Thoracic Sertoli-Leydig cell tumor: An alternative type of pleuropulmonary blastoma associated with DICER1 variation.

A 2-year-old boy presented with a large cystic and solid chest mass arising from the lung, radiographically consistent with pleuropulmonary blastoma (PPB). He underwent right lower lobectomy with resection of a well-circumscribed, mixed solid and cystic mass. The solid areas were composed of cords and nests of tumor cells in the myxoid stroma and retiform foci whose pathologic and immunophenotypic findings were consistent with a sex cord-stromal tumor with features of a Sertoli-Leydig cell tumor. Tumor testing showed a pathogenic variant in the DICER1 RNase IIIb hotspot domain. Family history was suggestive of DICER1 germline pathogenic DICER1 variation in absence of a detectable germline variant. He received 12 cycles of chemotherapy with ifosfamide, vincristine, dactinomycin and doxorubicin (IVADo) and surgery with complete response. One year after completion of chemotherapy, imaging studies showed concern for recurrence confirmed by thorascopic biopsy of a pleural-based mass. He is currently receiving cisplatin-based chemotherapy with reduction in tumor size. Review of the literature showed no similar cases; however, review of our pathology files revealed a single similar case of anterior mediastinal Sertoli cell tumor in a 3-year-old girl.

Ovarian Sertoli-Leydig Cell Tumor With Heterologous Hepatocytes and a Hepatocellular Carcinomatous Element.

Sertoli-Leydig cell tumors are a group of tumors composed of variable proportions of Sertoli cells, Leydig cells, and sometimes heterologous elements. We describe the case of a 68-yr-old woman who presented with abdominal distention. A computed tomographic scan revealed a large right adnexal mass without evidence of intrahepatic tumors, and a complete cytoreductive surgery was performed. Pathologic examination revealed a moderately differentiated Sertoli-Leydig cell tumor with various heterologous elements, including gastrointestinal-type glands, insular carcinoid, and aggregations of hepatocytes without significant cytologic atypia. Moreover, adjacent to these hepatocytes, extensive overgrowth of highly atypical hepatocyte-like cells, providing a striking morphologic similarity to hepatocellular carcinoma of the liver, was identified. Both the heterologous hepatocytes and hepatocellular carcinomatous tumor cells were immunohistochemically positive for alpha-fetoprotein, hepatocyte paraffin 1, and arginase-1. Some Sertoli cells adjacent to the heterologous hepatocytes were also positive for alpha-fetoprotein and hepatocyte paraffin 1. The present case showed that a tumor morphologically and immunohistochemically analogous to hepatocellular carcinoma of the liver can arise in the ovary, in association with Sertoli-Leydig cell tumors.

Pediatric Gynecologic Cancers.

PURPOSE OF REVIEW: Three primary categories of gynecologic cancer are found in pediatric and adolescent patients: stromal carcinomas including juvenile granulosa cell tumors and Sertoli-Leydig cell tumors, rhabdomyosarcomas arising from the vagina and cervix (sarcoma botryoides), and ovarian germ cell tumors which comprise a wide range of histologies. These entities are rare and treatment approaches have focused on decreasing late effects of chemotherapy treatment. Here, we review presentation, histologic classifications, diagnosis, and treatment recommendations for pediatric gynecologic cancers. RECENT FINDINGS: Event-free and overall survival for these cancers is high, and the goals of treatment are minimization of morbidity and preservation of fertility with unilateral salpingo-oophorectomies and limited staging. Surveillance of tumor markers after surgery is helpful in monitoring for disease progression and adjuvant chemotherapy is often reserved for patients at recurrence. Recent literature supports avoiding chemotherapy even in high-grade germ cell tumors in the pediatric population.

Ovarian sertoli-leydig cell tumors: A multicenter long-term clinicopathological analysis of 27 patients.

AIM: Information on the clinical behavior of ovarian Sertoli-Leydig cell tumors (SLCTs) as well as its prognostic factors and optimal management is limited due to a substantially low incidence of the disease. Also, limited data is available regarding the role of chemotherapy in the management of SLCTs. The aim of the study is to evaluate clinicopathological features and outcome of patients with ovarian SLCTs. MATERIALS AND METHODS: Twenty-seven patients with SLCT treated at two centers were reviewed retrospectively during 21 years. RESULTS: The median age was 45 years (range, 16-81) and the mean follow-up time was 86 months (range, 16-181). Twenty-three patients had stage IA, three patients had IC, and one patient had stage II disease. Eleven tumors (41%) were well-differentiated and 16 (59%) tumors were intermediately differentiated. Nine patients underwent unilateral salpino-oophorectomy and one patient, with a history of infertility, underwent cystectomy for fertility preservation. Eight patients with intermediately differentiated types of SLCT received adjuvant systemic chemotherapy including the combination bleomycin, etoposide, and cisplatin (BEP). Recurrence occurred in one patient with intermediated differentiated type SLCT with heterologous elements. She received four cycles of BEP chemotherapy. Twelve months later, she underwent cytoreductive surgery and received six cycles of cisplatin plus carboplatin. She died 24 months after the initial diagnosis. CONCLUSION: SLCTs of the ovary are usually in early stage, unilateral, and benign. Fertility-sparing surgery is the preferred option in young women. In the adjuvant treatment setting, although information about chemotherapy is limited, BEP is a commonly used regimen. The degree of differentiation and the presence of heterologous elements relate to a poor prognosis.

A Rare Case of Intra-Endometrial Leiomyoma of Uterus Simulating Degenerated Submucosal Leiomyoma Accompanied by a Large Sertoli-Leydig Cell Tumor.

A 50-year-old peri-menopausal woman presented with hard palpable mass on her lower abdomen and anemia from heavy menstrual bleeding. Ultrasonography showed a 13×12 cm sized hypoechoic solid mass in pelvis and a 2.5×2 cm hypoechoic cystic mass in uterine endometrium. Abdomino-pelvic computed tomography revealed a hypodense pelvic mass without enhancement, suggesting a leiomyoma of intraligamentary type or sex cord tumor of right ovary with submucosal myoma of uterus. Laparoscopy revealed a large Sertoli-Leydig cell tumor of right ovary with a very rare entity of intra-endometrial uterine leiomyoma accompanied by adenomyosis. The final diagnosis of ovarian sex-cord tumor (Sertoli-Leydig cell), stage Ia with intra-endometrial leiomyoma with adenomyosis, was made. Considering the large size of the tumor and poorly differentiated nature, 6 cycles of chemotherapy with Taxol and Carboplatin regimen were administered. There is neither evidence of major complications nor recurrence during 20 months' follow-up.

Tumor ovárico no epitelial: tumor de Sertoli-Leydig / Non-epithelial ovarian cancer: Sertoli-Leydig cell tumor

Los tumores de Sertoli-Leydig son un tipo infrecuente de tumor de ovario de estirpe no epitelial; concretamente, pertenecen al grupo de los tumores de los cordones sexuales-estroma, con una incidencia aproximada de un 2-5% de todas las neoplasias ováricas malignas. Presentamos el caso de una paciente de 42 años intervenida de un tumor de Sertoli con focos sarcomatoides. El diagnóstico de estos tumores es difícil de establecer prequirúrgicamente, y debido a la escasez de publicaciones, existe mucha controversia acerca de la estadificación quirúrgica idónea y sobre la conveniencia de realizar o no tratamiento adyuvante (AU)

Sertoli-Leydig cell tumors are a rare type of non-epithelial ovarian cancer; these tumors belong to the group of sex cord-stromal tumors and account for approximately 2-5% of all malignant ovarian tumors. We report the case of a 42-year-old woman who underwent surgery for a Sertoli tumor with sarcomatoid foci. Diagnosis of these tumors is difficult before surgery and, due to the scarcity of publications on the topic, there is controversy about the appropriate surgical staging and the role of adjuvant therapy in the management of this entity (AU)

Sertoli-Leydig cell tumor of the ovary: analysis of a single institution database.

AIM: To evaluate the clinicopathological features, management, survival and prognostic factors of patients with Sertoli-Leydig cell tumors of the ovary (SLCT) managed at a single institution. MATERIAL AND METHODS: The clinical records of patients with Sertoli-Leydig cell tumors of the ovary managed at the KK Women's and Children's Hospital, Singapore, between October 1998 and December 2008 were reviewed. Data of pathological features, treatment given and progress on follow-up was studied. RESULTS: Sertoli-Leydig cell tumor of the ovary accounted for 1.3% of malignant ovarian neoplasms. The median age of the patient was 30 years. The most common mode of presentation was with hormonal-related symptoms (80%) in the form of secondary amenorrhea, irregular menses and features of virilization. Thirteen of the 15 patients underwent surgical staging and all were found to have stage-I disease at the time of diagnosis. Ten patients with intermediate and poorly differentiated tumors received adjuvant bleomycin, etoposide and cisplatin (BEP) chemotherapy. Recurrent disease was detected in two patients (13.3%) during a median follow-up of 63 months, both of whom had poorly differentiated type of tumor. Both these patients underwent optimal debulking surgery followed by postoperative chemotherapy (BEP regimen). There were no disease -elated deaths and all patients were under complete remission at the last follow-up. CONCLUSION: As most Sertoli-Leydig cell tumors of the ovary are seen in young women and detected while still in the early stages, a favorable outcome can be achieved by conservative surgery. Patients with moderate and poorly differentiated types of tumors benefit from adjuvant chemotherapy. Recurrences tend to occur early and are commonly seen in patients with poorly differentiated tumors.

Ovarian Sertoli-Leydig cell tumor: a report of seven cases and a review of the literature.

The aim of this study was to investigate the clinicopathologic features, treatment and outcome of seven patients with an ovarian Sertoli-Leydig cell tumor (SLCT). Five patients presented with feminization, two with accompanying virilization. One presented with amenorrhea alone. Three of the five patients showing feminization symptoms had endocrine-related diseases. Histologically, five tumors were well differentiated, the other two were poorly differentiated. The latter two patients were misdiagnosed as having an ovarian epithelial carcinoma or granulosa cell tumor from frozen sections. Immunohistochemistry showed that the tumors were calretinin-positive in two patients and one was inhibin-positive. Four patients underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy(TAH/BSO) and two were treated by unilateral salpingo-oophorectomy. Among them, two patients received adjuvant chemotherapy. Six patients were free of disease in a follow-up of 2-34 years and one achieved a pregnancy. The remaining patient recurred 4 years later. Feminization as well as virilization might provide important clues for a preoperative diagnosis. Histological misdiagnosis is probable in poorly differentiated tumors. Conservative surgery including retention of fertility can be considered. However, the tendency for recurrence in poorly differentiated tumors should be considered.

Treatment of high risk Sertoli-Leydig cell tumors of the ovary using a gonadotropin releasing hormone (GnRH) analog.

Sertoli-Leydig cell tumors are rare ovarian neoplasms. We report two unusual cases with bilateral SLCTs suggesting evidence of genetic predisposition and at high risk of recurrence. To reduce this risk, we exploited the use of GnRH analog to lower gondadotropin and potentially directly inhibit the tumors through expressed GnRH receptors. We used it as maintenance antitumor therapy for 2 years after completion of chemotherapy, to cover the period of risk for recurrence. Both patients remain in complete remission at >2 years after completing leuprorelin therapy. Of note, both patients carry DICER1 mutations, frequently found in pleuropulmonary blastoma syndrome.

Ovarian Sertoli-Leydig cell tumors. a retrospective MITO study.

OBJECTIVE: To evaluate clinicopathologic features and to investigate the outcome of patients with ovarian Sertoli-Leydig cell tumors (SLCTs). METHODS: Data concerning 21 patients treated in 11 MITO centers were retrospectively reviewed. RESULTS: Median age was 37 (range 16-76). FIGO stage was: 17 (81%) IA, 1 (4.8%) IC, 1 (4.8%) IIB and 2 (9.5%) IIIC. Five patients (23.8%) had G1 tumor, ten (47.6%) had G2, and six (28.6%) had G3. Fertility-sparing operation was performed in 11 patients, while hysterectomy with bilateral salpingo-oophorectomy was executed in 10 patients; five patients received adjuvant chemotherapy (G2-3). Seven patients (33.3%) relapsed with a median time to recurrence of 14 months. Six recurrent patients had G2-3 disease, while one had G1. Four patients had stage IA disease, one IC and 2 stage IIIC. Patients with stage IA disease did not receive adjuvant chemotherapy. Two patients had pelvic recurrence, 4 abdominal (one with lymph nodal involvement), one on the contralateral ovary and the trocar access. Five patients underwent salvage surgery plus chemotherapy, while one received only salvage chemotherapy and one palliation. Five patients died of disease, four had received first treatment not in a MITO center. 5 year overall survival was 100% for patients with G1 disease and 77.8% for G2-3. 5 year overall survival was 92.3% for stage I and 33.3% for stage>I. CONCLUSIONS: The prognosis of patients with grade 1 SLCT is excellent without adjuvant chemotherapy. Patients with advanced stage or grade 2-3 tumors appear to benefit from postoperative chemotherapy.

[Malignant arrhenoblastoma. Case report and literature review]. / Arrenoblastoma maligno. Caso clínico y revisión bibliográbfica.

The arrenoblastome is an ovary tumor with masculine hormone production, testosterone and other hormones. Other names are: stromatic tumor or gonadal stromatic tumor, also steroid cell tumor. They are rare tumors; represent 0.5% of all ovary tumors. It could be present in all age women groups, more frequently in young people. Most of times unilateral (95%), solids or quistic-solids. Anaplastic grade give them a malignity disease in 5 to 10 % cases. We report the case of a 35 year-old woman with clinical appearance of androgenism for ovary tumor, she was accepted for surgery, founded 7 liters of ascitis, produced for an ovary tumor, integral capsule, it produced masculine hormones. Histological study reported ovarian sex cord tumor, high grade, 30 cm size, integral capsule, all normally. Stage IC. Size and differential cellular grade need systemic chemotherapy. At the time of this report her tumoral marks are normal, and she has gradual diminution of virilizing characters produced for ovary tumor. Prognosis of the disease depends the grade of cell differentiation and stage in surgical-pathological events. Survival to five years stage I is approximate in 70 to 90% of the cases. Angular stone treatment is surgery. Disseminate cases, chemotherapy or radiotherapy most be considerate. Usually arrenoblastome has poor possibilities of dissemination and considering the early detection the histological grade of healthy is very high.

An unusual case of membranous nephropathy associated with an ovarian tumor.

Secondary membranous nephropathy (MN) associated with malignancy is not uncommon in adults, but it is rare in children. We report a 6-year-old girl who developed nephrotic-range proteinuria following diagnosis of a Sertoli-Leydig ovarian tumor. A renal biopsy was performed, which led to the diagnosis of MN. The patient maintained normal renal function and gradually showed improvement in proteinuria over several months without the use of corticosteroids or angiotensin-converting enzyme inhibitors. Our case highlights the importance of performing screening urinalyses in children with tumors to recognize the presence of clinically significant, but potentially asymptomatic kidney disease.

[Clinical analysis of 11 cases of ovarian Setoli-Leydig cell tumor].

OBJECTIVE: To study the clinical characteristics, treatment and prognostic factors of ovarian Setoli-Leydig cell tumor. METHODS: During 1962 - 2002, a total of 11 patients with Setoli-Leydig cell tumor were retrospectively analyzed. RESULTS: Microscopically, seven of the neoplasms were well differentiated, 3 were moderately differentiated and 1 was poorly differentiated. Nine of the tumors were stage Ia, 1 was stage IIc and 1 was stage IIIc. The most frequent symptoms were abdominal-pelvic masses. Six patients presented with androgenization and virilization, 3 of which had their serum testosterone tested, and the levels were elevated. Five patients presented with metromenorrhagia and abnormal vaginal bleeding. One of them was both androgenized and estrogenized. In addition, five patients had diseases associated with excessive estrogenic stimulation, such as uterine myoma and endometrial hyperplasia. Two patients suffered from breast cancer. All patients were subjected to operation. And 5 patients with poorly differentiated or stage II-III tumors were subjected to postoperational chemotherapy. After 6 months to 34 years follow-up, no patient died of this disease. Three patients who received conservative surgery achieved normal menstruation 1 - 3 months after operation, and one of them gave birth to a child. CONCLUSIONS: Ovarian Setoli-Leydig cell tumor has good prognosis. Surgery alone is a currently acceptable treatment for patients with well-differentiated early stage tumors. For patients with poorly differentiated or advanced tumors, postoperational chemotherapy seems to be necessary. Conservative surgery should be the treatment of choice in young patients who need future fertility.

Sertoli-Leydig cell tumor of the ovary.

Sertoli-Leydig cell tumors of the ovary are rare diseases that occur primarily in young women. The majority of these tumors are unilaterally localized, and conservative surgery is sufficient. However, these tumors exhibit a variety of histological patterns, which are significant prognostic factors. To date, no standard therapy exists. Here we report 4 cases of Sertoli-Leydig cell tumors of the ovary. One patient whose tumor was a poorly differentiated Sertoli-Leydig cell tumor with mesenchymal heterologous elements received adjuvant chemotherapy postoperatively but died of disease 2.5 years after surgery. The other 3 patients remained free of disease during follow-up. Conservative surgery is an appropriate treatment for young patients with Sertoli-Leydig cell tumors. Those who have poor prognostic factors may need adjuvant chemotherapy with a combination of bleomycin, etoposide and cisplatin.

Tumor de celulas de sertoli y leydig del ovario

Se reporta el caso de una paciente de 16 años con un tumor de células de Sertoli y Leydig del ovario en etapa I tratado con cirugía exclusiva. Además, se realiza una revisión de la literatura sobre este tipo de tumor.