Sex Cord-stromal Tumors Research Articles

The Oncogenic FOXL2 C134W Mutation Is a Key Driver of Granulosa Cell Tumors.

A newly generated mouse model carrying a FOXL2 mutation characteristic of adult-type granulosa cell tumors shows that FOXL2 C134W shifts the transcriptome towards a signature of granulosa cell cancer and drives tumorigenesis.

Review of Sclerosing stromal tumor: A relatively rare ovarian neoplasm

Abstract Introduction/Objective Sclerosing stromal tumor (SST) is sex cord stromal tumor which is rare primary ovarian neoplasm. Because its clinical presentation and imaging findings accurate preoperative clinical diagnosis can be difficult. We report three cases of a sclerosing stromal tumor of ovary and histopathological presentation. Methods/Case Report Our patients had an age ranged from 27-40 years with primary ovarian neoplasm and as incidental in 64-year-old patient. Radiologically lobulated and heterogeneously enhancing, partially calcified masses with nodular rind of avid enhancement about the periphery of the lesion with high risk malignancy were seen. Grossly the masses had firm tan-white, solid, lobulated cut surfaces with patchy calcifications Results (if a Case Study enter NA) Microscopically all the three tumors showed a hyalinized, well-circumscribed nodule with few scattered trabeculae of spindled to plump cells with pale grey cytoplasm within a sclerotic stroma with few staghorn- like blood vessels around the periphery. Scattered calcifications were identified. No mitoses are identified. The reticulin stain showed pericellular focal nested staining pattern. There was no necrosis, pleomorphism, or features of malignancy were identified. Findings were consistent with a sclerosing stromal tumor. Conclusion Ovarian sex cord-stromal tumors are relatively infrequent neoplasms of all primary ovarian tumors. Clinical and sonographic findings alone do not predict the presence of this tumor. SSTs should be considered in differentials in young females with unilateral masses with cystic and solid masses. These tumors carry a good prognosis. Histopathological features along with immunohistochemsitry establish the diagnosis.

Malignant testicular unclassified sex cord stromal tumor: a case report

BackgroundMost testicular tumors are germ cell tumors; sex cord stromal tumors are infrequent, accounting for only 3–5% of testicular tumors. Unclassified sex cord stromal tumors are extremely rare. Generally, 10% of sex cord stromal tumors are malignant. We report a case of malignant unclassified sex cord stromal tumor with retroperitoneal lymph node metastasis at first visit and a corresponding literature review.Case presentationA 72-year-old Japanese man visited our department primarily for indolent right scrotum enlargement in September 2020. Blood biochemistry examination, urinalysis, and tumor markers (alpha-fetoprotein, human chorionic gonadotropin, and lactate dehydrogenase) showed no abnormal findings. Contrast-enhanced computed tomography showed enlarged para-aortic lymph node (18 × 16 and 10 × 102 mm); a 50 × 452 mm mass with uneven contents was found in the right testicle. The patient underwent inguinal orchiectomy in September 2020. As per immunohistochemistry, the tumor cells were diffusely positive for SF-1 and Ki-67, partially positive for inhibin, and negative for CAM5.2, CK7, CK20, C-KIT, CD30, LCA, GATA-3, TTF-1, and PAX8. Calretinin was expressed in approximately 5% of tumor cells; thus, sex cord/gonadal stroma components were considered to be involved. The final pathological diagnosis was unclassified malignant sex cord stromal tumor. The patient was diagnosed with pT1, N1, M0, S0, and tumor–node–metastasis stage IIA disease. The patient received postoperative chemotherapy with four courses of etoposide and cisplatin therapy from November 2020. Post-chemotherapeutic computed tomography showed new metastatic lesions including lung, liver, pancreas, and para-aortic lymphadenopathy, which increased in size. Disease progression was observed. Cancer genome research was performed using the OncoGuide National Cancer Center oncopanel system; however, no gene mutation for which the drug could be expected to be effective was found. The patient opted for best supportive care at a nearby hospital and died from cancer progression in January 2022.ConclusionWe encountered a case of malignant testicular unclassified sex cord stromal tumor pathologically diagnosed as testicular tumor with retroperitoneal lymph node metastasis in a patient who underwent inguinal orchiectomy. Future data collection is necessary to establish multimodality therapy for malignant testicular unclassified sex cord stromal tumor.

Pathological Features of Ovarian Tumors, Diagnosed at a Tertiary Care Hospital in Afghanistan: A Cross-sectional Study.

In Afghanistan, research work is still in its infancy and there is no national level tumor registry at the moment in the country that could elaborate the histopathological features of ovarian tumors in the country. The current study was conducted with the aim to describe pathological characteristics of ovarian tumors diagnosed at tertiary level in Afghanistan. A descriptive cross-sectional study was conducted, including 198 cases diagnosed with ovarian tumors, that were consecutively included in the study from July 2017 to August 2020. All the cases were diagnosed at the Department of Pathology, French Medical Institute for Mothers and Children, Kabul, Afghanistan, that receives biopsy samples from all of the tertiary care institutions in Kabul. In the current study, majority of the ovarian tumors were benign and presented with nonspecific symptoms. The mean age at diagnosis was 34.4 (SD ±13.4). Benign tumors comprised 81.8%, borderline 1.5% and malignant 16.7% of the cases. Majority of the diagnosed tumors were from surface epithelium in origin, followed by germ cell tumors, sex cord stromal tumors, and a single metastatic tumor. The most common benign neoplasm was mature cystic teratoma, followed by benign serous cystadenoma. Considering the malignant tumors, serous cystadenocarcinoma and adult granulosa cell tumors were predominant, followed by endometrioid adenocarcinoma and mucinous cystadenocarcinoma. More than half of the ovarian tumors occurred between 21 and 40 years of age. In the current study, the proportion of malignant ovarian neoplasms was significantly less than benign lesions. Although, many of the pathological features related to ovarian neoplasms were similar to the features demonstrated in other regions of the world, there were important findings that were exclusively noted in the cases diagnosed in Afghanistan.

ODP622 A Case of a Rare Steroid Cell Tumor in a Patient with PCOS

Abstract Background Ovarian sex cord-stromal tumors are rare and represent approximately 7% of all primary ovarian tumors. Steroid cell tumors of the ovary are uncommon sex-hormone secreting tumors and are classified as pure stromal tumors. These tumors are known to secrete hormones like androstenedione, alpha-hydroxyprogesterone, and testosterone. Polycystic Ovarian Syndrome (PCOS), a disorder affecting 6% to 10%, is characterized by hyperandrogenism and ovulatory dysfunction. We report a case of a patient with known PCOS who was found to have steroid secreting tumor. Clinical Case A 35 years old female presented to our office complaining of worsening hirsutism, amenorrhea and weight gain. Her past medical history was significant for PCOS, hypothyroidism, and obesity. Physical Examination was notable for hirsutism, obesity and clitoromegaly. Initial labs showed elevated testosterone level of 134 ng/dL (normal levels 0-70 ng/dL). Other labs ruled out congenital adrenal hyperplasia (CAH). Pelvic US was consistent with known PCOS but did not revealed additional findings. Endometrial biopsy was unremarkable. Subsequent labs showed continuous significant elevation in testosterone to 382 ng/dL with free testosterone level of 24 ng/dL. CT abdomen and pelvis with and without contrast showed a right solid adnexal mass. Patient underwent staging laparotomy with bilateral salpingo-oophorectomy. Pathology result was significant for well differentiated sex cord-stromal tumor, steroid secreting type. Genetic testing came positive for BRCA 1. Conclusion This case illustrates that in the setting of rapid onset of hirsutism and elevated testosterone, an androgen secreting ovarian tumor should be considered and ruled out, even in the setting of known PCOS. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

PMON227 Rare ovarian luteinized fibrothecoma as cause of severe hyperandrogenism in a pre-menopausal woman.

Abstract Ovarian fibromas and fibrothecomas constitute up to 4.7% of ovarian sex cord stromal tumors (OSCSTs). Luteinized fibrothecomas (LFs) are a histological subvariant of OSCSTs. Typically benign and rare, LFs are mostly diagnosed in post-menopausal women with only 30% in women <30. Only 11% of LFs present with androgen excess (AE). We describe a case of severe hyperandrogenism from an ovarian LF in a young woman with primary amenorrhea and infertility.A 23-year-old woman presented for evaluation of hyperandrogenism. Iatrogenic menarche occurred at age 22, induced by letrozole and medroxyprogesterone (MP). BP was 133/86 mm Hg and BMI was 52 kg/m2. Exam revealed diffuse obesity, severe hirsutism (Ferriman Gallwey score 29), and severe acanthosis nigricans, but no scalp alopecia, deep voice, violaceous striae or bruising. Total testosterone (TT) was 108 ng/dl (2-45), free testosterone (FT) 24.7 pg/ml (0.1-6.4 dialysis), prolactin 13.2 ng/ml (<30), DHEAS 2308 ng/ml (240-4330), and TSH 1.29 µIU/mL (0.45-5.33). FSH, LH and estradiol could not be interpreted due to unclear timing in cycle. 17-hydroxyprogesterone after MP challenge was 73 ng/dl (<200). Pelvic ultrasound (US) gave normal endometrial stripe of 3.82 mm. Pelvic US and MRI showed polycystic ovarian morphology without masses. CT abdomen showed normal adrenal glands. Ovarian and adrenal vein sampling (OAVS) lateralized AE to the left ovary (LO). LO TT was 5743 ng/dl with associated IVC sample of 139 ng/dl, ratio of 41, and right ovarian TT was 381 ng/dl with associated IVC sample of 32 ng/dl, ratio of 1.18. Just prior to left oophorectomy, preoperative TT was 103 ng/dl, FT 27.8 pg/ml (0.1-6.4 dialysis), and androstenedione (A4) 212 ng/dl (mid-follicular 51-213). Two days post-operatively, TT dropped to 55 ng/dl, FT 11 pg/ml (0.2-5), and A4 105 ng/dl. Pathology revealed a 3.8 cm LF. Six weeks post-operatively, TT and FT normalized to 18 and 3.6 (0.2-5), respectively.Features atypical for PCOS, including presentation outside age 15-25, rapid progression, TT >2× upper limit of normal (ULN), and free androgen index >4× ULN should prompt thorough evaluation. Adherence to guidelines for TT threshold >150 in a pre-menopausal woman and focus on PCO morphology would have provided false reassurance. Maintaining a high degree of suspicion for an androgen-secreting tumor (AST) is paramount when clinical presentation is atypical. OAVS is usually performed when imaging fails to reveal an abnormality, as small ovarian tumors cannot be excluded. Our patient presented with symptoms atypical for PCOS including severe hirsutism and TT >2× ULN, raising concern for an AST. OAVS lateralized AE to her LO. Resection resulted in testosterone normalization and finding of a rare LF. Identifying the source of AE is especially important in women of reproductive age to restore normal reproductive function. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

Uterine Tumors Resembling Ovarian Sex Cord Tumors: A Clinicopathologic Study of 75 Cases Emphasizing Features Predicting Adverse Outcome and Differential Diagnosis.

Uterine tumors resembling ovarian sex cord tumors (UTROSCTs), first characterized by Drs Clement and Scully in 1976, are rare neoplasms showing clinical, morphologic, and immunohistochemical overlap with a number of other uterine tumors, most being mesenchymal. Criteria for aggressive behavior are not clearly established. We report 75 tumors from patients ranging from 21 to 84 (mean=52.4) years. Seventy-one patients were treated by hysterectomy and 4 by conservative total excision. Thirty-eight tumors were intramyometrial, 34 submucosal, and 3 cervical; they ranged from 0.6 to 20 (mean=4.9)cm and were typically tan-yellow. Sixty-eight neoplasms were well-circumscribed and 7 had infiltrative borders (4 only minimally). In 56 tumors, a smooth muscle component was intimately admixed with the neoplastic cells ("pseudoinfiltration"; extensive in 29). Architectural patterns included cords (n=53), diffuse (n=51), hollow tubules (n=48), nests (n=38), trabeculae (n=37), retiform (n=23), solid tubules (n=21), pseudoangiomatoid (n=11), pseudopapillary (n=4), and whorled (n=2); typically, more than 1 pattern was seen. Tumor cells were epithelioid (n=62), epithelioid and spindled (n=12), or spindled (n=1) and/or rhabdoid (n=20; extensive in 2). Cytologic atypia was absent to mild in 57, moderate in 16, and moderate to severe in 2 tumors. Fifty-seven UTROSCTs had ≤2mitoses/10 high power fields (HPF), 12 had 3 to 5/10 HPF, and 6 >5/10 HPF. Necrosis was present in 3 and lymphovascular invasion in 1. Tumor cells showed a polyphenotypic immunohistochemical profile (with positivity for sex cord, smooth muscle, and epithelial markers), most commonly inhibin (17/33+) and calretinin (22/31+) positive. Five of 58 patients with follow-up (22 to 192; mean=73.2mo) had recurrences/metastases from 30 to 144 months, and 2 died of disease. Malignant tumors showed >3 of the following 5 features compared with benign tumors: size >5cm, at least moderate cytologic atypia, ≥3 mitoses/10 HPF, infiltrative borders, and necrosis. One of the 5 malignant tumors showed an extensive rhabdoid morphology. UTROSCTs are uncommon, show a wide morphologic spectrum, often pose problems in differential diagnosis, and typically have a benign outcome. Rare tumors are associated with late recurrences and a combination of more than 3 of the 5 features listed above predicted aggressive behavior in this series.

Adult-Type Granulosa Cell Tumor, an Unusual Site of Recurrent Tumor at The Sigmoid Colon: A Case Report and Literature Review

Granulosa cell tumor constitutes approximately 1% of all ovarian tumors. Extraovarian spread can occur as a late recurrence. The authors reported a case of 41-year-old woman with adult-type granulosa cell tumor at sigmoid colon. The patient visited the outpatient department for a yearly medical check-up with no specific complaints. She had a history of ovarian cystectomy 12 years prior. A mass in left pelvic cavity was revealed upon transvaginal ultrasonography and computer tomography (CT). At the time of laparoscopy, a well-defined subserosa mass was detected at the antimesenteric side of the sigmoid colon. The right and left ovaries appeared grossly normal. The tumor was successfully removed with laparoscopic sigmoidectomy. The pathologic findings were that of adult-type granulosa cell tumor, which was corresponding to the previous diagnosis made in the ovarian cystectomy specimen 12 years ago. The authors presented this case as an example of late recurrence of ovarian granulosa cell tumor in sigmoid colon, which could potentially be mistaken as other usual colonic tumors in the absence of previous history of ovarian tumor. Keywords: Colonic granulosa cell tumor; Extraovarian granulosa cell tumor; Sex cord-stromal tumor

SCLEROSING STROMAL TUMOR: AN INCIDENTAL FINDING IN PATIENT OF INFERTILLITY

Ovarian sex cord stromal tumors are infrequent and represents only 7% of all ovarian neoplasm. Cells of origin of these tumors are primitive sex cords or stromal cells. The stromal cells comprise of theca cells, fibroblasts and Leydig cells whereas the gonadal primitive sex cords include granulosa cells and Sertoli cells. According to recent WHO classification sex cord stromal tumors are further subdivided into pure stromal tumors, pure sex cord tumors and mixed sex cord stromal tumors. Here we are going to discuss a rare case of pure stromal tumors i.e., Sclerosing stromal tumor.

Serous cystadenocarcinoma of the testis: case report of a rare entity

BackgroundGerm cell tumors account for more than 95% of testicular cancers followed by sex-cord stromal tumors. The occurrence of serous cystadenocarcinoma in testicular and paratesticular tissue is very rare with distinct clinical behavior.Case presentationHerein, we report the case of a 39-year-old man who presented with right testicular swelling. Anatomopathological examination of the right orchiectomy revealed a high-grade paratesticular serous cystadenocarcinoma. Long-term follow-up with close monitoring was adopted in the absence of concomitant metastasis. The patient was disease-free at two years post-surgery.ConclusionOwing to its scarcity, there are no standardized guidelines for optimal management of this histological entity. Sharing case reports provide valuable information to support clinical decisions.

Inhibin Alpha Expression in Human Tumors: A Tissue Microarray Study on 12,212 Tumors.

As a result of its expression in corresponding normal cell types, inhibin alpha (INHA) is used as an immunohistochemical marker for adrenocortical neoplasms and testicular or ovarian sex cord stromal tumors. However, other tumors can also express INHA. To comprehensively determine INHA expression in cancer, a tissue microarray containing 15,012 samples from 134 different tumor types and subtypes was analyzed by immunohistochemistry. INHA positivity was found in 72 of 134 tumor categories, including 26 categories with ≥1 strongly positive case. A moderate to strong INHA positivity was found in 100% of 37 granulosa cell tumors of the ovary, 100% of 43 other sex cord stromal tumors of the ovary/testis, 100% of 31 granular cell tumors, 78.5% of 28 adenomas, 44% of 25 carcinomas of the adrenal cortex, and 46.7% of 15 pancreatic acinar cell carcinomas. At least a weak INHA positivity was seen in <33% of cases of 46 additional tumor entities. In summary, these data support the use of INHA antibodies for detecting sex cord stromal tumors, granular cell tumors, and adrenocortical neoplasms. Since INHA can also be found in other tumor entities, INHA immunohistochemistry should only be considered as a part of any panel for the distinction of tumor entities.

S3279 A Rare Case of Late Metastatic Disease From Granulosa Cell Tumor as a Solitary Hepatic Mass

Introduction: Granulosa Cell Tumor (GCT) is a rare type of sex cord-stromal neoplasm that often has an indolent course. These tumors make up 2-5% of all ovarian malignancies. When they do metastasize, it is often within the lower abdomen and pelvis. Recurrence of the disease often occurs within 10 years of the diagnosis, but there is potential for recurrent disease beyond this timeframe. Because of the risk of late recurrence with GCT, continued surveillance is crucial beyond surgical resection of the primary tumor. Inhibin has been shown to be a valid marker in both management of primary disease and surveillance for late recurrence. The overall prognosis for GCT is generally favorable given that the disease is detected early. Surgical intervention is mainstay of treatment for these tumors, although systemic chemotherapy is considered in nonresectable or advanced cases. Case Description/Methods: A 53-year-old female with a past medical history of bilateral GCT of the ovaries status post bilateral salpingo-oophorectomy (2009), gastroesophageal reflux disease, and Hepatitis C presented to the emergency department with lower abdominal pain and occasional hematochezia in 2021. She was discharged with referrals for further evaluation. During her work up, she underwent a CT scan that revealed a 4.3 x 3.4 cm exophytic mass in the right lobe of the liver. Further evaluation with MRI showed that the mass was irregular and exophytic with minimally increased size, now measuring 4.6 x 3.7 cm. A CT-guided biopsy showed that the tumor was positive for inhibin, suggestive of metastatic GCT. The solitary hepatic metastasis was treated with microwave ablation. (Figure) Discussion: GCTs are a rare subtype of sex-cord stromal tumor that are typically primary ovarian neoplasms and often have an indolent course and favorable prognosis, although there is evidence that metastatic disease can occur at distant sites and greater than 10 years after diagnosis and surgical resection of the primary tumor. This case describes the recurrence of disease as a solitary hepatic mass. Diagnostic confirmation was made by CT-guided biopsy that found the presence of inhibin, a protein known to be produced by GCT. This case highlights the importance of long-term surveillance in patients with a history of GCT beyond surgical resection and undetectable disease activity.Figure 1.: MRI of the abdomen showing metastatic granulosa cell tumor (arrow).

Unilateral and Bilateral Involvement of Ovarian Tumors According to Histopathological Types

Introduction: Ovarian neoplasm is produced by the rapid growth and division of cells within the ovary. Under normal circumstances, ovarian cells reproduced to maintain tissue health. When growth control is lost and cellular division is too much and too fast, a cellular mass or tumor is formed. Ovarian neoplasm is the one of major gynecological malignancies &amp; is a frequent site for primary &amp; metastatic tumors. Due to its complex structure, primary ovarian neoplasm manifests a wide spectrum of clinical, morphological &amp; histological features. Ovarian neoplasm has become increasingly important, not only because of a large variety of neoplastic entities but also because they have gradually increased the mortality rate due to genital cancer. The study aimed to find out the unilateral and bilateral involvement of ovarian tumors according to histopathological types. Methods: This cross-sectional study was carried out among forty-five patients who were admitted to the department of gynecology at Shaheed Suhrawardy Medical College &amp; Hospital with features of ovarian neoplasm from February 2013 to February 2014. Result: A total of 45 cases were studied in this present study. Patients were of age group 2nd to 7th decade with ovarian tumors. Among 45 cases majority had benign tumors (82.3%), followed by malignant tumors (15.5%). A case of borderline tumor (2.2%) was also found. Benign tumors were the commonest in the 3rd decade. The highest frequency of malignant tumors was in the 4th to 6th decade. Germ cell tumors were found at a young age and sex cord-stromal tumor was found in the 5th decade. Most of the benign tumors (67.56%) were cystic, the rest 27.03%, and 5.42% of cases were partly cystic and partly solid respectively. Among benign tumors, two of the mature teratomas were solid. About 42.86% of malignant tumors were solid, 42.86% were partly cystic partly solid and only 14.28% cases were cystic. Among the malignant tumors, serous cystadenocarcinoma was solid in 3 out of 4 cases and the rest of the malignant tumors of different histology were partly cystic and partly solid. Borderline Brenner tumor was solid in morphology. Most of the benign cases were unilateral 78.38% and the rest 21.62% were bilateral. Among the benign cases, serous cystadenoma showed the highest percentage covering 45.95% followed by mucinous cystadenoma at 18.92%. The borderline tumor showed unilateral distribution. Among malignant cases 57.14% were bilateral and 42.86% were unilateral. Conclusion: This study concludes that benign ovarian tumors are more common from the 3rd decade &amp; malignant tumors occur in the 4th to 6th decade. This study also showed that most of the benign tumors were cystic. Malignant ovarian tumors had an equal percentage of being solid entirely and partly cystic partly solid. Most benign cases were unilateral and malignant cases were mostly bilateral.

Ovarian sex cord tumor with annular tubules: case report and review of the literature

To report a case of ovarian sex cord tumor with annular tubules (SCTAT) and conduct a literature review on diagnosis, treatment and prognosis of this condition. Case report of a woman with a final diagnosis of advanced SCTAT seen at the National Cancer Institute in Bogota (Colombia) who received surgical treatment and chemotherapy with a satisfactory course after 6 months. A literature search was conducted in the Medline via PubMed, LILACS and Scielo databases, including case reports and series of women diagnosed with SCTAT published since 1990, not using age ranges. Information about diagnosis, treatment and reported prognosis was retrieved. A narrative summary of the findings was prepared. Fourteen publications with 26 patients were included. Mean age at diagnosis was 22.5 years. The main symptoms were menstruation abnormalities and pelvic pain. Computed tomography (CT) was the imaging technology most frequently used. Surgical treatment was used in all cases, together with chemotherapy in 29 %; 2 patients received radiotherapy. Recurrence occurred in 20 % of cases. Mortality was 12.5 %, with all deaths occurring within the first year. There is a paucity of information about the diagnostic utility of imaging, tumor markers and histochemical studies, as well as prognosis of this disease condition. Surgery is the treatment of choice, taking into consideration the patient’s wishes regarding fertility, as well as the stage of the tumor. Further studies are needed to provide more detailed information about this condition.

Depression and anxiety in women with malignant ovarian germ cell (MOGCT) and sex cord stromal tumors (SCST): an analysis of the AGO-CORSETT database

IntroductionThe intention of this study was to evaluate the level of anxiety and depression of malignant ovarian germ cell (MOGCT) and sex cord stromal tumors (SCST) survivors and to identify possible alterable cofactors.MethodsCORSETT was an observational, multicenter, mixed retrospective/prospective cohort study of the AGO Studygroup. Women who had been diagnosed with MOGCTs and SCSTs between 2001 and 2011 were asked to complete the Hospital Anxiety and Depression Scale (HADS) to evaluate distress. Predictors of distress (type of surgery, chemotherapy, time since diagnosis, recurrence, second tumor, pain) were investigated using multivariate linear regression analysis.Results150 MOGCT and SCST patients with confirmed histological diagnosis completed the questionnaire median seven years after diagnosis. They had a HADS total score ≥ 13 indicating severe mental distress in 34% of cases. Patients after fertility-conserving surgery had lower probability of severe mental distress than those without fertility-conserving treatment (β = − 3.1, p = 0.04). Pain was associated with the level of distress in uni- and multivariate analysis (coef 0.1, p < 0.01, coef. Beta 0.5).DiscussionSevere mental distress was frequent in patients with MOGCT and SCST and the level of pain was associated with the level of distress. Fertility conserving therapy, however, was associated with less mental distress. Screening and treatment of pain and depression is required to improve mental well-being in survivors of MOGCT and SCST.

New 2022 WHO classification of testicular tumours

The 5th edition of the WHO Classification of Tumours: Urinary and Male Genital Tumours includes some important revisions of the testicular tumour classifications, which are summarized in this article. They concern, for example, the definition of tumours of neuroectodermal and neuroendocrine origin. Furthermore, signet-ring stromal tumours and myoid gonadal stromal tumours are listed as separate entities in the section about sex-cord stromal tumours. Moreover, lymphatic neoplasia as well as soft tissue tumours of the urinary and male genital tract are each combined in acommon section.

Clinicopathological spectrum of ovarian neoplasms in pre and post menopausal Indian women

Ovarian cancers are the third most common cancers in women trailing behind cervical and breast cancer. Various molecular insults are seen in hereditary cancer syndromes that results in different morphological types of ovarian cancer.Accurate histological typing suggests screening of the first degree relatives and also targeted therapy for the same. Age specific incidence rate showed that ovarian cancer increases from 35 years of age and peaks between 55 to 64 years of age. Present study was conducted to know the clinical and histopathological spectrum of ovarian neoplasms. To correlate the histopathological subtype with the pre and post menopausal age group. This is a retrospective observational study of 196 cases found over 2.5 years during January 2018 to June 2020. Surface epithelial tumors(SET) are commonest in our study (benign 50%, borderline 6% and malignant 17%) followed by germ cell tumors (12%), sex cord stromal tumors (9%), secondary tumors (5%) and mixed epithelial and mesenchymal tumor (0.5%). Benign SET and germ cell tumors are significantly associated with premenopausal age whereas malignant SET has significant association with post menopausal age. However sex cord stromal tumors did not show any significant association with both the groups. Bilateral involvement was commonest with secondary tumors followed by malignant SET. Mean age of presentation was 41 years with maximum cases presenting in 4 and 5 decade. Various histomorphological parameters were evaluated in detail along with immunohistochemistry(IHC). Inhibin negativity helped to differentiate sertoliform variant of endometrial carcinoma from sertoli cell tumor. WT1 positivity in serous carcinoma ruled out poorly differentiated endometrioid carcinoma in two cases. Thirteen cases show extraovarian spread. Synchronous neoplasms of female genital tract (fibroma with uterine endometrial carcinoma and Brenner with basaloid carcinoma cervix ) were found. Collision tumors (sex cord stromal tumors with germ cell tumors) were also found in present study. Using WHO classification, ovarian tumors can be precisely subcategorized into different morphological subtypes. However overlapping features may need IHC to pinpoint the correct diagnosis. Age specific occurrence of different morphological subtypes may suggest other ancillary investigations and different treatment modalities

Could fertility-sparing surgery be considered for stage I ovarian sex cord-stromal tumors? A comparison of the Fine-Gray model with Cox model.

ObjectiveTo evaluate the oncologic outcomes in patients with International Federation of Gynecology and Obstetrics (FIGO) stage I ovarian sex cord-stromal tumors (SCSTs) who underwent fertility-sparing surgery (FSS) and the independent risk factors affecting overall survival (OS) and cancer-specific survival (CSS).MethodsData were acquired from the Surveillance, Epidemiology, and End Results (SEER) database between 1973 and 2018. A total of 240 patients diagnosed with stage I ovarian SCSTs were divided into the definitive surgery group (N=116) and FSS group (N=124). The Kaplan-Meier analysis and Cox model were used to evaluate the overall survival (OS) and cancer-specific survival (CSS) of the two groups and assess the independent risk factors respectively. The Fine-Gray model evaluated cancer-specific mortality (CSM) and the independent risk factors that affected CSM.ResultsKaplan-Meier survival analysis showed no statistically significant differences in OS and CSS between the two groups (P>0.05). Univariate analysis of the Fine-Gray model also showed that there was no difference in the CSS between the two groups (P>0.05). However, from the 15th year postoperatively, the CSS of the FSS group decreased by 13.21% compared with that of the control group and by 17.49% in the 20th and 25th years postoperatively. The Cox proportional hazards model found that surgical methods (“defined surgery” vs “FSS”; HR=0.03259, P=0.0196) and FIGO stage (“stage IA” vs “stage IC”; HR=0.03073, P=0.0300) were independent risk factors for OS. The multivariate analysis of Fine-Gray model showed that the cancer-specific mortality of patients receiving definitive surgery was 40.1% lower than that of patients receiving FSS (“definitive surgery” vs “FSS”; HR=0.599, P=0.005), indicating that FSS might lead to higher tumor-specific mortality and lower CSS. However, age, race, laterality, history, FIGO stage, and tumor size had no significant influence on the tumor-specific mortality (P>0.05).ConclusionsFSS is considered for patients with stage I SCSTs with reproductive needs, but the follow-up period should not be less than 15 years. For patients with stage IC disease, FSS should be selected carefully, and close follow-up is necessary. Perhaps, definitive surgery after birth is a means to improve long-term survival rates.

Diagnostic problems in testicular tumours in consultant pathology : A practical guide

The great variety of pathological patterns in germ cell tumours, especially in yolk sac tumours but also the possibility of somatic-type malignancies, can complicate daily diagnosis. For the correct diagnosis, knowledge of morphological aspects and additional immunohistochemical staining can be helpful. Also, rare entities like sex cord stromal tumours, tumours of testicular adnexa or mesenchymal tumours of the spermatic cord can be diagnostically challenging.

535P Long term quality of life after chemotherapy among nonepithelial ovarian cancer survivors: The case-control vivrovaire rare tumours study

Treatments (ttt) of non-epithelial rare germ cell tumors (GCT) and sex cord stromal tumors (SCST) are associated with long survival. The standard treatment mainly includes conservative surgery combined with chemotherapy (CT) [bleomycin, etoposide and cisplatin (BEP)] depending on stage and prognostic factors. As reported in testicular cancer survivors, BEP may induce late side effects with negative impact on quality of life (QOL). The French Rare Malignant Gynecological Tumors (TMRG)/GINECO case-control VIVROVAIRE Rare tumors study assessed long term QOL among survivors treated with BEP as compared to age-matched healthy women (HW). Non-epithelial ovarian cancer survivors (nEOCS), cancer-free ≥2 years after end of treatment, were identified from the INCa French Network for TMRG. HW were issued from the ‘Seintinelles’ research platform. QOL (FACT-G and FACT-O), chronic fatigue (MFI), anxiety/depression (HADS), insomnia (ISI), neurotoxicity (FACT/GOG-NTX), cognition (FACT-COG) and sexuality items (from the FACT-O OCS) were compared between nEOCS and HW. A minimal 5% difference for the score between groups was considered as clinically relevant. 144 nEOCS were included with 144 age-matched HW (mean age at inclusion: 38 yrs; 60% aged below 40). Most nEOCS were treated for GCT (n=112). Median delay from the end of ttt to inclusion was 6 yrs. At inclusion, 42% of nEOCS were menopausal versus 17% of HW (p<0.001). General and ovarian QOL, fatigue, anxiety/depression and insomnia scores were similar between nEOCS and HW. nEOCS reported clinically significant (6%) better social functioning (p=0.006). However, nEOCS reported more perceived cognitive impairment than HW (31 vs 14%, p<0.001) and clinically significant (8%) neurotoxicity (p<0.001). In addition, nEOCS reported less interest in sex (35% vs 55%, p<0.001), felt less like a woman (67% vs 81%, p=0.012), and were more afraid of not having children (31% vs 13%, p=0.007) than HW, whatever the menopausal status. 6 yrs after BEP CT, most of nEOCS reported similar global QOL as HW, but experienced more risk of premature menopause, some late side effects on cognition, neurotoxicity and sexuality that may impact their daily life.