Malignant yolk sac tumor of the ovary presenting as adnexal torsion in a child: A rare case report with review of literature

Malignant yolk sac tumor of the ovary presenting as adnexal torsion in a child: A rare case report with review of literature | 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 Malignant yolk sac tumor of the ovary presenting as adnexal torsion in a child: A rare case report with review of literature Kabhilan A K, Ebinesh Arulnathan, Karunakaran N, Padma S, Praislin Gideon This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-6546441/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 Introduction: Malignant yolk sac tumor is a rare ovarian neoplasm with peak incidence in young women. Its occurrence in children less than 10 years old is extremely uncommon. In this manuscript we report a rare case of malignant yolk sac tumor presenting with adnexal torsion, emphasizing the role of multimodality imaging in management. Case Presentation: A 9-year-old female child was brought with the complaints of acute right lower quadrant pain, nausea and vomiting. On examination, the abdomen was tender with guarding. Laboratory investigations revealed leukocytosis, raised inflammatory markers and markedly elevated alpha-fetoprotein (AFP) levels. Ultrasonography revealed a mixed echogenic tumor in the right adnexa with features of adnexal torsion. Cross sectional imaging confirmed the presence of a heterogeneously enhancing tumor in the right adnexa with pelvic and paraaortic lymphadenopathy. Subsequently, the child underwent emergency laparotomy for adnexal detorsion with tumor excision. Postoperative histopathological examination confirmed malignant yolk sac tumor. Conclusions This case underscores the rare presentation of ovarian malignant yolk sac tumor as adnexal torsion in a child and the role of multimodality imaging in its management. Malignant Yolk Sac Tumor Ovarian Germ Cell Tumor Adnexal Torsion Pediatric Ovarian Tumors Multimodality Imaging Figures Figure 1 Figure 2 Figure 3 Figure 4 Introduction Ovarian germ cell tumors arise from the germ cells of the ovary and account for ~ 15-20% of all ovarian neoplasms (1). Malignant germ cell tumors of the ovary are rare with an incidence of ~ 1-2% (2). Yolk sac tumors, also known as endodermal sinus tumors, are highly aggressive with a predilection for gonadal sites (3). The gonads, particularly the ovaries, are the primary sites for these tumors. However, extragonadal sites like broad ligament, retroperitoneum, mediastinum and sacrococcygeal region have also been reported (3-5) . These tumors are usually seen in young women and adolescent girls. Occurrence in children less than 10 years of age is extremely uncommon with a few sporadic case reports. Unlike epithelial tumors, malignant yolk sac tumors are locally aggressive and possess high metastatic potential. Due to their aggressive nature, early detection and management are critical for improving survival outcomes This highlights the rare presentation of malignant yolk sac tumor complicated by adnexal torsion, emphasizing the role of multimodality imaging in timely management. Case Presentation A 9-year-old girl child was brought to the emergency department with acute onset, severe right lower abdominal pain, nausea and few episodes of non-bilious vomiting. No history of preceding trauma, fever, or changes in urinary or bowel habits could be elicited. She had no prior abdominal surgeries or known health issues. On physical examination, the patient appeared visibly distressed and was clutching her abdomen. She was afebrile with stable vital signs. Abdominal examination revealed marked tenderness in the right lower quadrant with guarding. Laboratory investigations showed a leucocytosis (white blood cell count of 18,600/µL) and elevated inflammatory markers (ESR 32 mm/hr). Notably, alpha-fetoprotein (AFP) level was markedly elevated at 32,134 ng/mL, strongly suggestive of a malignant germ cell tumor. Lactate Dehydrogenase (LDH) was also elevated at 635 IU/L. Levels of other tumor markers were within normal limits (Human Chorionic Gonadotropin (HCG)- 0.74 ng/ml; CA-125- 14.6 U/ml; CA-19.9 <0.8 U/ml; Carcinoembryonic Antigen (CEA)- 1.52 ng/ml). Imaging Findings Pelvic ultrasound revealed a large, heterogeneous, solid-cystic mass measuring 8 x 7.5cm in the right adnexa. The mass exhibited mixed echogenicity, with areas of necrosis (Figure 1). Colour Doppler imaging demonstrated no vascularity within the lesion, suspicious of adnexal torsion. Free fluid was noted in the pelvis, raising further concern of a possible adnexal torsion. Few enlarged para aortic lymph nodes also noted. Contrast enhanced computed tomography (CECT) confirmed the presence of a lobulated heterogeneously enhancing solid pelvic mass measuring approximately 8.0 x 8.0 cm in the right adnexa (Figure 2). It was seen compressing the uterus, displacing it towards the left, and causing a significant mass effect on the urinary bladder and the right ureter, resulting in moderate right-sided hydroureteronephrosis. Minimal ascites was present. Multiple enlarged heterogeneously enhancing para-aortic and pelvic lymph nodes were identified, largest measuring up to 2.5 cm in diameter, consistent with nodal metastasis. No evidence of distant organ involvement was observed. Magnetic resonance imaging (MRI) was subsequently performed for further characterization. It revealed a lobulated solid lesion in the right hemipelvis, appearing isointense on T1 and heterogeneously hyperintense on T2 with areas of restricted diffusion (FIgure 3). Right ovary could not be visualized separately from the lesion. Uterus was displaced to the left. The left ovary was visualized distinctly and measured 1.5 x 1.0 cm. Minimal hemoperitoneum was noted. It was also compressing the urinary bladder and the right ureter, causing moderate hydroureteronephrosis. Additionally, multiple enlarged heterogeneous pelvic and paraaortic lymph nodes were noted. Considering the radiological features, malignant ovarian germ cell tumor with multiple metastatic lymph nodes was considered. In view of acute presentation, absent vascularity on colour Doppler and presence of intraperitoneal hemorrhagic fluid, possibility of associated adnexal torsion was also considered. Management Considering the possibility of adnexal torsion, emergency laparotomy was performed. Intraoperatively, a solid right adnexal mass with necrotic lymphadenopathy was observed, complicated by twisted vascular pedicles. Right salpingo-oopherectomy with lymph node dissection was performed. Histopathological examination: Histopathological examination (Figure 4) revealed a tumor composed of cells arranged in microcystic, festoon, and glandular patterns. The tumor cells exhibited nuclear pleomorphism and atypia. Hyaline globules were observed, along with many areas showing Schiller-Duval body formation. Extensive areas of hemorrhage and necrosis were noted. Dense inflammatory infiltrate composed of neutrophils was present, and brisk mitotic activity was observed. The ovarian capsule was ruptured. The fallopian tubes were also infiltrated by tumor cells. The tumor cells showed Periodic Acid Schiff (PAS)- positive hyaline globules and strong immunoreactivity to Alpha-FetoProtein (AFP) and Sal-like protein 4 (SALL4). These findings were diagnostic of malignant yolk sac tumor. Para aortic and Pelvic lymph nodes showed evidence of metastatic involvement. Discussion One of the most common causes of Right lower quadrant (RLQ) pain in children is acute appendicitis, which typically presents with periumbilical pain migrating to the RLQ, accompanied by fever, anorexia, and nausea.( 6 ). Ultrasonography is often the first-line imaging modality, with sensitivity ranging from 75–90% ( 7 ). Another consideration is mesenteric adenitis, which mimics appendicitis but often follows a viral upper respiratory infection. It is more common in younger children and is usually self-limiting ( 8 ). Other important causes include intussusception, which presents with intermittent abdominal pain and bloody stools ("red currant jelly stool"). It is most common in infants and toddlers and requires emergent ultrasound or air enema for diagnosis and treatment In female children, especially adolescents, gynecologic causes must be considered. Ovarian torsion is a surgical emergency and typically presents with sudden onset of severe RLQ pain, often associated with nausea and vomiting. Ultrasound with Doppler is the diagnostic imaging modality of choice, though surgical exploration may be required in equivocal cases ( 9 ). Another important consideration is ovarian cyst rupture, which may cause acute RLQ pain and free fluid visible on imaging. Additionally, Mittelschmerz, or mid-cycle ovulation pain, may present as self-limited RLQ pain in pubescent girls ( 10 ).For sexually active adolescents, pelvic inflammatory disease (PID) and ectopic pregnancy are critical to rule out. A urine or serum pregnancy test is essential in this population to exclude ectopic pregnancy, even if the patient denies sexual activity ( 11 ).Urinary tract infections (UTIs) and pyelonephritis are common in girls and may present with RLQ pain, dysuria, fever, or flank pain. A urinalysis and urine culture are critical in evaluating these conditions. Kidney stones can also present with RLQ pain radiating to the groin, though they are less common in children( 12 ). Radiological imaging plays a critical role in the evaluation of adnexal lesions in female children, guiding both diagnosis and management. Ultrasound (US) is the first-line imaging modality due to its non-invasive nature, lack of ionizing radiation, and high sensitivity in characterizing adnexal masses. Gray-scale ultrasound can identify simple cysts, complex cysts, or solid masses, while Doppler imaging assesses vascular flow to detect conditions such as ovarian torsion, which presents with reduced or absent blood flow to the affected ovary ( 13 ). Functional ovarian cysts, the most common adnexal lesions in children, typically appear as simple, thin-walled, anechoic cysts, whereas neoplastic lesions may have mixed echogenicity or solid components. Color Doppler studies integrated with ultrasound can enhance diagnostic accuracy in emergencies like ovarian torsion, as normal vascular flow on Doppler can help rule out torsion with high sensitivity. For complex or indeterminate masses, magnetic resonance imaging (MRI) is preferred for further evaluation due to its superior soft-tissue resolution and ability to characterize the lesion’s origin and composition (e.g., fat, fluid, or solid). MRI is particularly helpful in distinguishing benign from malignant lesions, such as differentiating mature teratomas from malignant germ cell tumors ( 14 ).While computed tomography (CT) is not routinely used in pediatric adnexal lesions due to radiation exposure, it may be employed in cases where malignancy is suspected or for staging purposes, particularly in advanced tumors. CT is also useful in identifying metastatic spread or assessing adjacent organ involvement ( 13 ). In summary, a tailored approach combining ultrasound, MRI, and occasionally CT allows clinicians to effectively evaluate adnexal lesions in children while minimizing risks. Ovarian germ cell tumors (OGCTs) are the most common ovarian neoplasms in children, accounting for 60–80% of all pediatric ovarian neoplasms ( 15 ). Unlike in adults, the majority of pediatric ovarian germ cell tumors are malignant ( 16 , 17 ). Germ cell tumors include teratoma (mature and immature), dysgerminoma, yolk sac tumor, embryonal carcinoma and mixed germ cell tumors. These tumors arise from primitive germ cells and include both benign and malignant types. Benign tumors, such as mature teratomas (dermoid cysts) typically consist of mature tissues like skin, hair, and fat. These tumors are often asymptomatic unless complications like torsion or rupture occur. Malignant OGCTs include dysgerminomas, yolk sac tumors, immature teratomas, and mixed germ cell tumors. Dysgerminomas, the most common malignant subtype, are solid and radiosensitive, while yolk sac tumors are aggressive and characterized by elevated alpha-fetoprotein (AFP) levels. Immature teratomas contain immature neural or mesenchymal tissue with malignant potential, and mixed germ cell tumors feature elements of multiple subtype ( 18 ). Serum tumor markers play a pivotal role in the diagnosis, monitoring, and follow-up of ovarian tumors, particularly in pediatric and adolescent patients.. Among ovarian germ cell tumors (OGCTs), alpha-fetoprotein (AFP) is the most widely used marker and is elevated in yolk sac tumors (endodermal sinus tumors). AFP levels are both diagnostic and prognostic, helping to monitor treatment response and detect recurrences. Beta-human chorionic gonadotropin (β-hCG) is another crucial marker, often elevated in choriocarcinomas and some mixed germ cell tumors. Dysgerminomas, the most common malignant OGCT, are associated with elevated lactate dehydrogenase (LDH) levels, reflecting their high metabolic activity. For epithelial ovarian tumors, which are rare in children, markers such as CA-125 may be elevated, though it is more commonly used in adults. Inhibin and estradiol may be elevated in sex cord-stromal tumors like granulosa cell tumors, which are rare but hormonally active, potentially causing precocious puberty. The utility of tumor markers extends beyond diagnosis to guiding management. Preoperative levels of AFP, β-hCG, and LDH can provide insights into the likelihood of malignancy and the tumor subtype, assisting in surgical planning. Postoperative monitoring of these markers is essential to evaluate treatment response and identify early recurrences. Despite their utility, serum tumor markers must be interpreted alongside clinical findings and imaging, as false positives can occur in certain physiological or pathological conditions ( 19 ). The treatment of germ cell tumors (GCTs) in children involves a multidisciplinary approach that focuses on achieving optimal oncological outcomes while preserving fertility and minimizing treatment-related toxicities. The primary modality for both benign and malignant GCTs is surgical resection. For benign ovarian GCTs, such as mature teratomas, fertility-sparing surgery is the cornerstone of treatment. The goal is to remove the tumor while preserving the unaffected ovary and uterus. Malignant germ cell tumors, such as dysgerminomas, yolk sac tumors, and immature teratomas, require more extensive surgical excision, often accompanied by lymph node evaluation and staging procedures. However, fertility preservation remains a critical consideration during these surgeries ( 20 ). In cases of malignant GCTs, adjuvant chemotherapy is a crucial component of treatment, particularly for advanced-stage or recurrent disease. Platinum-based chemotherapy regimens, most commonly consisting of bleomycin, etoposide, and cisplatin (BEP), are highly effective and have significantly improved survival rates in pediatric patients. For localized dysgerminomas, surgery alone may be sufficient, as these tumors are highly radiosensitive. However, chemotherapy is preferred for advanced-stage disease or for tumors like yolk sac tumors that exhibit aggressive behavior ( 21 ). Radiotherapy is used sparingly in children to avoid long-term complications, such as infertility or secondary malignancies, and is generally reserved for specific cases of dysgerminomas. Treatment response is monitored using serum tumor markers such as alpha-fetoprotein (AFP), beta-human chorionic gonadotropin (β-hCG), and lactate dehydrogenase (LDH). Declining marker levels post-treatment indicate effective tumor control, while persistent elevation suggests residual disease or recurrence. Long-term follow-up is essential to monitor for late effects of chemotherapy, including nephrotoxicity, ototoxicity, and secondary malignancies, as well as to assess fertility outcomes. With the advent of modern multimodal treatments, the prognosis for pediatric GCTs is excellent, with 5-year survival rates exceeding 90% for early-stage disease and around 70–80% for advanced disease ( 22 ). Conclusions This case report highlights the role of imaging in preoperative evaluation, diagnosis and management of ovarian tumors in children. Multimodality imaging involving ultrasonography, computed tomography and magnetic resonance imaging is indispensable in characterizing pediatric ovarian tumors and evaluation of associated complications like tumor rupture, torsion and metastasis. Abbreviations AFP - Alpha-Fetoprotein LDH - Lactate Dehydrogenase HCG - Human Chorionic Gonadotropin CA-125 - Cancer Antigen 125 CA-19.9 - Cancer Antigen 19.9 CEA - Carcinoembryonic Antigen CECT - Contrast Enhanced Computed Tomography MRI - Magnetic Resonance Imaging US - Ultrasonography RLQ - Right Lower Quadrant BEP - Bleomycin, Etoposide, and Cisplatin (Chemotherapy regimen) OGCT - Ovarian Germ Cell Tumors GCT - Germ Cell Tumors CT - Computed Tomography DWI - Diffusion Weighted Imaging ADC - Apparent Diffusion Coefficient PID - Pelvic Inflammatory Disease UTI - Urinary Tract Infection ESR - Erythrocyte Sedimentation Rate PAS - Periodic Acid-Schiff SALL4 - Spalt-Like Transcription Factor 4 Declarations Funding information The author(s) received no financial support for the research, authorship, and/or publication of this article. Competing interests The author(s) declare that they have no financial or personal relationship(s) that may have inappropriately influenced them in writing this article. Ethical considerations All procedures performed in this study involving a human participant were in accordance with the ethical standards of the institutional ethical committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Ethics and Consent for p articipation: Not Applicable Consent for publication Informed consent was obtained from the patient's parents to publish the case and use their radiological images involved in the preparation of this manuscript. Data availability Data sharing is not applicable to this research article as no new data were created or analysed in this study. Code Availability The data supporting the findings of this case report are available within the manuscript. No custom code was developed. Any software used for image processing is cited appropriately in the references. Authors’ contributions KAK: study of concept and design, literature research, manuscript preparation, manuscript editing EA: guarantor of integrity of the entire study, study of concept and design, literature research, manuscript preparation, manuscript editing KN: study of concept and design, literature research, manuscript editing PS: study of concept and design, literature research, manuscript editing PG: literature research, manuscript preparation, manuscript editing References Kojimahara T, Nakahara K, Takano T, Yaegashi N, Nishiyama H, Fujimori K, et al. Yolk sac tumor of the ovary: a retrospective multicenter study of 33 Japanese women by Tohoku Gynecologic Cancer Unit (TGCU). Tohoku J Exp Med. 2013; 230: 211–7. Dallenbach P, Bonnefoi H, Pelte MF, Vlastos G. Yolk sac tumours of the ovary: an update. Eur J Surg Oncol. 2006; 32:1063–75. Farah RA, Matta J K, Metri AA, Bejjani- Doueihy NA, Saikali IC. Extragonadal germ cell tumor presenting with spinal cord compression: a case report and literature review. Ann Pediatr Surg. 2022; 18, 36. Gao Y, Jiang J, Liu Q. Extragonadal malignant germ cell tumors: A clinicopathological and immunohistochemical analysis of 48 cases at a single Chinese institution. Int J Clin Exp Pathol. 2015; 8: 5650-7. Rudaitis V, Mickys U, Katinaitė J, Dulko J. Successful treatment of advanced stage yolk sac tumour of extragonadal origin: a case report and review of literature. Acta Med Litu. 2016; 23: 110-6. Lotfollahzadeh S, Lopez RA, Deppen JG. Appendicitis. [Updated 2024 Feb 12]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan. Azizaddini S, Mani N. Liver Imaging. [Updated 2023 Feb 20]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan. Otto M, Nagalli S. Mesenteric Adenitis. [Updated 2023 Aug 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan. Poonai N, Poonai C, Lim R, Lynch T. Pediatric ovarian torsion: case series and review of the literature. Can J Surg. 2013; 56: 103-8. Mobeen S, Apostol R. Ovarian Cyst. [Updated 2023 Jun 5]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan. Tay JI, Moore J, Walker JJ. Ectopic pregnancy. BMJ. 2000; 320: 916-9. Car J. Urinary tract infections in women: diagnosis and management in primary care. BMJ. 2006; 332: 94-7. Iyer VR, Lee SI. MRI, CT, and PET/CT for ovarian cancer detection and adnexal lesion characterization. AJR Am J Roentgenol . 2010; 194: 311–21. Zhang J, Li Y, Zhao Y, Qiao J. CT and MRI of superficial solid tumors. Quant Imaging Med Surg. 2018; 8: 232-251. Heo SH, Kim JW, Shin SS, Jeong SI, Lim HS, Choi YD et al. Review of ovarian tumors in children and adolescents: radiologic-pathologic correlation. Radiographics. 2014; 34: 2039–55. Cass DL, Hawkins E, Brandt ML, Chintagumpala M, Bloss RS, Milewicz AL, et al. Surgery for ovarian masses in infants, children, and adolescents: 102 consecutive patients treated in a 15-year period. J Pediatr Surg. 2001; 36: 693-9. Schultz KA, Sencer SF, Messinger Y, Neglia JP, Steiner ME. Pediatric ovarian tumors: a review of 67 cases. Pediatr Blood Cancer. 2005; 44: 167-73. Gică N, Peltecu G, Chirculescu R, Gică C, Stoicea MC, Serbanica AN, Panaitescu AM. Ovarian Germ Cell Tumors: Pictorial Essay. Diagnostics (Basel). 2022; 12: 2050. Jezierska M, Gawrychowska A, Stefanowicz J. Diagnostic, Prognostic and Predictive Markers in Pediatric Germ Cell Tumors-Past, Present and Future. Diagnostics (Basel). 2022; 12: 278. Bhuta R, Shah R, Gell JJ, Poynter JN, Bagrodia A, Dicken BJ et al. Children's Oncology Group's 2023 blueprint for research: Germ cell tumors. Pediatr Blood Cancer. 2023; 70 Suppl 6: e30562. De Giorgi U, Casadei C, Bergamini A, Attademo L, Cormio G, Lorusso D et al. Therapeutic Challenges for Cisplatin-Resistant Ovarian Germ Cell Tumors. Cancers (Basel). 2019; 11: 1584. Dieckmann KP, Simonsen-Richter H, Kulejewski M, Anheuser P, Zecha H, Isbarn H, Pichlmeier U. Serum Tumour Markers in Testicular Germ Cell Tumours: Frequencies of Elevated Levels and Extents of Marker Elevation Are Significantly Associated with Clinical Parameters and with Response to Treatment. Biomed Res Int. 2019; 2019: 5030349. Additional Declarations No competing interests reported. Supplementary Files CAREchecklistEnglish20131.pdf Cite Share Download PDF Status: Posted Version 1 posted 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. We do this by developing innovative software and high quality services for the global research community. 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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-6546441","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":455320492,"identity":"f9c17d36-ec68-4197-8ecc-57acbbaeeb6f","order_by":0,"name":"Kabhilan A K","email":"","orcid":"","institution":"Hannah Joseph Hospital","correspondingAuthor":false,"prefix":"","firstName":"Kabhilan","middleName":"A","lastName":"K","suffix":""},{"id":455320494,"identity":"22a43db7-6c4d-4d5f-a636-0173a4f54dfb","order_by":1,"name":"Ebinesh Arulnathan","email":"data:image/png;base64,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","orcid":"","institution":"Lincoln County Hospital","correspondingAuthor":true,"prefix":"","firstName":"Ebinesh","middleName":"","lastName":"Arulnathan","suffix":""},{"id":455320495,"identity":"5976bae3-c461-4ae1-bcb4-3dd0d1563f38","order_by":2,"name":"Karunakaran N","email":"","orcid":"","institution":"Meenakshi Mission Hospital and Research Centre","correspondingAuthor":false,"prefix":"","firstName":"Karunakaran","middleName":"","lastName":"N","suffix":""},{"id":455320496,"identity":"1dc065ba-dbcb-4ac0-aa76-ff1d30a58a4c","order_by":3,"name":"Padma S","email":"","orcid":"","institution":"Meenakshi Mission Hospital and Research Centre","correspondingAuthor":false,"prefix":"","firstName":"Padma","middleName":"","lastName":"S","suffix":""},{"id":455320498,"identity":"a28267d0-e3ef-46ba-816e-82f8c5483aba","order_by":4,"name":"Praislin Gideon","email":"","orcid":"","institution":"Meenakshi Mission Hospital and Research Centre","correspondingAuthor":false,"prefix":"","firstName":"Praislin","middleName":"","lastName":"Gideon","suffix":""}],"badges":[],"createdAt":"2025-04-28 09:53:15","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-6546441/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-6546441/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":82797476,"identity":"189887f6-d187-41e5-b1fb-3bc64c7336c1","added_by":"auto","created_at":"2025-05-15 10:44:23","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":138715,"visible":true,"origin":"","legend":"\u003cp\u003eGrey-scale ultrasonographic image showing a solid heterogeneous in the pelvis, predominantly involving the right adnexa.\u003c/p\u003e","description":"","filename":"Fig1.png","url":"https://assets-eu.researchsquare.com/files/rs-6546441/v1/8cd17b043e2bd61dc895ef5e.png"},{"id":82794125,"identity":"adbe3051-7723-484a-b553-face241005b3","added_by":"auto","created_at":"2025-05-15 10:28:23","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":259527,"visible":true,"origin":"","legend":"\u003cp\u003eSagittal reformatted (A) and axial (B and C) contrast enhanced CT images show a heterogeneous lesion in the pelvis (asterisk) with enhancing solid areas, predominantly in the periphery. An enlarged heterogeneously enhancing paraaortic lymph node is also noted (white arrows).\u003c/p\u003e","description":"","filename":"Fig2.png","url":"https://assets-eu.researchsquare.com/files/rs-6546441/v1/39600bef781149f3a3afc27f.png"},{"id":82795744,"identity":"a80c526e-4133-483b-95a8-fe096e6d310b","added_by":"auto","created_at":"2025-05-15 10:36:23","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":315661,"visible":true,"origin":"","legend":"\u003cp\u003eCoronal T2 (A), axial T2 (B), axial DWI (C and E) and axial ADC (D and F) images show a heterogeneous solid lesion (asterisk) in the pelvis appearing hyperintense on T2 with internal hypointense fibrous septae and demonstrating restricted diffusion. An enlarged necrotic paraaortic lymph node (white arrow) is also noted with diffusion restriction.\u003c/p\u003e","description":"","filename":"Fig3.png","url":"https://assets-eu.researchsquare.com/files/rs-6546441/v1/db97e732dcfe15178abe8d14.png"},{"id":82794131,"identity":"84c086c3-50e6-41f5-9469-a0470ac57773","added_by":"auto","created_at":"2025-05-15 10:28:23","extension":"png","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":1010107,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eMicroscopic images representing the histomorphology and immunophenotyping of the tumor. \u003c/strong\u003eHistopathological images with hematoxylin and eosin (H\u0026amp;E) stain in 10x (A) and 20x (B and C) magnification show tumor cells arranged in microcystic and glandular configuration, demonstrating an eosinophilic cytoplasm, nuclear pleomorphism and cellular atypia. Multiple Schiller-Duval bodies (black arrows in C) characterized by a central vessel with surrounding sheets of tumor cells are noted. Periodic Acid Schiff (PAS) stain (D) highlights the presence of hyaline globules. The tumor cells show positive immunoreactivity to AFP (E) and SALL4 (F).\u003c/p\u003e","description":"","filename":"Fig4.png","url":"https://assets-eu.researchsquare.com/files/rs-6546441/v1/35df83c1e84280478139452d.png"},{"id":87720039,"identity":"02cc2c9d-717a-4c68-861b-71014074cad4","added_by":"auto","created_at":"2025-07-28 09:39:40","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":2207179,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-6546441/v1/fe679704-8270-4b73-98c9-0f2fe4fe1397.pdf"},{"id":82794143,"identity":"3680c840-088b-4919-86c3-cc099dd6d94f","added_by":"auto","created_at":"2025-05-15 10:28:23","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"supplement","size":3075114,"visible":true,"origin":"","legend":"","description":"","filename":"CAREchecklistEnglish20131.pdf","url":"https://assets-eu.researchsquare.com/files/rs-6546441/v1/369e171ba62d0ff4677dd737.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Malignant yolk sac tumor of the ovary presenting as adnexal torsion in a child: A rare case report with review of literature","fulltext":[{"header":"Introduction","content":"\u003cp\u003eOvarian germ cell tumors arise from the germ cells of the ovary and account for ~ 15-20% of all ovarian neoplasms (1). Malignant germ cell tumors of the ovary are rare with an incidence of ~ 1-2% (2). Yolk sac tumors, also known as endodermal sinus tumors, are highly aggressive with a predilection for gonadal sites (3). The gonads, particularly the ovaries, are the primary sites for these tumors. However, extragonadal sites like broad ligament, retroperitoneum, mediastinum and sacrococcygeal region have also been reported (3-5) . These tumors are usually seen in young women and adolescent girls. \u0026nbsp;Occurrence in children less than 10 years of age is extremely uncommon with a few sporadic case reports. Unlike epithelial tumors, malignant yolk sac tumors are locally aggressive and possess high metastatic potential. Due to their aggressive nature, early detection and management are critical for improving survival outcomes\u003c/p\u003e\n\u003cp\u003eThis highlights the rare presentation of malignant yolk sac tumor complicated by adnexal torsion, emphasizing the role of multimodality imaging in timely management.\u003c/p\u003e"},{"header":"Case Presentation","content":"\u003cp\u003eA 9-year-old girl child was brought to the emergency department with acute onset, severe right lower abdominal pain, nausea and few episodes of non-bilious vomiting. No history of preceding trauma, fever, or changes in urinary or bowel habits could be elicited. She had no prior abdominal surgeries or known health issues.\u003c/p\u003e\n\u003cp\u003eOn physical examination, the patient appeared visibly distressed and was clutching her abdomen. She was afebrile with stable vital signs. Abdominal examination revealed marked tenderness in the right lower quadrant with guarding. Laboratory investigations showed a leucocytosis (white blood cell count of 18,600/\u0026micro;L) and elevated inflammatory markers (ESR 32 mm/hr). Notably, alpha-fetoprotein (AFP) level was markedly elevated at 32,134 ng/mL, strongly suggestive of a malignant germ cell tumor. Lactate Dehydrogenase (LDH) was also elevated at 635 IU/L. Levels of other tumor markers were within normal limits (Human Chorionic Gonadotropin (HCG)- 0.74 ng/ml; CA-125- 14.6 U/ml; CA-19.9 \u0026lt;0.8 U/ml; Carcinoembryonic Antigen (CEA)- 1.52 ng/ml).\u0026nbsp;\u003c/p\u003e\n\u003ch3\u003eImaging Findings\u003c/h3\u003e\n\u003cp\u003ePelvic ultrasound revealed a large, heterogeneous, solid-cystic mass measuring 8 x 7.5cm in the right adnexa. The mass exhibited mixed echogenicity, with areas of necrosis (Figure 1). Colour Doppler imaging demonstrated no vascularity within the lesion, suspicious of adnexal torsion. Free fluid was noted in the pelvis, raising further concern of a possible adnexal torsion. Few enlarged para aortic lymph nodes also noted.\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;Contrast enhanced computed tomography (CECT) confirmed the presence of a lobulated heterogeneously enhancing solid pelvic mass measuring approximately 8.0 x 8.0 cm in the right adnexa (Figure 2). It was seen compressing the uterus, displacing it towards the left, and causing a significant mass effect on the urinary bladder and the right ureter, resulting in moderate right-sided hydroureteronephrosis. Minimal ascites was present. Multiple enlarged heterogeneously enhancing para-aortic and pelvic lymph nodes were identified, largest measuring up to 2.5 cm in diameter, consistent with nodal metastasis. No evidence of distant organ involvement was observed.\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;Magnetic resonance imaging (MRI) was subsequently performed for further characterization. It revealed a lobulated solid lesion in the right hemipelvis, appearing isointense on T1 and heterogeneously hyperintense on T2 with areas of restricted diffusion (FIgure 3). Right ovary could not be visualized separately from the lesion. Uterus was displaced to the left. The left ovary was visualized distinctly and measured 1.5 x 1.0 cm. Minimal hemoperitoneum was noted. It was also compressing the urinary bladder and the right ureter, causing moderate hydroureteronephrosis. Additionally, multiple enlarged heterogeneous pelvic and paraaortic lymph nodes were noted.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;Considering the radiological features, malignant ovarian germ cell tumor with multiple metastatic lymph nodes was considered. In view of acute presentation, absent vascularity on colour Doppler and presence of intraperitoneal hemorrhagic fluid, possibility of associated adnexal torsion was also considered.\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;Management\u003c/p\u003e\n\u003cp\u003eConsidering the possibility of adnexal torsion, emergency laparotomy was performed. Intraoperatively, a solid right adnexal mass with necrotic lymphadenopathy was observed, complicated by twisted vascular pedicles. Right salpingo-oopherectomy with lymph node dissection was performed.\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;Histopathological examination:\u003c/p\u003e\n\u003cp\u003eHistopathological examination (Figure 4) revealed a tumor composed of cells arranged in microcystic, festoon, and glandular patterns. The tumor cells exhibited nuclear pleomorphism and atypia. Hyaline globules were observed, along with many areas showing Schiller-Duval body formation. Extensive areas of hemorrhage and necrosis were noted. Dense inflammatory infiltrate composed of neutrophils was present, and brisk mitotic activity was observed. The ovarian capsule was ruptured. The fallopian tubes were also infiltrated by tumor cells. The tumor cells showed Periodic Acid Schiff (PAS)- positive hyaline globules and strong immunoreactivity to Alpha-FetoProtein (AFP) and Sal-like protein 4 (SALL4). These findings were diagnostic of malignant yolk sac tumor. Para aortic and Pelvic lymph nodes showed evidence of metastatic involvement.\u003c/p\u003e\n"},{"header":"Discussion","content":"\u003cp\u003eOne of the most common causes of Right lower quadrant (RLQ) pain in children is acute appendicitis, which typically presents with periumbilical pain migrating to the RLQ, accompanied by fever, anorexia, and nausea.(\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e). Ultrasonography is often the first-line imaging modality, with sensitivity ranging from 75\u0026ndash;90% (\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e). Another consideration is mesenteric adenitis, which mimics appendicitis but often follows a viral upper respiratory infection. It is more common in younger children and is usually self-limiting (\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e). Other important causes include intussusception, which presents with intermittent abdominal pain and bloody stools (\"red currant jelly stool\"). It is most common in infants and toddlers and requires emergent ultrasound or air enema for diagnosis and treatment\u003c/p\u003e \u003cp\u003eIn female children, especially adolescents, gynecologic causes must be considered. Ovarian torsion is a surgical emergency and typically presents with sudden onset of severe RLQ pain, often associated with nausea and vomiting. Ultrasound with Doppler is the diagnostic imaging modality of choice, though surgical exploration may be required in equivocal cases (\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eAnother important consideration is ovarian cyst rupture, which may cause acute RLQ pain and free fluid visible on imaging. Additionally, Mittelschmerz, or mid-cycle ovulation pain, may present as self-limited RLQ pain in pubescent girls (\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e).For sexually active adolescents, pelvic inflammatory disease (PID) and ectopic pregnancy are critical to rule out. A urine or serum pregnancy test is essential in this population to exclude ectopic pregnancy, even if the patient denies sexual activity (\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e).Urinary tract infections (UTIs) and pyelonephritis are common in girls and may present with RLQ pain, dysuria, fever, or flank pain. A urinalysis and urine culture are critical in evaluating these conditions. Kidney stones can also present with RLQ pain radiating to the groin, though they are less common in children(\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eRadiological imaging plays a critical role in the evaluation of adnexal lesions in female children, guiding both diagnosis and management. Ultrasound (US) is the first-line imaging modality due to its non-invasive nature, lack of ionizing radiation, and high sensitivity in characterizing adnexal masses. Gray-scale ultrasound can identify simple cysts, complex cysts, or solid masses, while Doppler imaging assesses vascular flow to detect conditions such as ovarian torsion, which presents with reduced or absent blood flow to the affected ovary (\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e). Functional ovarian cysts, the most common adnexal lesions in children, typically appear as simple, thin-walled, anechoic cysts, whereas neoplastic lesions may have mixed echogenicity or solid components. Color Doppler studies integrated with ultrasound can enhance diagnostic accuracy in emergencies like ovarian torsion, as normal vascular flow on Doppler can help rule out torsion with high sensitivity.\u003c/p\u003e \u003cp\u003eFor complex or indeterminate masses, magnetic resonance imaging (MRI) is preferred for further evaluation due to its superior soft-tissue resolution and ability to characterize the lesion\u0026rsquo;s origin and composition (e.g., fat, fluid, or solid). MRI is particularly helpful in distinguishing benign from malignant lesions, such as differentiating mature teratomas from malignant germ cell tumors (\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e).While computed tomography (CT) is not routinely used in pediatric adnexal lesions due to radiation exposure, it may be employed in cases where malignancy is suspected or for staging purposes, particularly in advanced tumors. CT is also useful in identifying metastatic spread or assessing adjacent organ involvement (\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e). In summary, a tailored approach combining ultrasound, MRI, and occasionally CT allows clinicians to effectively evaluate adnexal lesions in children while minimizing risks.\u003c/p\u003e \u003cp\u003eOvarian germ cell tumors (OGCTs) are the most common ovarian neoplasms in children, accounting for 60\u0026ndash;80% of all pediatric ovarian neoplasms (\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e). Unlike in adults, the majority of pediatric ovarian germ cell tumors are malignant (\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e, \u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e). Germ cell tumors include teratoma (mature and immature), dysgerminoma, yolk sac tumor, embryonal carcinoma and mixed germ cell tumors. These tumors arise from primitive germ cells and include both benign and malignant types. Benign tumors, such as mature teratomas (dermoid cysts) typically consist of mature tissues like skin, hair, and fat. These tumors are often asymptomatic unless complications like torsion or rupture occur. Malignant OGCTs include dysgerminomas, yolk sac tumors, immature teratomas, and mixed germ cell tumors. Dysgerminomas, the most common malignant subtype, are solid and radiosensitive, while yolk sac tumors are aggressive and characterized by elevated alpha-fetoprotein (AFP) levels. Immature teratomas contain immature neural or mesenchymal tissue with malignant potential, and mixed germ cell tumors feature elements of multiple subtype (\u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eSerum tumor markers play a pivotal role in the diagnosis, monitoring, and follow-up of ovarian tumors, particularly in pediatric and adolescent patients.. Among ovarian germ cell tumors (OGCTs), alpha-fetoprotein (AFP) is the most widely used marker and is elevated in yolk sac tumors (endodermal sinus tumors). AFP levels are both diagnostic and prognostic, helping to monitor treatment response and detect recurrences. Beta-human chorionic gonadotropin (β-hCG) is another crucial marker, often elevated in choriocarcinomas and some mixed germ cell tumors. Dysgerminomas, the most common malignant OGCT, are associated with elevated lactate dehydrogenase (LDH) levels, reflecting their high metabolic activity.\u003c/p\u003e \u003cp\u003eFor epithelial ovarian tumors, which are rare in children, markers such as CA-125 may be elevated, though it is more commonly used in adults. Inhibin and estradiol may be elevated in sex cord-stromal tumors like granulosa cell tumors, which are rare but hormonally active, potentially causing precocious puberty.\u003c/p\u003e \u003cp\u003eThe utility of tumor markers extends beyond diagnosis to guiding management. Preoperative levels of AFP, β-hCG, and LDH can provide insights into the likelihood of malignancy and the tumor subtype, assisting in surgical planning. Postoperative monitoring of these markers is essential to evaluate treatment response and identify early recurrences. Despite their utility, serum tumor markers must be interpreted alongside clinical findings and imaging, as false positives can occur in certain physiological or pathological conditions (\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eThe treatment of germ cell tumors (GCTs) in children involves a multidisciplinary approach that focuses on achieving optimal oncological outcomes while preserving fertility and minimizing treatment-related toxicities. The primary modality for both benign and malignant GCTs is surgical resection. For benign ovarian GCTs, such as mature teratomas, fertility-sparing surgery is the cornerstone of treatment. The goal is to remove the tumor while preserving the unaffected ovary and uterus. Malignant germ cell tumors, such as dysgerminomas, yolk sac tumors, and immature teratomas, require more extensive surgical excision, often accompanied by lymph node evaluation and staging procedures. However, fertility preservation remains a critical consideration during these surgeries (\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eIn cases of malignant GCTs, adjuvant chemotherapy is a crucial component of treatment, particularly for advanced-stage or recurrent disease. Platinum-based chemotherapy regimens, most commonly consisting of bleomycin, etoposide, and cisplatin (BEP), are highly effective and have significantly improved survival rates in pediatric patients. For localized dysgerminomas, surgery alone may be sufficient, as these tumors are highly radiosensitive. However, chemotherapy is preferred for advanced-stage disease or for tumors like yolk sac tumors that exhibit aggressive behavior (\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e). Radiotherapy is used sparingly in children to avoid long-term complications, such as infertility or secondary malignancies, and is generally reserved for specific cases of dysgerminomas.\u003c/p\u003e \u003cp\u003eTreatment response is monitored using serum tumor markers such as alpha-fetoprotein (AFP), beta-human chorionic gonadotropin (β-hCG), and lactate dehydrogenase (LDH). Declining marker levels post-treatment indicate effective tumor control, while persistent elevation suggests residual disease or recurrence. Long-term follow-up is essential to monitor for late effects of chemotherapy, including nephrotoxicity, ototoxicity, and secondary malignancies, as well as to assess fertility outcomes. With the advent of modern multimodal treatments, the prognosis for pediatric GCTs is excellent, with 5-year survival rates exceeding 90% for early-stage disease and around 70\u0026ndash;80% for advanced disease (\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e).\u003c/p\u003e"},{"header":"Conclusions","content":"\u003cp\u003eThis case report highlights the role of imaging in preoperative evaluation, diagnosis and management of ovarian tumors in children. Multimodality imaging involving ultrasonography, computed tomography and magnetic resonance imaging is indispensable in characterizing pediatric ovarian tumors and evaluation of associated complications like tumor rupture, torsion and metastasis.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003col start=\"1\" type=\"1\"\u003e\n \u003cli\u003eAFP - Alpha-Fetoprotein\u003c/li\u003e\n \u003cli\u003eLDH - Lactate Dehydrogenase\u003c/li\u003e\n \u003cli\u003eHCG - Human Chorionic Gonadotropin\u003c/li\u003e\n \u003cli\u003eCA-125 - Cancer Antigen 125\u003c/li\u003e\n \u003cli\u003eCA-19.9 - Cancer Antigen 19.9\u003c/li\u003e\n \u003cli\u003eCEA - Carcinoembryonic Antigen\u003c/li\u003e\n \u003cli\u003eCECT - Contrast Enhanced Computed Tomography\u003c/li\u003e\n \u003cli\u003eMRI - Magnetic Resonance Imaging\u003c/li\u003e\n \u003cli\u003eUS - Ultrasonography\u003c/li\u003e\n \u003cli\u003eRLQ - Right Lower Quadrant\u003c/li\u003e\n \u003cli\u003eBEP - Bleomycin, Etoposide, and Cisplatin (Chemotherapy regimen)\u003c/li\u003e\n \u003cli\u003eOGCT - Ovarian Germ Cell Tumors\u003c/li\u003e\n \u003cli\u003eGCT - Germ Cell Tumors\u003c/li\u003e\n \u003cli\u003eCT - Computed Tomography\u003c/li\u003e\n \u003cli\u003eDWI - Diffusion Weighted Imaging\u003c/li\u003e\n \u003cli\u003eADC - Apparent Diffusion Coefficient\u003c/li\u003e\n \u003cli\u003ePID - Pelvic Inflammatory Disease\u003c/li\u003e\n \u003cli\u003eUTI - Urinary Tract Infection\u003c/li\u003e\n \u003cli\u003eESR - Erythrocyte Sedimentation Rate\u003c/li\u003e\n \u003cli\u003ePAS - Periodic Acid-Schiff\u003c/li\u003e\n \u003cli\u003eSALL4 - Spalt-Like Transcription Factor 4\u003c/li\u003e\n\u003c/ol\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eFunding information\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe author(s) received no financial support for the research, authorship, and/or publication of this article.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe author(s) declare that they have no financial or personal relationship(s) that may have inappropriately influenced them in writing this article.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthical considerations\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll procedures performed in this study involving a human participant were in accordance with the ethical standards of the institutional ethical committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthics and\u0026nbsp;\u003c/strong\u003e\u003cstrong\u003eConsent for p\u003c/strong\u003e\u003cstrong\u003earticipation:\u0026nbsp;\u003c/strong\u003eNot Applicable\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eInformed consent was obtained from the patient\u0026apos;s parents to publish the case and use their radiological images involved in the preparation of this manuscript.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eData availability\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eData sharing is not applicable to this research article as no new data were created or analysed in this study.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCode Availability\u003c/strong\u003e\u003cbr\u003e\u0026nbsp;The data supporting the findings of this case report are available within the manuscript. No custom code was developed. Any software used for image processing is cited appropriately in the references.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors\u0026rsquo; contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eKAK: study of concept and design, literature research, manuscript preparation, manuscript editing\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eEA: guarantor of integrity of the entire study, study of concept and design, literature research, manuscript preparation, manuscript editing \u0026nbsp;\u003c/p\u003e\n\u003cp\u003eKN: study of concept and design, literature research, manuscript editing\u003c/p\u003e\n\u003cp\u003ePS: study of concept and design, literature research, manuscript editing\u003c/p\u003e\n\u003cp\u003ePG: literature research, manuscript preparation, manuscript editing\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eKojimahara T, Nakahara K, Takano T, Yaegashi N, Nishiyama H, Fujimori K, et al. Yolk sac tumor of the ovary: a retrospective multicenter study of 33 Japanese women by Tohoku Gynecologic Cancer Unit (TGCU). Tohoku J Exp Med. 2013; 230: 211\u0026ndash;7. \u003c/li\u003e\n\u003cli\u003eDallenbach P, Bonnefoi H, Pelte MF, Vlastos G. Yolk sac tumours of the ovary: an update. Eur J Surg Oncol. 2006; 32:1063\u0026ndash;75. \u003c/li\u003e\n\u003cli\u003eFarah RA, Matta J K, Metri AA, Bejjani- Doueihy NA, Saikali IC. Extragonadal germ cell tumor presenting with spinal cord compression: a case report and literature review. Ann Pediatr Surg. 2022; 18, 36.\u003c/li\u003e\n\u003cli\u003eGao Y, Jiang J, Liu Q. Extragonadal malignant germ cell tumors: A clinicopathological and immunohistochemical analysis of 48 cases at a single Chinese institution. Int J Clin Exp Pathol. 2015; 8: 5650-7.\u003c/li\u003e\n\u003cli\u003eRudaitis V, Mickys U, Katinaitė J, Dulko J. Successful treatment of advanced stage yolk sac tumour of extragonadal origin: a case report and review of literature. Acta Med Litu. 2016; 23: 110-6.\u003c/li\u003e\n\u003cli\u003eLotfollahzadeh S, Lopez RA, Deppen JG. Appendicitis. [Updated 2024 Feb 12]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan.\u003c/li\u003e\n\u003cli\u003eAzizaddini S, Mani N. Liver Imaging. [Updated 2023 Feb 20]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan.\u003c/li\u003e\n\u003cli\u003eOtto M, Nagalli S. Mesenteric Adenitis. [Updated 2023 Aug 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan.\u003c/li\u003e\n\u003cli\u003ePoonai N, Poonai C, Lim R, Lynch T. Pediatric ovarian torsion: case series and review of the literature. Can J Surg. 2013; 56: 103-8. \u003c/li\u003e\n\u003cli\u003eMobeen S, Apostol R. Ovarian Cyst. [Updated 2023 Jun 5]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan.\u003c/li\u003e\n\u003cli\u003eTay JI, Moore J, Walker JJ. Ectopic pregnancy. BMJ. 2000; 320: 916-9. \u003c/li\u003e\n\u003cli\u003eCar J. Urinary tract infections in women: diagnosis and management in primary care. BMJ. 2006; 332: 94-7. \u003c/li\u003e\n\u003cli\u003eIyer VR, Lee SI. MRI, CT, and PET/CT for ovarian cancer detection and adnexal lesion characterization. AJR Am J Roentgenol . 2010; 194: 311\u0026ndash;21. \u003c/li\u003e\n\u003cli\u003eZhang J, Li Y, Zhao Y, Qiao J. CT and MRI of superficial solid tumors. Quant Imaging Med Surg. 2018; 8: 232-251. \u003c/li\u003e\n\u003cli\u003eHeo SH, Kim JW, Shin SS, Jeong SI, Lim HS, Choi YD et al. Review of ovarian tumors in children and adolescents: radiologic-pathologic correlation. Radiographics. 2014; 34: 2039\u0026ndash;55.\u003c/li\u003e\n\u003cli\u003eCass DL, Hawkins E, Brandt ML, Chintagumpala M, Bloss RS, Milewicz AL, et al. Surgery for ovarian masses in infants, children, and adolescents: 102 consecutive patients treated in a 15-year period. J Pediatr Surg. 2001; 36: 693-9.\u003c/li\u003e\n\u003cli\u003eSchultz KA, Sencer SF, Messinger Y, Neglia JP, Steiner ME. Pediatric ovarian tumors: a review of 67 cases. Pediatr Blood Cancer. 2005; 44: 167-73.\u003c/li\u003e\n\u003cli\u003eGică N, Peltecu G, Chirculescu R, Gică C, Stoicea MC, Serbanica AN, Panaitescu AM. Ovarian Germ Cell Tumors: Pictorial Essay. Diagnostics (Basel). 2022; 12: 2050. \u003c/li\u003e\n\u003cli\u003eJezierska M, Gawrychowska A, Stefanowicz J. Diagnostic, Prognostic and Predictive Markers in Pediatric Germ Cell Tumors-Past, Present and Future. Diagnostics (Basel). 2022; 12: 278. \u003c/li\u003e\n\u003cli\u003eBhuta R, Shah R, Gell JJ, Poynter JN, Bagrodia A, Dicken BJ et al. Children\u0026apos;s Oncology Group\u0026apos;s 2023 blueprint for research: Germ cell tumors. Pediatr Blood Cancer. 2023; 70 Suppl 6: e30562. \u003c/li\u003e\n\u003cli\u003eDe Giorgi U, Casadei C, Bergamini A, Attademo L, Cormio G, Lorusso D et al. Therapeutic Challenges for Cisplatin-Resistant Ovarian Germ Cell Tumors. Cancers (Basel). 2019; 11: 1584. \u003c/li\u003e\n\u003cli\u003eDieckmann KP, Simonsen-Richter H, Kulejewski M, Anheuser P, Zecha H, Isbarn H, Pichlmeier U. Serum Tumour Markers in Testicular Germ Cell Tumours: Frequencies of Elevated Levels and Extents of Marker Elevation Are Significantly Associated with Clinical Parameters and with Response to Treatment. Biomed Res Int. 2019; 2019: 5030349. \u003c/li\u003e\n\u003c/ol\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":"Malignant Yolk Sac Tumor, Ovarian Germ Cell Tumor, Adnexal Torsion, Pediatric Ovarian Tumors, Multimodality Imaging","lastPublishedDoi":"10.21203/rs.3.rs-6546441/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-6546441/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eIntroduction:\u003c/h2\u003e \u003cp\u003eMalignant yolk sac tumor is a rare ovarian neoplasm with peak incidence in young women. Its occurrence in children less than 10 years old is extremely uncommon. In this manuscript we report a rare case of malignant yolk sac tumor presenting with adnexal torsion, emphasizing the role of multimodality imaging in management.\u003c/p\u003e\u003ch2\u003eCase Presentation:\u003c/h2\u003e \u003cp\u003eA 9-year-old female child was brought with the complaints of acute right lower quadrant pain, nausea and vomiting. On examination, the abdomen was tender with guarding. Laboratory investigations revealed leukocytosis, raised inflammatory markers and markedly elevated alpha-fetoprotein (AFP) levels. Ultrasonography revealed a mixed echogenic tumor in the right adnexa with features of adnexal torsion. Cross sectional imaging confirmed the presence of a heterogeneously enhancing tumor in the right adnexa with pelvic and paraaortic lymphadenopathy. Subsequently, the child underwent emergency laparotomy for adnexal detorsion with tumor excision. Postoperative histopathological examination confirmed malignant yolk sac tumor.\u003c/p\u003e\u003ch2\u003eConclusions\u003c/h2\u003e \u003cp\u003eThis case underscores the rare presentation of ovarian malignant yolk sac tumor as adnexal torsion in a child and the role of multimodality imaging in its management.\u003c/p\u003e","manuscriptTitle":"Malignant yolk sac tumor of the ovary presenting as adnexal torsion in a child: A rare case report with review of literature","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-05-15 10:28:18","doi":"10.21203/rs.3.rs-6546441/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":"080f227a-d70f-40cf-a628-f3ed9a6cada5","owner":[],"postedDate":"May 15th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2025-07-28T09:39:20+00:00","versionOfRecord":[],"versionCreatedAt":"2025-05-15 10:28:18","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-6546441","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-6546441","identity":"rs-6546441","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}