Histological-pathological and clinical T stage of Primary Adenoid Cystic Carcinoma of the Lacrimal Gland in a Chinese population

Histological-pathological and clinical T stage of Primary Adenoid Cystic Carcinoma of the Lacrimal Gland in a Chinese population | 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 Research Article Histological-pathological and clinical T stage of Primary Adenoid Cystic Carcinoma of the Lacrimal Gland in a Chinese population Jiayi Wu, Hui Cui, Meiqin Liang, Feng Wang This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-7131396/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 12 Dec, 2025 Read the published version in BMC Cancer → Version 1 posted 10 You are reading this latest preprint version Abstract Background To present clinical presentations, histological-pathological patterns, clinical T stage, divergent treating methods, and outcomes of primary adenoid cystic carcinoma (ACC) of the lacrimal gland in a Chinese population. Methods This case series study enrolled patients with primary ACC of the lacrimal gland admitted to authors’ Hospital in China between 2003 and 2014. Results A total of 38 patients included 16 men and 22 women with a median age of 46.3 years were enrolled. Sixteen patients (42.11%) had local recurrence, while nineteen patients (50%) had distant metastasis at the time of presentation. Twelve patients (31.58%) were in T1-3 stage and twenty-six (68.42%) were in T4 stage. The median disease-free survival for the entire group was 30.4 months. Nineteen patients (73.08%) in T4 stage had a predominantly solid histological pattern, and only three (25%) in T1 ~ T3 stage had a predominantly solid histological pattern. Twelve patients (46.15%) in T4 stage underwent an exenteration with removal of the bone of the superior and lateral orbit and radiation therapy. Fourteen patients (53.85%) underwent globe-preserving apparent gross tumor resection with radiation therapy. The median disease-free survival time for the entire group was 30.4 months. The 5-year survival rate was 52.63%. Conclusions The solid pattern might be associated with more aggressive biologic tumor behaviors and this stage of adenoid cystic carcinoma of the lacrimal gland may be considered as an indicator of poor prognosis. lacrimal gland adenoid cystic carcinoma surgery pathology Figures Figure 1 Figure 2 INTRODUCTION Adenoid cystic carcinoma (ACC) is a rare form of adenocarcinoma and is the most common malignant epithelial tumor of the lacrimal gland which represents about 1.6% of all orbital tumors and 29% of all epithelial tumors of the lacrimal gland [ 1 ] . The tumor generally presents in either a solid pattern or cribiform pattern (more holey) with survival being longer in those with the cribiform pattern [ 2 ] . Overall mortality associated with lacrimal ACC tumors are reported up to 87% despite maximum treatment [ 3 – 5 ] . Lacrimal adenoid cystic carcinoma is an aggressive, slow-growing and painless tumor with a potential to invade into the surrounding intracranial tissues often with grave outcomes [ 6 – 7 ] . Patients may present with nebulous symptoms including asymmetric orbital pain, double vision, ptosis, and decreased visual acuity [ 8 ] . Because of its rarity and non-specific presentation, a diagnosis is often difficult and frequently initially incorrect which can lead to poorer outcomes [ 9 – 10 ] . Studies of lacrimal ACC often include a very limited number of patients. Clinically diagnosing lacrimal ACC is done via the seventh edition of the American Joint Committee on Cancer (AJCC) Staging Manual which defines T stage based upon tumor size and its potential extensions [ 11 – 12 ] . While controversy exists over the appropriate management for adenoid cystic carcinoma of the lacrimal gland, some clinicians advocate conservative eye-sparing surgical therapy (external-beam radiotherapy or proton-beam therapy) while others believe that radical exenteration is more appropriate [ 13 ] . This study aimed to evaluate the outcomes of therapy for ACC of the lacrimal gland with the clinical and histological features of Chinese patients from local medical institutions. SUBJECTS AND METHODS Study design and patients All procedures were performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. This case series study reviewed the medical records of patients with primary ACC of the lacrimal gland who were treated in Tianjin Eye Hospital in China between 2003 and 2014. Prior to study onset, approval for the study was obtained from the Ethics Committee-Institutional Review Board (HEC-IRB) of the respectives. Written informed consent was obtained from all the patients. Data collection The pathology slides were examined by one of us, without prior knowledge of the clinical patient data. Clinical variables collected from medical records included age, gender, size of the lesion, presenting symptoms (pain, proptosis, diplopia, decreased visual acuity), histological characteristics, methods of treatment, the presence of perineural invasion, the presence of adequate free margin in specimen, occurrence of local, regional, or distant metastasis, disease-free and overall survival time. Disease-free survival was defined as time from the completion of treatment, and overall survival was calculated from date of initial diagnosis. Histological patterns (cribriform, tubular, solid) were studied [ 14 ] . Tumor staging was based upon the AJCC classification system (AJCC Cancer Staging Manual , 7th edition) (Table 1 ) [ 15 ] . Table 1 T stage definitions for lacrimal gland tumors Primary tumor (T) TX Primary tumor cannot be assessed T0 No evidence of primary tumor T1 Tumor 2 cm or smaller in greatest dimension, with or without extraglandular extension into the orbital soft tissue T2 Tumor larger than 2 cm but not larger than 4 cm in greatest dimension T3 Tumor larger than 4 cm in greatest dimension T4 Tumor invades periosteum or orbital bone or adjacent structures T4a Tumor invades periosteum T4b Tumor invades orbital bone T4c Tumor invades adjacent structures (brain, sinus, pterygoidfossa, temporal fossa) Statistical analysis Statistical analysis was performed with SPSS 26.0 statistical software (IBM, Armonk, NY, USA). Age, disease-free survival time, and survival time after metastases was described by median value. Follow-up time and recurrence time were described by mean ± SD. Chi-square test, fisher’s exact test, and independent sample t test were used to compare the data. Kaplan-Meier test were used for survival analysis. All P ≤ 0.05 was construed as statistically significant. RESULTS This study included 22 females and 16 males with a median age of patients at diagnosis of 46.3 years (14 ~ 73 years). None of 38 patients had bilateral involvement. Twenty patients had left eye tumor involvement and 18 were right eye. The most common presenting symptoms was proptosis (78.94%), followed by pain (68.42%), diplopia (36.84%), and decreased visual acuity (34.21%) (Table 2 ). Table 2 Patient demographics and outcomes Characteristics and outcomes All patients (n = 38) Age a 46.3 (14–73) Sex c Female 22 (57.89%) Male 16 (42.11%) Affected side c Right 20 (52.63%) Left 18 (47.37%) Presenting symptoms c Proptosis 30 (78.94%) Pain 26 (68.42%) Diplopia 14 (36.84%) Decreased visual acuity 13 (34.21%) Clinical T stage c T1 3 (7.89%) T2 4 (10.52%) T3 5 (13.16%) T4 26 (68.42%) Histologic subtype c Cribriform 2 (5.26%) Basaloid 22 (57.89%) Tubular 9 (23.68%) Mixed 5 (13.16%) Treatment c R + RT 24 (63.15%) E + RT 14 (36.85%) Time (mo) of follow-up b 44.05 ± 26.22 Time (mo) of recurrence b 21.06 ± 11.02 Local recurrence c Yes 16 (42.11%) No 22 (57.89%) 5-year survival rate c Yes 20 (52.63%) No 18 (47.37%) Median disease-free survival time, total (mo) 30.4 Patients in T1 ~ T3 stage 51.42 Patients in T4 stage 20.76 Location of distant metastasis c Lung 10 (26.32%) Liver 5 (13.16%) Bone 4 (10.53%) Kidney 3 (7.89%) Brain 2 (5.26%) Lymph nodes 2 (5.26%) Total 19 (50%) Median survival time after metastases (mo) 6.83 R + RT, globe-preserving apparent gross tumor resection with postoperative radiation therapy; E + RT, exenteration with removal of the bone of the superior and lateral orbit and postoperative radiation therapy. a Continuous variables are described using the mean (minimum, maximum). b Continuous variables are described using the mean (± standard deviation). c Categorical variables are described using n (%). At the time of presentation, 3 cases (7.89%) were in T1 stage, 4 cases (10.53%) were in T2 stage, 5 cases (13.16%) were in T3 stage and 26 cases (68.42%) were in T4 stage. The median disease-free survival for patients in T4 stage was 20.76 months and for patients in T1-T3 stage was 51.42 months. A significant difference was found in disease-free survival between the patients in T1-T3 stage and T4 stage ( P < 0.05). At histopathological examination 22 patients (57.89%) presented with a basaloid pattern (Fig. 1 A and Table 2 ), 5 (13.16%) with a tubular pattern (Fig. 1 B and Table 2 ), 2 (5.26%) had a cribriform pattern (Fig. 1 C and Table 2 ), and 9 (23.68%) a mixed pattern (Fig. 1 D and Table 2 ), respectively. Furthermore, nineteen patients (76%) in T4 stage had a predominantly basaloid pattern ( Supplemental Table 1 ). The cribriform and tubular patterns tended to be skewed more frequently to the lower stage Bone invasion (Fig. 1 E) was clinically or pathologically apparent in 81%(30/38) of the tumors, and nerve invasion (Fig. 1 F) was seen in 52.6%(20/38) of the tumors. Surgical intervention included globe-preserving apparent gross tumor resection in 24 patients (63.16%), and exenteration with removal of the bone of the superior and lateral orbit in 14 (36.84%). Overall, all patients received postoperative radiation therapy (Table 2 ). Sixteen patients (42.11%) developed regional recurrence and nineteen patients (50%) had distant metastasis. Common sites of metastasis included lung (26.32%), followed by liver (13.16%), bone (10.53%), kidney (7.89%), brain (5.26%), and lymph nodes (5.26%). Twenty patients, staged T4, had local recurrence or distant metastasis, and 18 patients had a basaloid pattern. The median survival after diagnosis of metastatic disease was 6.83 months. The median disease-free survival time for the entire group was 30.4 months. The 5-year survival rate in this series was 52.63% (Table 2 and Fig. 2 ). Moreover, the median disease-free survival for patients in T4 who had undergone globe-preserving apparent gross tumor resection followed by postoperative radiation therapy were 20.71 months, and for patients who underwent exenteration with bone removal were 20.83 months which was not significantly ( P > 0.05) ( Supplemental Table 1 ). DISCUSSION In the current study, 76% patients in T4 stage had a predominantly basaloid histological pattern, and 15% in T1-3 stage. Meanwhile, 46.2% patients in T4 stage underwent an orbital exenteration with bone removal and radiation therapy, and 53.8% underwent globe-preserving gross tumor resection with radiation therapy. Disease-free survival in the T4 stage was not affected by the surgical therapy. Adenoid cystic carcinoma is the most common malignant tumor of the lacrimal gland and overall, the prognosis for this pathology very poor [ 6 ] . Lacrimal ACC’s peak incidence occurs in the fourth decade of life [ 16 ] with the mean age at diagnosis in this case series (46.3 years) being similar to most other reported series in adults. Some literature has suggested that the disease is more common in women [ 17 ] which comports with the ratio of female patients (57.89%) who had ACC tumors in the present study. Wright et al. [ 18 ] emphasized that pain is a frequently reported symptom of lacrimal gland malignancies given its perineural infiltration. In the current study, the most common presenting symptoms was proptosis (83.33%), followed by pain (52.63%) which is consistent with rates reported by Wright et al. In light of the poor prognosis of lacrimal gland ACC, it is suggested that a 40-year-old female patient presenting with symptoms of proptosis with a painful palpable mass in supratemporal quadrant of the orbit be regarded as having a malignant neoplasm until proven otherwise. Perineural invasion and the failure to obtain free margin in specimen also indicated higher risk of recurrence and mortality. Ahmad et al. [ 19 ] report that 93% of local recurrence had histological evidence of perineural invasion and 53% of local recurrence had failure to obtain free margin. In our study, 85.71%(18/21) of local recurrence had histological evidence of perineural invasion. Solid predominant subtype were associated with histological tumor-cell-free margin and absence of perineural invasion. In the patients without perineural invasion, mortality rate was 22.22%(4/18), lower than those with perineural invasion (12/20, 60%). However, due to the limitation in sample size and different disease severities, it was difficult to derive statistically significant conclusions from this study. Classically, ACC of the lacrimal gland consists of 3 subtypes histologically (cribriform, tubular, and solid), the most predominant and frequent of which is the basaloid pattern. The 7th edition AJCC Staging Manual has subdivided the T4 stage based upon the extent of tumor involvement from the periosteum, orbital bone, and adjacent structures. A new feature of the 7th edition of the AJCC Cancer Staging Manual attempted to extend the classification system beyond solely an anatomically based one. Histological data demonstrated a predominance of higher tumor grades which were associated with a primarily solid pattern. Combined, higher tumor grade and principally solid patterns were associated with shorter disease-free survival. ACC may rarely harbor areas of dedifferentiation, which may denote more aggressive potential [ 20 ] . In the case series, nineteen patients (76%) in the T4 group had a predominantly solid pattern. We speculate that the solid pattern has the tendency of extraluminally growth and carries with it the worst prognosis. Management of adenoid cystic carcinoma of the lacrimal gland remains controversial [ 21 ] . Some authorities advocate conservative surgical therapy followed by external-beam radiation therapy. Others believe that radical surgery may result in better long-term survival [ 22 ] . The present study suggested that the disease-free survival may not have been improved by radical surgery. The median disease-free survival for T4 patients who underwent globe-preserving apparent gross tumor resection was 20.71 months, and patients who underwent exenteration were 20.83 months. Thus, it seems unlikely that radical surgical therapy would have a beneficial effect on long-term survival. The type of radical surgery required in the treatment of ACC of the lacrimal gland often evokes negative emotions, especially in female patients may regard as experience. We posit that one of the effects of these intense negative reactions and moods was to shorten long-term survival amongst those patients. Adjuvant radiotherapy is recommended after surgical resection, regardless of its extent, especially for patients at high risk of recurrence [ 22 – 23 ] . Adjuvant radiation therapy has proven to be effective in preventing locoregional recurrences with local control rates of approximately 50–80% at 5 years, despite no benefit in overall survival [ 5 ] . In this study, all patients received postoperative radiation therapy. The major challenge in the treatment of adenoid cystic carcinoma of the lacrimal gland is prevention of systemic metastasis. Despite aggressive local treatment, adenoid cystic carcinoma of the lacrimal gland spawn a high rate of local recurrence and late distant metastasis, with significant resultant mortality [ 24 ] . Lung is the most common site of metastasis, with the liver being the second most common site. Of the patients reviewed for this study, only 2 had lymph node and brain metastasis. Some investigators have advocated the use of neutron beam therapy as a possibly more effective form of delivery of radiation therapy. All patients received postoperative radiation therapy in this study. The 5-year survival rates in this case series were 58%, which consistent with rates reported by Esmaeli et al. [ 10 ] who reported that the median disease-free survival was 18 months. While this case series showed higher median survival for patients than reported previously, and the median survival after diagnosis of metastatic disease lower. The current study, however, had some limitations, such as its retrospective observational nature and an unavoidable selection of patients. This was a double-center study which recruited only thirty-eight patients all of which were of Chinese background. Therefore, a larger, multicentric study across the country might be required to verify the results from current one. In conclusion, the solid pattern might associate with a more aggressive biologic tumor behavior and this stage of adenoid cystic carcinoma of the lacrimal gland might be considered as an indicator of poor prognosis. It was noted that many patients did not seek medical treatment until disease advanced. Thus, it is vital to educate patients to seek medical care early while treatment options are still available. Declarations Competing interests The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. Funding None Ethics approval and consent to participate All procedures were performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. This case series study reviewed the medical records of patients with primary ACC of the lacrimal gland who were treated in Changzhi People’s Hospital in China between 2003 and 2014. Prior to study onset, approval for the study was obtained from the Medical Ethics Review Committee of Changzhi People's Hospital of Changzhi Medical College. Written informed consent was obtained from all the patients. Consent for publication NA Data availability Availability of data and materials All original data generated or analyzed during the current study are available from the corresponding author upon reasonable request. Authors' contributions Jiayi Wu and Feng Wang carried out the studies, participated in collecting data, and drafted the manuscript. Jiayi Wu and Feng Wang performed the statistical analysis and participated in its design. Hui Cui and Meiqin Liang participated in acquisition, analysis, or interpretation of data and draft the manuscript. All authors read and approved the final manuscript. Acknowledgements All authors thank the patients who participated in this study. References Rapidis AD, et al. Adenoid cystic carcinoma of the head and neck. Clinicopathological analysis of 23 patients and review of the literature. Oral Oncol. 2005;41(3):328–35. https://doi.org/10.1016/j.oraloncology.2004.12.004 . Lee DA, et al. A clinicopathologic study of primary adenoid cystic carcinoma of the lacrimal gland. Ophthalmology. 1985;92(1):128–. https://doi.org/10.1016/s0161-6420(85)34081-2 . 34. El-Sawy T, et al. Prognostic accuracy of the seventh edition vs sixth edition of the American Joint Committee on Cancer tumor classification for adenoid cystic carcinoma of the lacrimal gland. 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Cancer. 2012;118(18):4444–51. https://doi.org/10.1002/cncr.27408 . Wright JE. Factors affecting the survival of patients with lacrimal gland tumours. Can J Ophthalmol. 1982;17(1):3–9. Ahmad SM, Esmaeli B, Williams M, Nguyen J, Fay A, Woog J, et al. American Joint Committee on Cancer classifcation predicts outcome of patients with lacrimal gland adenoid cystic carcinoma. Ophthalmology. 2009;116:1210–5. Seethala RR, et al. Adenoid cystic carcinoma with high-grade transformation: a report of 11 cases and a review of the literature. Am J Surg Pathol. 2007;31(11):1683–94. https://doi.org/10.1097/PAS.0b013e3180dc928c . Yang J, et al. Multimodal therapy in the management of lacrimal gland adenoid cystic carcinoma. BMC Ophthalmol. 2019;19(1):125. https://doi.org/10.1186/s12886-019-1110-5 . Han J, et al. Long-Term Outcomes of Eye-Sparing Surgery for Adenoid Cystic Carcinoma of Lacrimal Gland. Ophthalmic Plast Reconstr Surg. 2018;34(1):74–8. https://doi.org/10.1097/iop.0000000000000877 . 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Supplementary Files SupplementalTable1.docx Cite Share Download PDF Status: Published Journal Publication published 12 Dec, 2025 Read the published version in BMC Cancer → Version 1 posted Editorial decision: Revision requested 01 Sep, 2025 Reviews received at journal 31 Aug, 2025 Reviews received at journal 20 Aug, 2025 Reviewers agreed at journal 11 Aug, 2025 Reviewers agreed at journal 01 Aug, 2025 Reviewers invited by journal 28 Jul, 2025 Editor assigned by journal 24 Jul, 2025 Editor invited by journal 23 Jul, 2025 Submission checks completed at journal 23 Jul, 2025 First submitted to journal 23 Jul, 2025 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-7131396","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":492501950,"identity":"cfbe90ca-2a8f-442f-b06e-8f70bdb0fe9b","order_by":0,"name":"Jiayi Wu","email":"","orcid":"","institution":"Changzhi Medical College","correspondingAuthor":false,"prefix":"","firstName":"Jiayi","middleName":"","lastName":"Wu","suffix":""},{"id":492501951,"identity":"c43313c9-2170-4afc-aa98-d7945a98cc26","order_by":1,"name":"Hui Cui","email":"","orcid":"","institution":"Changzhi Medical College","correspondingAuthor":false,"prefix":"","firstName":"Hui","middleName":"","lastName":"Cui","suffix":""},{"id":492501952,"identity":"ea48ad47-f2b0-49df-9330-eb2efb05e924","order_by":2,"name":"Meiqin Liang","email":"","orcid":"","institution":"Changzhi People's Hospital","correspondingAuthor":false,"prefix":"","firstName":"Meiqin","middleName":"","lastName":"Liang","suffix":""},{"id":492501953,"identity":"9359d67d-1e4d-41f8-9ed7-e52537ad0d19","order_by":3,"name":"Feng Wang","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA90lEQVRIiWNgGAWjYLCCCiDmZ28+/uGDgY0ccVrOALFkz7E0xhkFacbEazG4kWPGzPPhcCJB1QbHzx5+caDijl3DjbS0xzYGzAkM7IePbsCr5UxemsWBM8+SG3seHzfOMWDLY+BJS7uBT4vZgRwz449th5OZ2dMSpHMMeIoZJHjM8Gs5/8bM4OC/w8lsDDkG0hYGEokNBLXcyDF+cLDhsB0PR46ZNIOBAWEt9jfemDEcOHY4QYLnWLJhj0GCMRshv0j25xh/OFBz2N7+ePPBBz/+/JfjZz98DK8WIGCTABKJDXAuAeUgwPwB5EAiFI6CUTAKRsFIBQBbtlR6FwTLswAAAABJRU5ErkJggg==","orcid":"","institution":"Changzhi People's Hospital","correspondingAuthor":true,"prefix":"","firstName":"Feng","middleName":"","lastName":"Wang","suffix":""}],"badges":[],"createdAt":"2025-07-15 14:08:07","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-7131396/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-7131396/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1186/s12885-025-15426-9","type":"published","date":"2025-12-12T15:59:12+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":88004420,"identity":"d62427f5-c4cd-4037-9180-e8e81ae990d5","added_by":"auto","created_at":"2025-07-31 10:36:38","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":2049242,"visible":true,"origin":"","legend":"\u003cp\u003eRepresentative images of adenoid cystic carcinoma with HE staining. A: basaloid pattern; B: tubular pattern; C: cribriform pattern; D: combination of tubular pattern and cribriform pattern. Original magnification was 100× except in D which was 50×. E: Orbital bone invaded by tumor cells. Original magnification was 40×; F: Perineural sheath of orbital nerves invaded by tumor cells. Original magnification was 40×.\u003c/p\u003e","description":"","filename":"floatimage1.png","url":"https://assets-eu.researchsquare.com/files/rs-7131396/v1/41cd9275920d629445c7af27.png"},{"id":88002746,"identity":"6ae0367f-20d1-4282-8382-d93b611e5c21","added_by":"auto","created_at":"2025-07-31 10:28:38","extension":"jpeg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":344898,"visible":true,"origin":"","legend":"\u003cp\u003eKaplan-Meier test showing overall survival of all 38 patients.\u003c/p\u003e","description":"","filename":"floatimage2.jpeg","url":"https://assets-eu.researchsquare.com/files/rs-7131396/v1/e925b3585047048a35352536.jpeg"},{"id":98245550,"identity":"2e358644-b734-4a0e-8610-415a652281f3","added_by":"auto","created_at":"2025-12-15 16:18:04","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":3556199,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-7131396/v1/86f61d01-5f06-471e-ba7f-095e920a500e.pdf"},{"id":88002738,"identity":"0231b2a7-89cc-416b-9ebc-51acc7a11e9d","added_by":"auto","created_at":"2025-07-31 10:28:38","extension":"docx","order_by":0,"title":"","display":"","copyAsset":false,"role":"supplement","size":27350,"visible":true,"origin":"","legend":"","description":"","filename":"SupplementalTable1.docx","url":"https://assets-eu.researchsquare.com/files/rs-7131396/v1/f72050bf62a55958c4db88be.docx"}],"financialInterests":"No competing interests reported.","formattedTitle":"Histological-pathological and clinical T stage of Primary Adenoid Cystic Carcinoma of the Lacrimal Gland in a Chinese population","fulltext":[{"header":"INTRODUCTION","content":"\u003cp\u003eAdenoid cystic carcinoma (ACC) is a rare form of adenocarcinoma and is the most common malignant epithelial tumor of the lacrimal gland which represents about 1.6% of all orbital tumors and 29% of all epithelial tumors of the lacrimal gland\u003csup\u003e[\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]\u003c/sup\u003e. The tumor generally presents in either a solid pattern or cribiform pattern (more holey) with survival being longer in those with the cribiform pattern\u003csup\u003e[\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]\u003c/sup\u003e. Overall mortality associated with lacrimal ACC tumors are reported up to 87% despite maximum treatment\u003csup\u003e[\u003cspan additionalcitationids=\"CR4\" citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]\u003c/sup\u003e. Lacrimal adenoid cystic carcinoma is an aggressive, slow-growing and painless tumor with a potential to invade into the surrounding intracranial tissues often with grave outcomes\u003csup\u003e[\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]\u003c/sup\u003e. Patients may present with nebulous symptoms including asymmetric orbital pain, double vision, ptosis, and decreased visual acuity\u003csup\u003e[\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]\u003c/sup\u003e. Because of its rarity and non-specific presentation, a diagnosis is often difficult and frequently initially incorrect which can lead to poorer outcomes\u003csup\u003e[\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e]\u003c/sup\u003e. Studies of lacrimal ACC often include a very limited number of patients. Clinically diagnosing lacrimal ACC is done via the seventh edition of the American Joint Committee on Cancer (AJCC) Staging Manual which defines T stage based upon tumor size and its potential extensions\u003csup\u003e[\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]\u003c/sup\u003e.\u003c/p\u003e\u003cp\u003eWhile controversy exists over the appropriate management for adenoid cystic carcinoma of the lacrimal gland, some clinicians advocate conservative eye-sparing surgical therapy (external-beam radiotherapy or proton-beam therapy) while others believe that radical exenteration is more appropriate\u003csup\u003e[\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e]\u003c/sup\u003e. This study aimed to evaluate the outcomes of therapy for ACC of the lacrimal gland with the clinical and histological features of Chinese patients from local medical institutions.\u003c/p\u003e"},{"header":"SUBJECTS AND METHODS","content":"\u003cp\u003e\u003cb\u003eStudy design and patients\u003c/b\u003e\u003c/p\u003e\u003cp\u003eAll procedures were performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. This case series study reviewed the medical records of patients with primary ACC of the lacrimal gland who were treated in Tianjin Eye Hospital in China between 2003 and 2014. Prior to study onset, approval for the study was obtained from the Ethics Committee-Institutional Review Board (HEC-IRB) of the respectives. Written informed consent was obtained from all the patients.\u003c/p\u003e\u003cp\u003e\u003cb\u003eData collection\u003c/b\u003e\u003c/p\u003e\u003cp\u003eThe pathology slides were examined by one of us, without prior knowledge of the clinical patient data. Clinical variables collected from medical records included age, gender, size of the lesion, presenting symptoms (pain, proptosis, diplopia, decreased visual acuity), histological characteristics, methods of treatment, the presence of perineural invasion, the presence of adequate free margin in specimen, occurrence of local, regional, or distant metastasis, disease-free and overall survival time. Disease-free survival was defined as time from the completion of treatment, and overall survival was calculated from date of initial diagnosis. Histological patterns (cribriform, tubular, solid) were studied\u003csup\u003e[\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e]\u003c/sup\u003e. Tumor staging was based upon the AJCC classification system \u003cem\u003e(AJCC Cancer Staging Manual\u003c/em\u003e, 7th edition) (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e)\u003csup\u003e[\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e]\u003c/sup\u003e.\u003c/p\u003e\u003cp\u003e\u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e\u003ccaption language=\"En\"\u003e\u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e\u003cdiv class=\"CaptionContent\"\u003e\u003cp\u003eT stage definitions for lacrimal gland tumors\u003c/p\u003e\u003c/div\u003e\u003c/caption\u003e\u003ccolgroup cols=\"2\"\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e\u003cthead\u003e\u003ctr\u003e\u003cth align=\"left\" colspan=\"2\" nameend=\"c2\" namest=\"c1\"\u003e\u003cp\u003ePrimary tumor (T)\u003c/p\u003e\u003c/th\u003e\u003c/tr\u003e\u003c/thead\u003e\u003ctbody\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eTX\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003ePrimary tumor cannot be assessed\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eT0\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eNo evidence of primary tumor\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eT1\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eTumor 2 cm or smaller in greatest dimension, with or without extraglandular extension into the orbital soft tissue\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eT2\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eTumor larger than 2 cm but not larger than 4 cm in greatest dimension\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eT3\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eTumor larger than 4 cm in greatest dimension\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eT4\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eTumor invades periosteum or orbital bone or adjacent structures\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eT4a\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eTumor invades periosteum\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eT4b\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eTumor invades orbital bone\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eT4c\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003eTumor invades adjacent structures (brain, sinus, pterygoidfossa, temporal fossa)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003c/tbody\u003e\u003c/colgroup\u003e\u003c/table\u003e\u003c/div\u003e\u003c/p\u003e\u003cdiv id=\"Sec2\" class=\"Section2\"\u003e\u003ch2\u003eStatistical analysis\u003c/h2\u003e\u003cp\u003eStatistical analysis was performed with SPSS 26.0 statistical software (IBM, Armonk, NY, USA). Age, disease-free survival time, and survival time after metastases was described by median value. Follow-up time and recurrence time were described by mean\u0026thinsp;\u0026plusmn;\u0026thinsp;SD. Chi-square test, fisher\u0026rsquo;s exact test, and independent sample t test were used to compare the data. Kaplan-Meier test were used for survival analysis. All \u003cem\u003eP\u003c/em\u003e\u0026thinsp;\u0026le;\u0026thinsp;0.05 was construed as statistically significant.\u003c/p\u003e\u003c/div\u003e"},{"header":"RESULTS","content":"\u003cp\u003eThis study included 22 females and 16 males with a median age of patients at diagnosis of 46.3 years (14\u0026thinsp;~\u0026thinsp;73 years). None of 38 patients had bilateral involvement. Twenty patients had left eye tumor involvement and 18 were right eye. The most common presenting symptoms was proptosis (78.94%), followed by pain (68.42%), diplopia (36.84%), and decreased visual acuity (34.21%) (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e\u003cp\u003e\u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab2\" border=\"1\"\u003e\u003ccaption language=\"En\"\u003e\u003cdiv class=\"CaptionNumber\"\u003eTable 2\u003c/div\u003e\u003cdiv class=\"CaptionContent\"\u003e\u003cp\u003ePatient demographics and outcomes\u003c/p\u003e\u003c/div\u003e\u003c/caption\u003e\u003ccolgroup cols=\"2\"\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e\u003cthead\u003e\u003ctr\u003e\u003cth align=\"left\" colname=\"c1\"\u003e\u003cp\u003eCharacteristics and outcomes\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c2\"\u003e\u003cp\u003eAll patients (n\u0026thinsp;=\u0026thinsp;38)\u003c/p\u003e\u003c/th\u003e\u003c/tr\u003e\u003c/thead\u003e\u003ctbody\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eAge \u003csup\u003ea\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e46.3 (14\u0026ndash;73)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eSex \u003csup\u003ec\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eFemale\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e22 (57.89%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eMale\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e16 (42.11%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eAffected side \u003csup\u003ec\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eRight\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e20 (52.63%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eLeft\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e18 (47.37%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePresenting symptoms \u003csup\u003ec\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eProptosis\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e30 (78.94%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePain\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e26 (68.42%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eDiplopia\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e14 (36.84%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eDecreased visual acuity\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e13 (34.21%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eClinical T stage \u003csup\u003ec\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eT1\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e3 (7.89%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eT2\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e4 (10.52%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eT3\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e5 (13.16%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eT4\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e26 (68.42%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eHistologic subtype \u003csup\u003ec\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eCribriform\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e2 (5.26%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eBasaloid\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e22 (57.89%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eTubular\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e9 (23.68%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eMixed\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e5 (13.16%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eTreatment \u003csup\u003ec\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eR\u0026thinsp;+\u0026thinsp;RT\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e24 (63.15%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eE\u0026thinsp;+\u0026thinsp;RT\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e14 (36.85%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eTime (mo) of follow-up \u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e44.05\u0026thinsp;\u0026plusmn;\u0026thinsp;26.22\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eTime (mo) of recurrence \u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e21.06\u0026thinsp;\u0026plusmn;\u0026thinsp;11.02\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eLocal recurrence \u003csup\u003ec\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eYes\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e16 (42.11%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eNo\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e22 (57.89%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003e5-year survival rate \u003csup\u003ec\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eYes\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e20 (52.63%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eNo\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e18 (47.37%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eMedian disease-free survival time, total (mo)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e30.4\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePatients in T1\u0026thinsp;~\u0026thinsp;T3 stage\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e51.42\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePatients in T4 stage\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e20.76\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eLocation of distant metastasis \u003csup\u003ec\u003c/sup\u003e\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eLung\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e10 (26.32%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eLiver\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e5 (13.16%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eBone\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e4 (10.53%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eKidney\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e3 (7.89%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eBrain\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e2 (5.26%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eLymph nodes\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e2 (5.26%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eTotal\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e19 (50%)\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eMedian survival time after metastases (mo)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e6.83\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003c/tbody\u003e\u003c/colgroup\u003e\u003ctfoot\u003e\u003ctr\u003e\u003ctd colspan=\"2\"\u003eR\u0026thinsp;+\u0026thinsp;RT, globe-preserving apparent gross tumor resection with postoperative radiation therapy; E\u0026thinsp;+\u0026thinsp;RT, exenteration with removal of the bone of the superior and lateral orbit and postoperative radiation therapy.\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd colspan=\"2\"\u003e\u003csup\u003ea\u003c/sup\u003e Continuous variables are described using the mean (minimum, maximum).\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd colspan=\"2\"\u003e\u003csup\u003eb\u003c/sup\u003e Continuous variables are described using the mean (\u0026plusmn;\u0026thinsp;standard deviation).\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd colspan=\"2\"\u003e\u003csup\u003ec\u003c/sup\u003e Categorical variables are described using n (%).\u003c/td\u003e\u003c/tr\u003e\u003c/tfoot\u003e\u003c/table\u003e\u003c/div\u003e\u003c/p\u003e\u003cp\u003eAt the time of presentation, 3 cases (7.89%) were in T1 stage, 4 cases (10.53%) were in T2 stage, 5 cases (13.16%) were in T3 stage and 26 cases (68.42%) were in T4 stage. The median disease-free survival for patients in T4 stage was 20.76 months and for patients in T1-T3 stage was 51.42 months. A significant difference was found in disease-free survival between the patients in T1-T3 stage and T4 stage (\u003cem\u003eP\u003c/em\u003e\u0026thinsp;\u0026lt;\u0026thinsp;0.05). At histopathological examination 22 patients (57.89%) presented with a basaloid pattern (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eA and Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e), 5 (13.16%) with a tubular pattern (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eB and Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e), 2 (5.26%) had a cribriform pattern (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eC and Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e), and 9 (23.68%) a mixed pattern (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eD and Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e), respectively. Furthermore, nineteen patients (76%) in T4 stage had a predominantly basaloid pattern (\u003cb\u003eSupplemental Table\u0026nbsp;1\u003c/b\u003e). The cribriform and tubular patterns tended to be skewed more frequently to the lower stage Bone invasion (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eE) was clinically or pathologically apparent in 81%(30/38) of the tumors, and nerve invasion (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eF) was seen in 52.6%(20/38) of the tumors.\u003c/p\u003e\u003cp\u003e\u003c/p\u003e\u003cp\u003eSurgical intervention included globe-preserving apparent gross tumor resection in 24 patients (63.16%), and exenteration with removal of the bone of the superior and lateral orbit in 14 (36.84%). Overall, all patients received postoperative radiation therapy (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e\u003cp\u003eSixteen patients (42.11%) developed regional recurrence and nineteen patients (50%) had distant metastasis. Common sites of metastasis included lung (26.32%), followed by liver (13.16%), bone (10.53%), kidney (7.89%), brain (5.26%), and lymph nodes (5.26%). Twenty patients, staged T4, had local recurrence or distant metastasis, and 18 patients had a basaloid pattern. The median survival after diagnosis of metastatic disease was 6.83 months. The median disease-free survival time for the entire group was 30.4 months. The 5-year survival rate in this series was 52.63% (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e and Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e). Moreover, the median disease-free survival for patients in T4 who had undergone globe-preserving apparent gross tumor resection followed by postoperative radiation therapy were 20.71 months, and for patients who underwent exenteration with bone removal were 20.83 months which was not significantly (\u003cem\u003eP\u003c/em\u003e\u0026thinsp;\u0026gt;\u0026thinsp;0.05) (\u003cb\u003eSupplemental Table\u0026nbsp;1\u003c/b\u003e).\u003c/p\u003e\u003cp\u003e\u003c/p\u003e"},{"header":"DISCUSSION","content":"\u003cp\u003eIn the current study, 76% patients in T4 stage had a predominantly basaloid histological pattern, and 15% in T1-3 stage. Meanwhile, 46.2% patients in T4 stage underwent an orbital exenteration with bone removal and radiation therapy, and 53.8% underwent globe-preserving gross tumor resection with radiation therapy. Disease-free survival in the T4 stage was not affected by the surgical therapy.\u003c/p\u003e\u003cp\u003eAdenoid cystic carcinoma is the most common malignant tumor of the lacrimal gland and overall, the prognosis for this pathology very poor\u003csup\u003e[\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]\u003c/sup\u003e. Lacrimal ACC\u0026rsquo;s peak incidence occurs in the fourth decade of life\u003csup\u003e[\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e]\u003c/sup\u003e with the mean age at diagnosis in this case series (46.3 years) being similar to most other reported series in adults. Some literature has suggested that the disease is more common in women\u003csup\u003e[\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e]\u003c/sup\u003e which comports with the ratio of female patients (57.89%) who had ACC tumors in the present study. Wright et al.\u003csup\u003e[\u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e]\u003c/sup\u003e emphasized that pain is a frequently reported symptom of lacrimal gland malignancies given its perineural infiltration. In the current study, the most common presenting symptoms was proptosis (83.33%), followed by pain (52.63%) which is consistent with rates reported by Wright et al. In light of the poor prognosis of lacrimal gland ACC, it is suggested that a 40-year-old female patient presenting with symptoms of proptosis with a painful palpable mass in supratemporal quadrant of the orbit be regarded as having a malignant neoplasm until proven otherwise. Perineural invasion and the failure to obtain free margin in specimen also indicated higher risk of recurrence and mortality. Ahmad et al.\u003csup\u003e[\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e]\u003c/sup\u003e report that 93% of local recurrence had histological evidence of perineural invasion and 53% of local recurrence had failure to obtain free margin. In our study, 85.71%(18/21) of local recurrence had histological evidence of perineural invasion. Solid predominant subtype were associated with histological tumor-cell-free margin and absence of perineural invasion. In the patients without perineural invasion, mortality rate was 22.22%(4/18), lower than those with perineural invasion (12/20, 60%). However, due to the limitation in sample size and different disease severities, it was difficult to derive statistically significant conclusions from this study.\u003c/p\u003e\u003cp\u003eClassically, ACC of the lacrimal gland consists of 3 subtypes histologically (cribriform, tubular, and solid), the most predominant and frequent of which is the basaloid pattern. The 7th edition AJCC Staging Manual has subdivided the T4 stage based upon the extent of tumor involvement from the periosteum, orbital bone, and adjacent structures. A new feature of the 7th edition of the \u003cem\u003eAJCC Cancer Staging Manual\u003c/em\u003e attempted to extend the classification system beyond solely an anatomically based one. Histological data demonstrated a predominance of higher tumor grades which were associated with a primarily solid pattern. Combined, higher tumor grade and principally solid patterns were associated with shorter disease-free survival. ACC may rarely harbor areas of dedifferentiation, which may denote more aggressive potential\u003csup\u003e[\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e]\u003c/sup\u003e. In the case series, nineteen patients (76%) in the T4 group had a predominantly solid pattern. We speculate that the solid pattern has the tendency of extraluminally growth and carries with it the worst prognosis.\u003c/p\u003e\u003cp\u003eManagement of adenoid cystic carcinoma of the lacrimal gland remains controversial\u003csup\u003e[\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e]\u003c/sup\u003e. Some authorities advocate conservative surgical therapy followed by external-beam radiation therapy. Others believe that radical surgery may result in better long-term survival\u003csup\u003e[\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e]\u003c/sup\u003e. The present study suggested that the disease-free survival may not have been improved by radical surgery. The median disease-free survival for T4 patients who underwent globe-preserving apparent gross tumor resection was 20.71 months, and patients who underwent exenteration were 20.83 months. Thus, it seems unlikely that radical surgical therapy would have a beneficial effect on long-term survival. The type of radical surgery required in the treatment of ACC of the lacrimal gland often evokes negative emotions, especially in female patients may regard as experience. We posit that one of the effects of these intense negative reactions and moods was to shorten long-term survival amongst those patients. Adjuvant radiotherapy is recommended after surgical resection, regardless of its extent, especially for patients at high risk of recurrence\u003csup\u003e[\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e]\u003c/sup\u003e. Adjuvant radiation therapy has proven to be effective in preventing locoregional recurrences with local control rates of approximately 50\u0026ndash;80% at 5 years, despite no benefit in overall survival\u003csup\u003e[\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]\u003c/sup\u003e. In this study, all patients received postoperative radiation therapy.\u003c/p\u003e\u003cp\u003eThe major challenge in the treatment of adenoid cystic carcinoma of the lacrimal gland is prevention of systemic metastasis. Despite aggressive local treatment, adenoid cystic carcinoma of the lacrimal gland spawn a high rate of local recurrence and late distant metastasis, with significant resultant mortality\u003csup\u003e[\u003cspan citationid=\"CR24\" class=\"CitationRef\"\u003e24\u003c/span\u003e]\u003c/sup\u003e. Lung is the most common site of metastasis, with the liver being the second most common site. Of the patients reviewed for this study, only 2 had lymph node and brain metastasis. Some investigators have advocated the use of neutron beam therapy as a possibly more effective form of delivery of radiation therapy. All patients received postoperative radiation therapy in this study. The 5-year survival rates in this case series were 58%, which consistent with rates reported by Esmaeli et al.\u003csup\u003e[\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e]\u003c/sup\u003e who reported that the median disease-free survival was 18 months. While this case series showed higher median survival for patients than reported previously, and the median survival after diagnosis of metastatic disease lower.\u003c/p\u003e\u003cp\u003eThe current study, however, had some limitations, such as its retrospective observational nature and an unavoidable selection of patients. This was a double-center study which recruited only thirty-eight patients all of which were of Chinese background. Therefore, a larger, multicentric study across the country might be required to verify the results from current one.\u003c/p\u003e\u003cp\u003eIn conclusion, the solid pattern might associate with a more aggressive biologic tumor behavior and this stage of adenoid cystic carcinoma of the lacrimal gland might be considered as an indicator of poor prognosis. It was noted that many patients did not seek medical treatment until disease advanced. Thus, it is vital to educate patients to seek medical care early while treatment options are still available.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNone\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll procedures were performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. This case series study reviewed the medical records of patients with primary ACC of the lacrimal gland who were treated in Changzhi People\u0026rsquo;s Hospital in China between 2003 and 2014. Prior to study onset, approval for the study was obtained from the Medical Ethics Review Committee of Changzhi People\u0026apos;s Hospital of Changzhi Medical College. Written informed consent was obtained from all the patients.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNA\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eData availability\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll original data generated or analyzed during the current study are available from the corresponding author upon reasonable request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors\u0026apos; contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eJiayi Wu and Feng Wang carried out the studies, participated in collecting data, and drafted the manuscript. Jiayi Wu and Feng Wang performed the statistical analysis and participated in its design. Hui Cui and Meiqin Liang participated in acquisition, analysis, or interpretation of data and draft the manuscript. All authors read and approved the final manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcknowledgements\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll authors thank the patients who participated in this study.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eRapidis AD, et al. Adenoid cystic carcinoma of the head and neck. Clinicopathological analysis of 23 patients and review of the literature. Oral Oncol. 2005;41(3):328\u0026ndash;35. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1016/j.oraloncology.2004.12.004\u003c/span\u003e\u003cspan address=\"10.1016/j.oraloncology.2004.12.004\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eLee DA, et al. A clinicopathologic study of primary adenoid cystic carcinoma of the lacrimal gland. 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Ophthalmic Plast Reconstr Surg. 2016;32(1):1\u0026ndash;10. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1097/iop.0000000000000531\u003c/span\u003e\u003cspan address=\"10.1097/iop.0000000000000531\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"bmc-cancer","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"bcan","sideBox":"Learn more about [BMC Cancer](http://bmccancer.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/bcan/default.aspx","title":"BMC Cancer","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"lacrimal gland, adenoid cystic carcinoma, surgery, pathology","lastPublishedDoi":"10.21203/rs.3.rs-7131396/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-7131396/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eBackground\u003c/h2\u003e\u003cp\u003eTo present clinical presentations, histological-pathological patterns, clinical T stage, divergent treating methods, and outcomes of primary adenoid cystic carcinoma (ACC) of the lacrimal gland in a Chinese population.\u003c/p\u003e\u003ch2\u003eMethods\u003c/h2\u003e\u003cp\u003eThis case series study enrolled patients with primary ACC of the lacrimal gland admitted to authors\u0026rsquo; Hospital in China between 2003 and 2014.\u003c/p\u003e\u003ch2\u003eResults\u003c/h2\u003e\u003cp\u003eA total of 38 patients included 16 men and 22 women with a median age of 46.3 years were enrolled. Sixteen patients (42.11%) had local recurrence, while nineteen patients (50%) had distant metastasis at the time of presentation. Twelve patients (31.58%) were in T1-3 stage and twenty-six (68.42%) were in T4 stage. The median disease-free survival for the entire group was 30.4 months. Nineteen patients (73.08%) in T4 stage had a predominantly solid histological pattern, and only three (25%) in T1\u0026thinsp;~\u0026thinsp;T3 stage had a predominantly solid histological pattern. Twelve patients (46.15%) in T4 stage underwent an exenteration with removal of the bone of the superior and lateral orbit and radiation therapy. Fourteen patients (53.85%) underwent globe-preserving apparent gross tumor resection with radiation therapy. The median disease-free survival time for the entire group was 30.4 months. The 5-year survival rate was 52.63%.\u003c/p\u003e\u003ch2\u003eConclusions\u003c/h2\u003e\u003cp\u003eThe solid pattern might be associated with more aggressive biologic tumor behaviors and this stage of adenoid cystic carcinoma of the lacrimal gland may be considered as an indicator of poor prognosis.\u003c/p\u003e","manuscriptTitle":"Histological-pathological and clinical T stage of Primary Adenoid Cystic Carcinoma of the Lacrimal Gland in a Chinese population","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-07-31 10:28:33","doi":"10.21203/rs.3.rs-7131396/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2025-09-01T19:40:53+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-08-31T13:40:42+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-08-20T12:07:49+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"269884702933017285994654446490043346075","date":"2025-08-11T22:55:37+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"190278090053838774644992986869787424388","date":"2025-08-02T01:43:52+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2025-07-28T17:06:59+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2025-07-24T17:07:05+00:00","index":"","fulltext":""},{"type":"editorInvited","content":"","date":"2025-07-23T16:23:08+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2025-07-23T14:38:17+00:00","index":"","fulltext":""},{"type":"submitted","content":"BMC Cancer","date":"2025-07-23T14:35:42+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"bmc-cancer","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"bcan","sideBox":"Learn more about [BMC Cancer](http://bmccancer.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/bcan/default.aspx","title":"BMC Cancer","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"5ce0bebe-2d40-45a3-b59d-bc15db9b1dec","owner":[],"postedDate":"July 31st, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2025-12-15T16:15:03+00:00","versionOfRecord":{"articleIdentity":"rs-7131396","link":"https://doi.org/10.1186/s12885-025-15426-9","journal":{"identity":"bmc-cancer","isVorOnly":false,"title":"BMC Cancer"},"publishedOn":"2025-12-12 15:59:12","publishedOnDateReadable":"December 12th, 2025"},"versionCreatedAt":"2025-07-31 10:28:33","video":"","vorDoi":"10.1186/s12885-025-15426-9","vorDoiUrl":"https://doi.org/10.1186/s12885-025-15426-9","workflowStages":[]},"version":"v1","identity":"rs-7131396","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-7131396","identity":"rs-7131396","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}