with poorly defined borders, such as those with diffuse margins, the lesions could be unambiguously distinguished from odontogenic cyst. However, since cases with well-defined borders can be misdiagnosed as odontogenic cyst, a differential diagnosis should be thoroughly considered. According to Kaffe et al. [21], a defined but non-corticated border could be a useful feature for differential diagnosis. A well-defined but non-corticated border was observed in five of the 10 pediatric PIOC patients (Table 1). Moreover, tooth displacement and root resorption should be considered as other radiologic features as PIOC tends to grow too rapidly to produce such features [7,19,21,25]. However, four of the pediatric cases in our review showed tooth displacement and five showed root resorption. It was peculiar that these features occurred in the pediatric patients. Although tooth displacement and root resorption are features for slowly growing lesions such as odontogenic cysts, these features should be considered for differential diagnosis of PIOC in pediatric populations. As a rare finding, radiopaque foci were observed in this case and in Punnya et al.’s pediatric case [15]. Although PIOC usually presents as an osteolytic lesion, small radiopaque foci due to calcification or periosteal reaction can be observed, albeit rarely [10,26-28]. Among the 10 pediatric cases we reviewed, the initial diagnosis for five was odontogenic cyst. Huang et al. [7] reported that this diagnostic delay did not show any statistically significant prognostic difference. However, Naruse et al. [25] reported that preoperative dental procedures might be potential prognostic factors and suggested that no intervention before definitive diagnosis could achieve a better prognosis. Therefore, incisional biopsy with obtaining multiple specimens is necessary to rule out an underlying carcinoma [8,9,14,17]. Regardless of patient age, biopsy should be considered for any lesion with any of the unusual radiographic presentations mentioned above. The pediatric patient reported by Charles et al. [17] was accurately diagnosed by biopsy and had the longest follow-up period without recurrence. A biopsy was not considered for definitive diagnosis in the present case although there were atypical radiographic findings. Local recurrence occurred five months after the initial operation, that is, four months after the definitive surgery. The primary treatment for PIOC is surgical resection [3,8,11,29]. In the present case, because simultaneous reconstruction was necessary, hand-wrist radiography was analyzed for assessment of growth potential. Her skeletal age was assessed as 15-16 years old. Previous research has concluded that the face matures between 12 and 15 years in males and two years earlier in females [30,31]. The vascularized fibular free flap is a reliable option for mandibular reconstruction, even in pediatric patients [32,33]. Therefore, the fibular free flap was employed for this 14-year-old female patient. Recent reviews reported the rate of cervical lymph node metastasis to be 12.8% [3] and 70.1% [11]. In our case review, three of the 10 pediatric cases showed cervical metastasis. Wenguang et al. [11] reported nodal status to be a significantly poor prognostic factor for survival. However, de Morais et al. [3] reported that lymph node metastasis was not statistically associated with survival. Although these outcomes conflict, it seems reasonable that neck dissection be considered among the surgical procedures for PIOC. In the present case, there were no metastatic lymph nodes on enhanced CT or MRI. However, because there were borderline-sized lymph nodes at the right level IB, selective neck dissection was performed. In recent literature, de Morais et al. [3] reported a local recurrence rate of 22.1% and Ye et al. [4] reported a local recurrence rate of 24.1%. In this study, local recurrence occurred in three pediatric patients, including the present case, a rate of 30% although the total cases were only 10. In one report, a four-year-old female patient suffered recurrence five months after excision and underwent additional radical excision [3]. However, 10 months later, recurrence recurred, the lesion was removed, and the area was irradiated. She was alive after one year of follow-up. In another case, a 16-year-old male patient suffered recurrence two months after excision and underwent total mandibulectomy after one month. However, he died two months after the surgery [13]. The 14-year-old female patient in the present study suffered recurrence four months after definitive surgery. She underwent CCRT for one months, but the recurred lesion did not decrease. According to de Morais et al. [3] and Ye et al. [4], local recurrence is a significant prognostic factor for survival. Likewise, pediatric PIOC cases with local recurrence showed poor prognosis. The five-year survival rate has been reported as 44.6% [3] and 53.2% [4]. However, the five-year survival rate of the pediatric patients could not be evaluated because of the rarity of the cases and relatively short follow-up periods. Because PIOC has a poor prognosis, accurate diagnosis and adequate surgical procedures are important. Continuous updates are required to analyze the pathophysiologic mechanism of PIOC, and a recent approach such as genetic analysis [34] could contribute to understanding the pathophysiology of PIOC. Conclusion PIOC is a rare malignant odontogenic tumor that can be misdiagnosed as odontogenic cyst because it occasionally presents with a well-defined border on radiography. According to the literature review, 10 pediatric PIOC cases have been reported, five of which were initially diagnosed as odontogenic cyst. PIOC with a well-defined border usually has non-corticated margin. Atypically, tooth displacement and root resorption were observed in approximately half of the pediatric cases. Incisional biopsy with multiple specimens is necessary to rule out PIOC in cases with atypical radiographic findings. PIOC should be differentially diagnosed from odontogenic cyst even in pediatric populations. Abbreviations PIOC: Primary intraosseous carcinoma; WHO: World health organization; CT: computed cosmography; MRI: Magnetic resonance imaging; PET-CT: Positron emission tomography-computed cosmography; CCRT: concurrent chemo-radiation therapy Declarations Ethics approval and consent to participate This article was approved by the Institutional Review Board of Seoul National University (S-D20200010). Consent for publication Written informed consent was obtained from the patient for publication of this manuscript and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Availability of data and materials The datasets used and/or analyzed during the current study are available from the corresponding author upon reasonable request. Competing interests The authors declare that they have no competing interests. Funding Not applicable. Authors' contributions All authors read and approved the final manuscript. HJO read and wrote the manuscript, DHS collected the literature data, HM designed the article, HJY prepared the histopathologic data, and SMK prepared the figures and wrote the manuscript. Acknowledgement This research was supported by Basic Science Research Program through the National Research Foundation of Korea (NRF) funded by the Ministry of Education(No. 2017R1D1A1B04029339). References Eversole LR, Siar CH, van der Waal I. Primary intraosseous squamous cell carcinomas. In: Barnes L, Eveson JW, Reichart P, Sidransky D, editors. Pathology and genetics of head and neck tumours. Lyon, France: IARC Press; 2005. p. 290-291. Wright JM, Vered M. Update from the 4th Edition of the World Health Organization Classification of Head and Neck Tumours: Odontogenic and Maxillofacial Bone Tumors. Head Neck Pathol. 2017;11(1):68-77. de Morais EF, Carlan LM, de Farias Morais HG, Pinheiro JC, Martins HDD, Barboza CAG, et al. Primary Intraosseous Squamous Cell Carcinoma Involving the Jaw Bones: A Systematic Review and Update. Head Neck Pathol. 2020. Ye P, Wei T, Gao Y, Zhang W, Peng X. Primary intraosseous squamous cell carcinoma arising from an odontogenic keratocyst: case series and literature review. Med Oral Patol Oral Cir Bucal. 2021;26(1):e49-e55. Thomas G, Pandey M, Mathew A, Abraham EK, Francis A, Somanathan T, et al. Primary intraosseous carcinoma of the jaw: pooled analysis of world literature and report of two new cases. Int J Oral Maxillofac Surg. 2001;30(4):349-355. Zwetyenga N, Pinsolle J, Rivel J, Majoufre-Lefebvre C, Faucher A, Pinsolle V. Primary intraosseous carcinoma of the jaws. Arch Otolaryngol Head Neck Surg. 2001;127(7):794-797. Huang JW, Luo HY, Li Q, Li TJ. Primary intraosseous squamous cell carcinoma of the jaws. Clinicopathologic presentation and prognostic factors. Arch Pathol Lab Med. 2009;133(11):1834-1840. Lugakingira M, Pytynia K, Kolokythas A, Miloro M. Primary intraosseous carcinoma of the mandible: case report and review of the literature. J Oral Maxillofac Surg. 2010;68(10):2623-2629. Bodner L, Manor E, Shear M, van der Waal I. Primary intraosseous squamous cell carcinoma arising in an odontogenic cyst: a clinicopathologic analysis of 116 reported cases. J Oral Pathol Med. 2011;40(10):733-738. Matsuzaki H, Katase N, Matsumura T, Hara M, Yanagi Y, Nagatsuka H, et al. Solid-type primary intraosseous squamous cell carcinoma of the mandible: a case report with histopathological and imaging features. Oral Surg Oral Med Oral Pathol Oral Radiol. 2012;114(5):e71-77. Wenguang X, Hao S, Xiaofeng Q, Zhiyong W, Yufeng W, Qingang H, et al. Prognostic Factors of Primary Intraosseous Squamous Cell Carcinoma (PIOSCC): A Retrospective Review. PLoS One. 2016;11(4):e0153646. Jones JH. Soft tissue oral tumours in children: their structure, histogenesis and behaviour. Proc R Soc Med. 1966;59(7):673-680. Sirsat MV, Sampat MB, Shrikhande SS. 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Primary intraosseous carcinoma of the mandible: A report of two cases. J Oral Maxillofac Pathol. 2010;14(2):69-72. Boni P, Sozzi D, Novelli G, Pagni F, Valente G, Bozzetti A. Primary Intraosseous Squamous Cell Carcinoma of the Jaws: 6 New Cases, Experience, and Literature Comparison. J Oral Maxillofac Surg. 2016;74(3):541-546. Nokovitch L, Bodard AG, Corradini N, Crozes C, Guyennon A, Deneuve S. Pediatric case of squamous cell carcinoma arising from a keratocystic odontogenic tumor. Int J Pediatr Otorhinolaryngol. 2018;112:121-125. Kaffe I, Ardekian L, Peled M, Machtey E, Laufer D. Radiological features of primary intra-osseous carcinoma of the jaws. Analysis of the literature and report of a new case. Dentomaxillofac Radiol. 1998;27(4):209-214. Scheer M, Koch AM, Drebber U, Kubler AC. Primary intraosseous carcinoma of the jaws arising from an odontogenic cyst--a case report. J Craniomaxillofac Surg. 2004;32(3):166-169. Choi YJ, Oh SH, Kang JH, Choi HY, Kim GT, Yu JJ, et al. Primary intraosseous squamous cell carcinoma mimicking periapical disease: a case report. Imaging Sci Dent. 2012;42(4):265-270. Abdelkarim AZ, Elzayat AM, Syed AZ, Lozanoff S. Delayed diagnosis of a primary intraosseous squamous cell carcinoma: A case report. Imaging Sci Dent. 2019;49(1):71-77. Naruse T, Yanamoto S, Sakamoto Y, Ikeda T, Yamada SI, Umeda M. Clinicopathological Study of Primary Intraosseous Squamous Cell Carcinoma of the Jaw and a Review of the Literature. J Oral Maxillofac Surg. 2016;74(12):2420-2427. Bennett JH, Jones J, Speight PM. Odontogenic squamous cell carcinoma with osseous metaplasia. J Oral Pathol Med. 1993;22(6):286-288. Ide F, Shimoyama T, Horie N, Kaneko T. Primary intraosseous carcinoma of the mandible with probable origin from reduced enamel epithelium. J Oral Pathol Med. 1999;28(9):420-422. Lopes Dias J, Borges A, Lima Rego R. Primary intraosseous squamous cell carcinoma of the mandible: a case with atypical imaging features. BJR Case Rep. 2016;2(4):20150276. Woolgar JA, Triantafyllou A, Ferlito A, Devaney KO, Lewis JS, Jr., Rinaldo A, et al. Intraosseous carcinoma of the jaws: a clinicopathologic review. Part III: Primary intraosseous squamous cell carcinoma. Head Neck. 2013;35(6):906-909. Farkas LG, Posnick JC, Hreczko TM. Growth patterns of the face: a morphometric study. Cleft Palate Craniofac J. 1992;29(4):308-315. Li JS, Chen WL, Huang ZQ, Zhang DM. Pediatric mandibular reconstruction after benign tumor ablation using a vascularized fibular flap. J Craniofac Surg. 2009;20(2):431-434. Guo L, Ferraro NF, Padwa BL, Kaban LB, Upton J. Vascularized fibular graft for pediatric mandibular reconstruction. Plast Reconstr Surg. 2008;121(6):2095-2105. Benoit MM, Vargas SO, Bhattacharyya N, McGill TA, Robson CD, Ferraro N, et al. The presentation and management of mandibular tumors in the pediatric population. Laryngoscope. 2013;123(8):2035-2042. Yukimori A, Tsuchiya M, Wada A, Michi Y, Kayamori K, Sakamoto K, et al. Genetic and histopathological analysis of a case of primary intraosseous carcinoma, NOS with features of both ameloblastic carcinoma and squamous cell carcinoma. World J Surg Oncol. 2020;18(1):45. Tables Table 1. Clinical and radiological characteristics of 10 pediatric patients with primary intraosseous carcinoma in jaw. Case Year Author Country Age Sex Location Symptom Locularity Density Border Tooth displacement Root resorption 1 1966 Jones [12] Ireland 4 F Mandible Swelling N/S N/S N/S Yes N/S 2 1973 Sirsat et al. [13] India 16 M Mandible Swelling Not loculated Mixed Diffuse Yes N/S 3 2002 Gulbranson et al. [14] America 16 F Mandible Swelling Unilocular Radiolucency Cortical thinning N/S N/S 4 2004 Punnya et al. [15] India 18 F Maxilla Swelling N/S Radiolucency, multiple radiopaque foci Well defined, non-corticated N/S Yes 5 2006 Chaisuparat et al. [16] America 18 F Maxilla Swelling Unilocular Radiolucency, Well defined, non-corticated N/S N/S 6 2008 Charles et al. [17] Canada 5 F Mandible Swelling Unilocular Radiolucency, enlarged crypt, Well defined, non-corticated Yes Yes 7 2010 Sengupta et al. [18] India 16 M Mandible Swelling, pain Unilocular Radiolucency Well defined, non-corticated N/S Yes 8 2016 Boni et al. [19] Italy 14 M Maxilla Swelling N/S N/S N/S No No 9 2018 Nokovitch et al. [20] France 15 F Mandible Swelling, pain, intermittent bleeding Unilocular Radiolucency, supernumerary tooth Lingual cortex lysis N/S Yes 10 2020 Present study Korea 14 F Mandible Swelling, pain, intermittent bleeding Unilocular Radiolucency, a radiopaque focus Well defined, non-corticated Yes Yes N/S not specified Table 2. Treatment outcomes of 10 pediatric patients with primary intraosseous carcinoma in jaw. Case Initial diagnosis Initial treatment Confirmed diagnosis Definitive treatment Cervical metastasis Local recurrence Salvage treatment Follow-up duration Survival status at the last F/U 1 N/S N/S N/S Excision Yes a Yes Radical Excision / SND, RT a 27 months alive 2 N/S N/S N/S Excision Yes b Yes Mandibulectomy, SND 8 months dead 3 Dentigerous cyst Incisional biopsy Carcinoma Mandibulectomy, SND No No No 8 months alive 4 Odontogenic cyst FNAC Odontogenic cyst Enucleation N/S No No 16 months alive 5 N/S N/S N/S Maxillectomy, RT No No No 44 months alive 6 More aggressive lesion than odontogenic cyst Incisional biopsy with extraction PIOC Mandibulectomy, mRND, Reconstruction with plate, RT Yes No No 7 years alive 7 OKC Enucleation with extraction PIOC Refer N/S N/S N/S N/S N/S 8 N/S N/S N/S Maxillectomy N/S No No 5 years alive 9 OKC Incisional biopsy OKC Enucleation with extraction No No No 18 months alive 10 Odontogenic cyst Enucleation with extraction PIOC Mandibulectomy, SND, Reconstruction with FFF No Yes CCRT 9 months c alive F/U follow-up, N/S not specified, OKC odontogenic keratocyst, FNAC fine needle aspiration cytology, PIOC primary intraosseous carcinoma, SND selective neck dissection, mRND modified radical neck dissection, RT radiation therapy, CCRT concurrent chemo-radiation therapy, FFF fibular free flap a at second recurrence b at recurrence c recurred but lost to follow-up. 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","description":"","filename":"Figure3.jpg","url":"https://assets-eu.researchsquare.com/files/rs-195662/v1/c39e36b5798a8efaded3a78d.jpg"},{"id":5795034,"identity":"03eee523-87cd-461f-83e2-7ff047c614e8","added_by":"auto","created_at":"2021-02-09 19:20:47","extension":"jpg","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":1545991,"visible":true,"origin":"","legend":"Surgical procedures. a Abnormal healing one month after enucleation surgery. b The 73-mm resin stent for reconstruction with fibula free flap. c En bloc resection of the right mandible and neck mass (right level I, II, III) d Intraoperative fibular contouring using the stent. ","description":"","filename":"Figure4.jpg","url":"https://assets-eu.researchsquare.com/files/rs-195662/v1/223f94bec3001ebcaf1522ed.jpg"},{"id":13657686,"identity":"36e440fe-5407-4895-840a-55f49507d51d","added_by":"auto","created_at":"2021-09-17 10:11:37","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":536970,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-195662/v1/0af13517-2e5e-468b-8788-508b9a8c0237.pdf"}],"financialInterests":"","formattedTitle":"\u003cp\u003ePrimary Intraosseous Carcinoma in the Pediatric Mandible\u003c/p\u003e","fulltext":[{"header":"Background","content":"\u003cp\u003ePrimary intraosseous carcinoma (PIOC) is a rare and infrequently reported malignant odontogenic tumor. In 2005, the World health organization (WHO) divided PIOC into three subcategories according to histogenesis [1]. However, in 2017, WHO reclassified it as a single entity after leaving out unsubstantiated references to histogenesis in 2017 [2]. Approximately 260 cases have been reported [3, 4]. PIOC is more common in males and usually occurs in people 50 years and older [3, 5-11]. PIOC has occurred extremely rarely in pediatric populations: prior to this case, only nine cases had been reported in the English literature [12-20]. PIOC is misdiagnosed frequently as odontogenic cyst because it occasionally shows well-defined borders in panoramic view or on computed tomography (CT) [21-24]. In this study, a PIOC case of a 14-year-old female patient who was misdiagnosed with odontogenic cyst is discussed along with a literature review of pediatric PIOC cases.\u003c/p\u003e"},{"header":"Case presentation","content":"\u003cp\u003eA 14-year-old female patient was referred from a local dental clinic to the Department of Oral and Maxillofacial Surgery at Seoul National University Dental Hospital, Seoul, Korea. She presented with painful swelling on the mandibular right premolar area and complained of intermittent bleeding when she brushed her teeth. A panoramic view showed a radiolucent lesion with a well-defined border (Fig 1a). Adjacent tooth displacement and external root resorption were noted. There was a radiopaque focus in the upper area of the lesion. The patient was diagnosed with odontogenic cyst, and the cystic mass was enucleated with extraction of the right first premolar under local anesthesia.\u003c/p\u003e\n\u003cp\u003eHistopathology of the surgical specimen revealed high cellularity and a sheet-like growth pattern with epithelial islands (Figs. 2a,2b). Microscopic examination showed round monotonous shaped tumor cells with high nucleus-cytoplasm ratio and bizarre mitosis (Figs. 2c,2d). On immunohistochemical staining, CK-7 and CK-14 showed focal positive. CK-18, CK-PAN, and Vimentin were positive. Moreover, cells were 40-60% positive for Ki-67. However, CD99, Desmin, S-100 protein, and NSE were negative. A final diagnosis was consistent with PIOC.\u003c/p\u003e\n\u003cp\u003eEnhanced CT, magnetic resonance imaging (MRI), positron emission tomography-CT (PET-CT), bone scintigraphy and neck ultrasonography were performed. In enhanced CT, a periosteal reaction was observed on the buccal side (Fig. 3a). In MRI, diffuse enhancement of the soft tissue was observed at the adjacent buccal area (Fig. 3b). There were no significant lymph nodes. In PET-CT, a soft tissue lesion on the right premolar area and a borderline-sized lymph node at the right level IB were observed (Fig. 3c). There was no distant metastasis. In bone scintigraphy, increased uptake in the right mandible was observed (Fig. 3d). In neck ultrasonography, no significant cervical lymph node enlargement was observed. Hand-wrist radiograph and lower extremity angiography were performed for reconstruction surgery with the fibular free flap. In hand-wrist radiograph, that patient\u0026rsquo;s skeletal maturation was estimated as 15-16 years old. On leg angiography, anterior tibial, posterior tibial, and peroneal arteries were intact.\u003c/p\u003e\n\u003cp\u003eOne month after enucleation surgery, the patient showed abnormal healing (Fig. 4a). Surgical resection and reconstruction with a fibula free flap were prepared. The required bone length was 73 mm, and a resin stent was prepared (Fig. 4b). She underwent partial mandibulectomy from the right retromolar area to the left central incisor and selective neck dissection (right levels I, II, III) under general anesthesia. The mandibular and neck masses were removed en bloc (Fig. 4c) with simultaneous reconstruction using the microvascular fibula free flap (Fig. 4d).\u003c/p\u003e\n\u003cp\u003eThe surgical resection margin was clear, and no metastatic cervical lymph nodes were found in the dissected mass. Perineural or vascular invasion was not seen. However, there was involvement of the underlying bone. The patient\u0026rsquo;s healing was uneventful. However, local recurrence and lung metastasis occurred four months after surgery. She underwent concurrent chemo-radiation therapy (CCRT) for one month; however, a recurred lesion on the right mandibular ramus did not decrease (Fig. 1b). She was lost to follow-up after her visit one month after the end of CCRT.\u0026nbsp;\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eAccording to the previous case studies, nine PIOCs have been reported in pediatric populations [12-20]. A summary of all 10 pediatric cases, including this case, is provided in Table 1 and 2. PIOC is more common in adult males [3,5-11]; however, seven of the total 10 pediatric cases were female. PIOC occurs more often in the mandible than maxilla [3,5-11], seen in seven of the pediatric patients. The most common symptom was swelling, followed by pain [3,5,7]. The symptoms of the pediatric patients were consistent.\u003c/p\u003e\n\u003cp\u003ePIOC can be misdiagnosed as odontogenic cyst because it occasionally presents with a well-defined border in panoramic view or CT [21-24]. Differential diagnosis of PIOC from odontogenic cyst is important because the surgical approach is different. Kaffe et al. [21] reported that 61% of PIOC cases presented as a unilocular radiolucent lesion. In our review of pediatric cases, six of 10 cases showed unilocular radiolucency. Radiologic borders that were defined but non-corticated were reported to occur in 57% of the PIOC cases and the remaining 43% had diffuse borders. In cases with poorly defined borders, such as those with diffuse margins, the lesions could be unambiguously distinguished from odontogenic cyst. However, since cases with well-defined borders can be misdiagnosed as odontogenic cyst, a differential diagnosis should be thoroughly considered. According to Kaffe et al. [21], a defined but non-corticated border could be a useful feature for differential diagnosis. A well-defined but non-corticated border was observed in five of the 10 pediatric PIOC patients (Table 1). Moreover, tooth displacement and root resorption should be considered as other radiologic features as PIOC tends to grow too rapidly to produce such features [7,19,21,25]. However, four of the pediatric cases in our review showed tooth displacement and five showed root resorption. It was peculiar that these features occurred in the pediatric patients. Although tooth displacement and root resorption are features for slowly growing lesions such as odontogenic cysts, these features should be considered for differential diagnosis of PIOC in pediatric populations. As a rare finding, radiopaque foci were observed in this case and in Punnya et al.\u0026rsquo;s pediatric case [15]. Although PIOC usually presents as an osteolytic lesion, small radiopaque foci due to calcification or periosteal reaction can be observed, albeit rarely [10,26-28].\u003c/p\u003e\n\u003cp\u003eAmong the 10 pediatric cases we reviewed, the initial diagnosis for five was odontogenic cyst. Huang et al. [7] reported that this diagnostic delay did not show any statistically significant prognostic difference. However, Naruse et al. [25] reported that preoperative dental procedures might be potential prognostic factors and suggested that no intervention before definitive diagnosis could achieve a better prognosis. Therefore, incisional biopsy with obtaining multiple specimens is necessary to rule out an underlying carcinoma [8,9,14,17]. Regardless of patient age, biopsy should be considered for any lesion with any of the unusual radiographic presentations mentioned above. The pediatric patient reported by Charles et al. [17] was accurately diagnosed by biopsy and had the longest follow-up period without recurrence. A biopsy was not considered for definitive diagnosis in the present case although there were atypical radiographic findings. Local recurrence occurred five months after the initial operation, that is, four months after the definitive surgery.\u003c/p\u003e\n\u003cp\u003eThe primary treatment for PIOC is surgical resection [3,8,11,29]. In the present case, because simultaneous reconstruction was necessary, hand-wrist radiography was analyzed for assessment of growth potential. Her skeletal age was assessed as 15-16 years old. Previous research has concluded that the face matures between 12 and 15 years in males and two years earlier in females [30,31]. The vascularized fibular free flap is a reliable option for mandibular reconstruction, even in pediatric patients [32,33]. Therefore, the fibular free flap was employed for this 14-year-old female patient.\u003c/p\u003e\n\u003cp\u003eRecent reviews reported the rate of cervical lymph node metastasis to be 12.8% [3] and 70.1% [11]. In our case review, three of the 10 pediatric cases showed cervical metastasis. Wenguang et al. [11] reported nodal status to be a significantly poor prognostic factor for survival. However, de Morais et al. [3] reported that lymph node metastasis was not statistically associated with survival. Although these outcomes conflict, it seems reasonable that neck dissection be considered among the surgical procedures for PIOC. In the present case, there were no metastatic lymph nodes on enhanced CT or MRI. However, because there were borderline-sized lymph nodes at the right level IB, selective neck dissection was performed.\u003c/p\u003e\n\u003cp\u003eIn recent literature, de Morais et al. [3] reported a local recurrence rate of 22.1% and Ye et al. [4] reported a local recurrence rate of 24.1%. In this study, local recurrence occurred in three pediatric patients, including the present case, a rate of 30% although the total cases were only 10. In one report, a four-year-old female patient suffered recurrence five months after excision and underwent additional radical excision [3]. However, 10 months later, recurrence recurred, the lesion was removed, and the area was irradiated. She was alive after one year of follow-up. In another case, a 16-year-old male patient suffered recurrence two months after excision and underwent total mandibulectomy after one month. However, he died two months after the surgery [13]. The 14-year-old female patient in the present study suffered recurrence four months after definitive surgery. She underwent CCRT for one months, but the recurred lesion did not decrease. According to de Morais et al. [3] and Ye et al. [4], local recurrence is a significant prognostic factor for survival. Likewise, pediatric PIOC cases with local recurrence showed poor prognosis. The five-year survival rate has been reported as 44.6% [3] and 53.2% [4]. However, the five-year survival rate of the pediatric patients could not be evaluated because of the rarity of the cases and relatively short follow-up periods.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eBecause PIOC has a poor prognosis, accurate diagnosis and adequate surgical procedures are important. Continuous updates are required to analyze the pathophysiologic mechanism of PIOC, and a recent approach such as genetic analysis [34] could contribute to understanding the pathophysiology of PIOC.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003ePIOC is a rare malignant odontogenic tumor that can be misdiagnosed as odontogenic cyst because it occasionally presents with a well-defined border on radiography. According to the literature review, 10 pediatric PIOC cases have been reported, five of which were initially diagnosed as odontogenic cyst. PIOC with a well-defined border usually has non-corticated margin. Atypically, tooth displacement and root resorption were observed in approximately half of the pediatric cases. Incisional biopsy with multiple specimens is necessary to rule out PIOC in cases with atypical radiographic findings. PIOC should be differentially diagnosed from odontogenic cyst even in pediatric populations.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cp\u003ePIOC: Primary intraosseous carcinoma; WHO: World health organization; CT: computed cosmography; MRI: Magnetic resonance imaging; PET-CT: Positron emission tomography-computed cosmography; CCRT: concurrent chemo-radiation therapy\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis article was approved by the Institutional Review Board of Seoul National University (S-D20200010).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent was obtained from the patient for publication of this manuscript and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe datasets used and/or analyzed during the current study are available from the corresponding author upon reasonable request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare that they have no competing interests.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding \u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors' contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll authors read and approved the final manuscript. \u003cstrong\u003eHJO\u003c/strong\u003e read and wrote the manuscript, \u003cstrong\u003eDHS\u003c/strong\u003e collected the literature data, \u003cstrong\u003eHM\u003c/strong\u003e designed the article, \u003cstrong\u003eHJY\u003c/strong\u003e prepared the histopathologic data, and \u003cstrong\u003eSMK\u003c/strong\u003e prepared the figures and wrote the manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcknowledgement\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis research was supported by Basic Science Research Program through the National Research Foundation of Korea (NRF) funded by the Ministry of Education(No. 2017R1D1A1B04029339).\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eEversole LR, Siar CH, van der Waal I. 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Int J Pediatr Otorhinolaryngol. 2018;112:121-125.\u003c/li\u003e\n\u003cli\u003eKaffe I, Ardekian L, Peled M, Machtey E, Laufer D. Radiological features of primary intra-osseous carcinoma of the jaws. Analysis of the literature and report of a new case. Dentomaxillofac Radiol. 1998;27(4):209-214.\u003c/li\u003e\n\u003cli\u003eScheer M, Koch AM, Drebber U, Kubler AC. Primary intraosseous carcinoma of the jaws arising from an odontogenic cyst--a case report. J Craniomaxillofac Surg. 2004;32(3):166-169.\u003c/li\u003e\n\u003cli\u003eChoi YJ, Oh SH, Kang JH, Choi HY, Kim GT, Yu JJ, et al. Primary intraosseous squamous cell carcinoma mimicking periapical disease: a case report. Imaging Sci Dent. 2012;42(4):265-270.\u003c/li\u003e\n\u003cli\u003eAbdelkarim AZ, Elzayat AM, Syed AZ, Lozanoff S. Delayed diagnosis of a primary intraosseous squamous cell carcinoma: A case report. Imaging Sci Dent. 2019;49(1):71-77.\u003c/li\u003e\n\u003cli\u003eNaruse T, Yanamoto S, Sakamoto Y, Ikeda T, Yamada SI, Umeda M. Clinicopathological Study of Primary Intraosseous Squamous Cell Carcinoma of the Jaw and a Review of the Literature. J Oral Maxillofac Surg. 2016;74(12):2420-2427.\u003c/li\u003e\n\u003cli\u003eBennett JH, Jones J, Speight PM. Odontogenic squamous cell carcinoma with osseous metaplasia. J Oral Pathol Med. 1993;22(6):286-288.\u003c/li\u003e\n\u003cli\u003eIde F, Shimoyama T, Horie N, Kaneko T. Primary intraosseous carcinoma of the mandible with probable origin from reduced enamel epithelium. J Oral Pathol Med. 1999;28(9):420-422.\u003c/li\u003e\n\u003cli\u003eLopes Dias J, Borges A, Lima Rego R. Primary intraosseous squamous cell carcinoma of the mandible: a case with atypical imaging features. BJR Case Rep. 2016;2(4):20150276.\u003c/li\u003e\n\u003cli\u003eWoolgar JA, Triantafyllou A, Ferlito A, Devaney KO, Lewis JS, Jr., Rinaldo A, et al. Intraosseous carcinoma of the jaws: a clinicopathologic review. Part III: Primary intraosseous squamous cell carcinoma. Head Neck. 2013;35(6):906-909.\u003c/li\u003e\n\u003cli\u003eFarkas LG, Posnick JC, Hreczko TM. Growth patterns of the face: a morphometric study. Cleft Palate Craniofac J. 1992;29(4):308-315.\u003c/li\u003e\n\u003cli\u003eLi JS, Chen WL, Huang ZQ, Zhang DM. Pediatric mandibular reconstruction after benign tumor ablation using a vascularized fibular flap. J Craniofac Surg. 2009;20(2):431-434.\u003c/li\u003e\n\u003cli\u003eGuo L, Ferraro NF, Padwa BL, Kaban LB, Upton J. Vascularized fibular graft for pediatric mandibular reconstruction. Plast Reconstr Surg. 2008;121(6):2095-2105.\u003c/li\u003e\n\u003cli\u003eBenoit MM, Vargas SO, Bhattacharyya N, McGill TA, Robson CD, Ferraro N, et al. The presentation and management of mandibular tumors in the pediatric population. Laryngoscope. 2013;123(8):2035-2042.\u003c/li\u003e\n\u003cli\u003eYukimori A, Tsuchiya M, Wada A, Michi Y, Kayamori K, Sakamoto K, et al. Genetic and histopathological analysis of a case of primary intraosseous carcinoma, NOS with features of both ameloblastic carcinoma and squamous cell carcinoma. World J Surg Oncol. 2020;18(1):45.\u003c/li\u003e\n\u003c/ol\u003e"},{"header":"Tables","content":"\u003cp\u003e\u003cstrong\u003eTable 1.\u003c/strong\u003e Clinical and radiological characteristics of 10 pediatric patients with primary intraosseous carcinoma in jaw.\u003c/p\u003e\n\u003ctable border=\"1\" width=\"0\"\u003e\n\u003ctbody\u003e\n\u003ctr\u003e\n\u003ctd width=\"40\"\u003e\n\u003cp\u003e\u003cstrong\u003eCase\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e\u003cstrong\u003eYear\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"119\"\u003e\n\u003cp\u003e\u003cstrong\u003eAuthor\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"65\"\u003e\n\u003cp\u003e\u003cstrong\u003eCountry\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e\u003cstrong\u003eAge\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e\u003cstrong\u003eSex\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"63\"\u003e\n\u003cp\u003e\u003cstrong\u003eLocation\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"116\"\u003e\n\u003cp\u003e\u003cstrong\u003eSymptom\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"84\"\u003e\n\u003cp\u003e\u003cstrong\u003eLocularity\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"136\"\u003e\n\u003cp\u003e\u003cstrong\u003eDensity\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"102\"\u003e\n\u003cp\u003e\u003cstrong\u003eBorder \u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"82\"\u003e\n\u003cp\u003e\u003cstrong\u003eTooth displacement\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"68\"\u003e\n\u003cp\u003e\u003cstrong\u003eRoot resorption\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"40\"\u003e\n\u003cp\u003e1\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e1966\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"119\"\u003e\n\u003cp\u003eJones [12]\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"65\"\u003e\n\u003cp\u003eIreland\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e4\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003eF\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"63\"\u003e\n\u003cp\u003eMandible\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"116\"\u003e\n\u003cp\u003eSwelling\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"84\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"136\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"102\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"82\"\u003e\n\u003cp\u003eYes\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"68\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"40\"\u003e\n\u003cp\u003e2\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e1973\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"119\"\u003e\n\u003cp\u003eSirsat et al. [13]\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"65\"\u003e\n\u003cp\u003eIndia\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e16\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003eM\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"63\"\u003e\n\u003cp\u003eMandible\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"116\"\u003e\n\u003cp\u003eSwelling\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"84\"\u003e\n\u003cp\u003eNot loculated\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"136\"\u003e\n\u003cp\u003eMixed\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"102\"\u003e\n\u003cp\u003eDiffuse\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"82\"\u003e\n\u003cp\u003eYes\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"68\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"40\"\u003e\n\u003cp\u003e3\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e2002\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"119\"\u003e\n\u003cp\u003eGulbranson et al. [14]\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"65\"\u003e\n\u003cp\u003eAmerica\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e16\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003eF\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"63\"\u003e\n\u003cp\u003eMandible\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"116\"\u003e\n\u003cp\u003eSwelling\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"84\"\u003e\n\u003cp\u003eUnilocular\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"136\"\u003e\n\u003cp\u003eRadiolucency\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"102\"\u003e\n\u003cp\u003eCortical thinning\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"82\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"68\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"40\"\u003e\n\u003cp\u003e4\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e2004\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"119\"\u003e\n\u003cp\u003ePunnya et al. [15]\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"65\"\u003e\n\u003cp\u003eIndia\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e18\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003eF\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"63\"\u003e\n\u003cp\u003eMaxilla\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"116\"\u003e\n\u003cp\u003eSwelling\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"84\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"136\"\u003e\n\u003cp\u003eRadiolucency,\u003cbr /\u003e multiple radiopaque foci\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"102\"\u003e\n\u003cp\u003eWell defined,\u003cbr /\u003e non-corticated\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"82\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"68\"\u003e\n\u003cp\u003eYes\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"40\"\u003e\n\u003cp\u003e5\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e2006\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"119\"\u003e\n\u003cp\u003eChaisuparat et al. [16]\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"65\"\u003e\n\u003cp\u003eAmerica\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e18\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003eF\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"63\"\u003e\n\u003cp\u003eMaxilla\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"116\"\u003e\n\u003cp\u003eSwelling\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"84\"\u003e\n\u003cp\u003eUnilocular\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"136\"\u003e\n\u003cp\u003eRadiolucency,\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"102\"\u003e\n\u003cp\u003eWell defined,\u003cbr /\u003e non-corticated\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"82\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"68\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"40\"\u003e\n\u003cp\u003e6\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e2008\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"119\"\u003e\n\u003cp\u003eCharles et al. [17]\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"65\"\u003e\n\u003cp\u003eCanada\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e5\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003eF\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"63\"\u003e\n\u003cp\u003eMandible\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"116\"\u003e\n\u003cp\u003eSwelling\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"84\"\u003e\n\u003cp\u003eUnilocular\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"136\"\u003e\n\u003cp\u003eRadiolucency,\u003cbr /\u003e enlarged crypt,\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"102\"\u003e\n\u003cp\u003eWell defined,\u003cbr /\u003e non-corticated\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"82\"\u003e\n\u003cp\u003eYes\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"68\"\u003e\n\u003cp\u003eYes\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"40\"\u003e\n\u003cp\u003e7\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e2010\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"119\"\u003e\n\u003cp\u003eSengupta et al. [18]\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"65\"\u003e\n\u003cp\u003eIndia\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e16\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003eM\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"63\"\u003e\n\u003cp\u003eMandible\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"116\"\u003e\n\u003cp\u003eSwelling, pain\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"84\"\u003e\n\u003cp\u003eUnilocular\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"136\"\u003e\n\u003cp\u003eRadiolucency\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"102\"\u003e\n\u003cp\u003eWell defined,\u003cbr /\u003e non-corticated\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"82\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"68\"\u003e\n\u003cp\u003eYes\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"40\"\u003e\n\u003cp\u003e8\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e2016\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"119\"\u003e\n\u003cp\u003eBoni et al. [19]\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"65\"\u003e\n\u003cp\u003eItaly\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e14\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003eM\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"63\"\u003e\n\u003cp\u003eMaxilla\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"116\"\u003e\n\u003cp\u003eSwelling\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"84\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"136\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"102\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"82\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"68\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"40\"\u003e\n\u003cp\u003e9\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e2018\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"119\"\u003e\n\u003cp\u003eNokovitch et al. [20]\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"65\"\u003e\n\u003cp\u003eFrance\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e15\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003eF\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"63\"\u003e\n\u003cp\u003eMandible\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"116\"\u003e\n\u003cp\u003eSwelling, pain, intermittent bleeding\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"84\"\u003e\n\u003cp\u003eUnilocular\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"136\"\u003e\n\u003cp\u003eRadiolucency,\u003cbr /\u003e supernumerary tooth\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"102\"\u003e\n\u003cp\u003eLingual cortex lysis\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"82\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"68\"\u003e\n\u003cp\u003eYes\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"40\"\u003e\n\u003cp\u003e10\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e2020\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"119\"\u003e\n\u003cp\u003ePresent study\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"65\"\u003e\n\u003cp\u003eKorea\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003e14\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"36\"\u003e\n\u003cp\u003eF\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"63\"\u003e\n\u003cp\u003eMandible\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"116\"\u003e\n\u003cp\u003eSwelling, pain, intermittent bleeding\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"84\"\u003e\n\u003cp\u003eUnilocular\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"136\"\u003e\n\u003cp\u003eRadiolucency,\u003cbr /\u003e a radiopaque focus\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"102\"\u003e\n\u003cp\u003eWell defined,\u003cbr /\u003e non-corticated\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"82\"\u003e\n\u003cp\u003eYes\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"68\"\u003e\n\u003cp\u003eYes\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003e\u003cem\u003eN/S\u003c/em\u003e not specified\u003c/p\u003e\n\u003cp\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eTable 2.\u003c/strong\u003e Treatment outcomes of 10 pediatric patients with primary intraosseous carcinoma in jaw.\u003c/p\u003e\n\u003ctable border=\"1\" width=\"0\"\u003e\n\u003ctbody\u003e\n\u003ctr\u003e\n\u003ctd width=\"38\"\u003e\n\u003cp\u003e\u003cstrong\u003eCase\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"130\"\u003e\n\u003cp\u003e\u003cstrong\u003eInitial diagnosis\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"95\"\u003e\n\u003cp\u003e\u003cstrong\u003eInitial treatment\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"94\"\u003e\n\u003cp\u003e\u003cstrong\u003eConfirmed diagnosis\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"151\"\u003e\n\u003cp\u003e\u003cstrong\u003eDefinitive treatment\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003e\u003cstrong\u003eCervical metastasis\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003e\u003cstrong\u003eLocal recurrence\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"113\"\u003e\n\u003cp\u003e\u003cstrong\u003eSalvage treatment\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"76\"\u003e\n\u003cp\u003e\u003cstrong\u003eFollow-up duration\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"85\"\u003e\n\u003cp\u003e\u003cstrong\u003eSurvival status at the last F/U\u003c/strong\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"38\"\u003e\n\u003cp\u003e1\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"130\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"95\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"94\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"151\"\u003e\n\u003cp\u003eExcision\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eYes \u003csup\u003ea\u003c/sup\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eYes\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"113\"\u003e\n\u003cp\u003eRadical Excision / SND, RT \u003csup\u003ea\u003c/sup\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"76\"\u003e\n\u003cp\u003e27 months\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"85\"\u003e\n\u003cp\u003ealive\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"38\"\u003e\n\u003cp\u003e2\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"130\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"95\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"94\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"151\"\u003e\n\u003cp\u003eExcision\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eYes \u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eYes\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"113\"\u003e\n\u003cp\u003eMandibulectomy, SND\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"76\"\u003e\n\u003cp\u003e8 months\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"85\"\u003e\n\u003cp\u003edead\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"38\"\u003e\n\u003cp\u003e3\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"130\"\u003e\n\u003cp\u003eDentigerous cyst\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"95\"\u003e\n\u003cp\u003eIncisional biopsy\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"94\"\u003e\n\u003cp\u003eCarcinoma\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"151\"\u003e\n\u003cp\u003eMandibulectomy, SND\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"113\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"76\"\u003e\n\u003cp\u003e8 months\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"85\"\u003e\n\u003cp\u003ealive\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"38\"\u003e\n\u003cp\u003e4\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"130\"\u003e\n\u003cp\u003eOdontogenic cyst\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"95\"\u003e\n\u003cp\u003eFNAC\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"94\"\u003e\n\u003cp\u003eOdontogenic cyst\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"151\"\u003e\n\u003cp\u003eEnucleation\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"113\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"76\"\u003e\n\u003cp\u003e16 months\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"85\"\u003e\n\u003cp\u003ealive\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"38\"\u003e\n\u003cp\u003e5\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"130\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"95\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"94\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"151\"\u003e\n\u003cp\u003eMaxillectomy, RT\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"113\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"76\"\u003e\n\u003cp\u003e44 months\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"85\"\u003e\n\u003cp\u003ealive\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"38\"\u003e\n\u003cp\u003e6\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"130\"\u003e\n\u003cp\u003eMore aggressive lesion than odontogenic cyst\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"95\"\u003e\n\u003cp\u003eIncisional biopsy with extraction\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"94\"\u003e\n\u003cp\u003ePIOC\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"151\"\u003e\n\u003cp\u003eMandibulectomy, mRND,\u003cbr /\u003e Reconstruction with plate, RT\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eYes\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"113\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"76\"\u003e\n\u003cp\u003e7 years\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"85\"\u003e\n\u003cp\u003ealive\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"38\"\u003e\n\u003cp\u003e7\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"130\"\u003e\n\u003cp\u003eOKC\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"95\"\u003e\n\u003cp\u003eEnucleation with extraction\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"94\"\u003e\n\u003cp\u003ePIOC\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"151\"\u003e\n\u003cp\u003eRefer\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"113\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"76\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"85\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"38\"\u003e\n\u003cp\u003e8\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"130\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"95\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"94\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"151\"\u003e\n\u003cp\u003eMaxillectomy\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eN/S\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"113\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"76\"\u003e\n\u003cp\u003e5 years\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"85\"\u003e\n\u003cp\u003ealive\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"38\"\u003e\n\u003cp\u003e9\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"130\"\u003e\n\u003cp\u003eOKC\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"95\"\u003e\n\u003cp\u003eIncisional biopsy\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"94\"\u003e\n\u003cp\u003eOKC\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"151\"\u003e\n\u003cp\u003eEnucleation with extraction\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"113\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"76\"\u003e\n\u003cp\u003e18 months\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"85\"\u003e\n\u003cp\u003ealive\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd width=\"38\"\u003e\n\u003cp\u003e10\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"130\"\u003e\n\u003cp\u003eOdontogenic cyst\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"95\"\u003e\n\u003cp\u003eEnucleation with extraction\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"94\"\u003e\n\u003cp\u003ePIOC\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"151\"\u003e\n\u003cp\u003eMandibulectomy, SND, Reconstruction with FFF\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eNo\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"66\"\u003e\n\u003cp\u003eYes\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"113\"\u003e\n\u003cp\u003eCCRT\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"76\"\u003e\n\u003cp\u003e9 months \u003csup\u003ec\u003c/sup\u003e\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd width=\"85\"\u003e\n\u003cp\u003ealive\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003e\u003cem\u003eF/U\u003c/em\u003e follow-up, \u003cem\u003eN/S\u003c/em\u003e not specified, \u003cem\u003eOKC\u003c/em\u003e odontogenic keratocyst, \u003cem\u003eFNAC\u003c/em\u003e fine needle aspiration cytology, \u003cem\u003ePIOC\u003c/em\u003e primary intraosseous carcinoma, \u003cem\u003eSND\u003c/em\u003e selective neck dissection, \u003cem\u003emRND\u003c/em\u003e modified radical neck dissection, \u003cem\u003eRT\u003c/em\u003e radiation therapy, \u003cem\u003eCCRT\u003c/em\u003e concurrent chemo-radiation therapy, \u003cem\u003eFFF\u003c/em\u003e fibular free flap\u003cbr /\u003e \u003csup\u003ea \u003c/sup\u003eat second recurrence\u003cbr /\u003e \u003csup\u003eb \u003c/sup\u003eat recurrence\u003cbr /\u003e \u003csup\u003ec \u003c/sup\u003erecurred but lost to follow-up.\u003c/p\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"odontogenic cyst, primary intraosseous carcinoma, pediatric mandible","lastPublishedDoi":"10.21203/rs.3.rs-195662/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-195662/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground:\u003c/strong\u003e Primary intraosseous carcinoma (PIOC) is a rare malignant odontogenic tumor that predominantly occurs in males older than 50 years. PIOC can be misdiagnosed as odontogenic cyst because it occasionally shows well-defined border on radiography. In this study, a case of a 14-year-old female with PIOC who was misdiagnosed with odontogenic cyst clinically is reported along with a literature review of pediatric PIOC cases.\u003c/p\u003e\u003cp\u003e\u003cstrong\u003eCase Presentation:\u003c/strong\u003e A 14-year-old female patient presented with painful swelling on the mandibular right premolar area. There was a radiolucent lesion with a well-defined border in panoramic view. She was diagnosed with odontogenic cyst, and the cystic mass was enucleated with extraction. However, the biopsy result was consistent with PIOC. After cancer work-up, she underwent partial mandibulectomy, selective neck dissection, and reconstruction with a fibular free flap. Although the surgical resection margins were clear, local recurrence and lung metastasis occurred four months after surgery. She underwent concurrent chemo-radiation therapy, but the prognosis was poor.\u003c/p\u003e\u003cp\u003e\u003cstrong\u003eConclusions:\u003c/strong\u003e PIOC should be diagnosed differentially from odontogenic cyst even in pediatric populations.\u003c/p\u003e","manuscriptTitle":"Primary Intraosseous Carcinoma in the Pediatric Mandible","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2021-02-09 19:17:45","doi":"10.21203/rs.3.rs-195662/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"f32620c8-dfde-4c11-8711-cde1dfdd1da0","owner":[],"postedDate":"February 9th, 2021","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[{"id":2259587,"name":"Surgery"},{"id":2259588,"name":"Oncology"}],"tags":[],"updatedAt":"2021-03-10T05:35:32+00:00","versionOfRecord":[],"versionCreatedAt":"2021-02-09 19:17:45","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-195662","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-195662","identity":"rs-195662","version":["v1"]},"buildId":"rHA-KDH7Qsr4HCuvH75dn","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}