Pediatric primary extra-ovarian Sertoli Leydig cell tumor of the retroperitoneum

Ovarian Sertoli Leydig cell tumors (SLCT) accounts for less than 0.5% of all ovarian malignancies. The incidence of primary extra-ovarian SLCT is extremely rare with reported cases occurring in young adult women till now. We report case of primary retroperitoneal extra-ovarian SLCT in a seven-year girl child without any hormonal manifestation. She presented with complaint of left side abdominal swelling associated with intermittent pain for a duration of six months. CT scan revealed a huge retroperitoneal space-occupying lesion abutting the dorsal vertebrae and present posterior to pancreas, spleen and left kidney. The tumor was diagnosed as extraovarian Sertoli Leydig cell tumor with intermediate differentiation on histopathology and immunohistochemistry.

Tumor de células de Sertoli-Leydig: a propósito de un caso y revisión de la literatura reciente / Ovarian Sertoli-Leydig cell tumour: a clinical case and recent basic literature review

INTRODUCCIÓN: Los tumores de células de Sertoli-Leydig son neoplasias de ovario infrecuentes, lo que dificulta su diagnóstico y tratamiento. OBJETIVO: Revisar y sintetizar el manejo actual de los tumores de células de Sertoli-Leydig. MÉTODO: Se realizó una revisión de la literatura reciente sobre tumores de células de Sertoli-Leydig, a propósito de un caso en nuestro centro. RESULTADOS: Los tumores de las células de Sertoli-Leydig son infrecuentes, con mayor incidencia en edades tempranas. Ante una paciente joven con una lesión anexial unilateral y signos de virilización deberán considerarse estos tumores dentro del diagnóstico diferencial. En los estadios iniciales y en pacientes jóvenes podrá plantearse un tratamiento quirúrgico que preserve la fertilidad, y la asociación de tratamiento adyuvante dependerá de la diferenciación y del estadiaje del tumor.

INTRODUCTION: Sertoli-Leydig cell tumors are infrequent ovarian neoplasms, which difficults their diagnosis and treatment. Objective: To review and synthesize the current management of the Sertoli-Leydig cell tumor. METHOD: A review of the recent literature regarding the Sertoli-Leydig cell tumor was carried out, regarding a case in our center. RESULTS: Sertoli-Leydig cell tumors are an infrequent entity, with a higher incidence in early ages. In a young patient with a unilateral adnexal lesion and signs of virilization, these tumors should be considered within the differential diagnosis. In early stages and young patients, a surgical treatment that preserves fertility may be considered, and the association of adjuvant treatment will depend on the differentiation and staging of the tumor.

Sex cord stromal tumor of ovary masquerading as polycystic ovarian syndrome

Virilization is a portentous sign that suggests the possibility of an ovarian or adrenal neoplasm. Diagnosis may be delayed in some patients due to nonspecific symptoms and overlapping symptoms with that of polycystic ovarian syndrome (PCOS). However, it must be remembered that PCOS usually causes mild to moderate elevation of serum testosterone with hirsutism whereas serum testosterone levels are many times elevated in cases of androgen secreting tumors and virilization is a norm. So high testosterone level with new onset virilization rule out PCOS. Authors are reporting two cases of Sertoli Leydig cell tumor despite their similar histopathology and equivalent levels of serum testosterone had a varied clinical spectrum of virilization.

Androgenic alopecia in post-menopausal female: a clinical presentation

Androgenic alopecia is a patterned hair loss occurring due to systemic androgens and genetic factors. It is the most common cause of hair loss in both genders. The appearance of this condition is the cause of significant stress and psychological problems, making appropriate management important. A 68-year-old postmenopausal female presented with complaints of increased hair loss from scalp, excessive hair growth at undesired sites and hirsutism not corrected with medications. On thorough investigations, CT scan whole abdomen and endocrinological workup, a clinical diagnosis of alopecia and hirsutism due to hyperandrogenemia secondary to ovarian tumor made. Abdominal hysterectomy with B/L salpingo-oophorectomy was done. Histopathological examination revealed an encapsulated tumor in right ovary-sex cord stromal tumor consistent with Leydig cell tumor in right ovary, no evidence of malignancy. Left ovary was normal. Patient showed significant regression of clinical signs and symptoms on follow up after 1 month. All women with severe hirsutism or androgenic alopecia needs further work up to locate the source of androgen over production.

Ovarian sertoli-leydig cell tumors: epidemiological, clinical and prognostic factors / Tumores de células de Sertoli-Leydig ovarianos: fatores epidemiológicos, clínicos e prognósticos

Abstract Objective To describe a series of cases of ovarian Sertoli-Leydig cell tumors (SLCTs). Methods Retrospective review of 12 cases of SLCT treated at the Hospital do Câncer de Barretos, Barretos, state of São Paulo, Brazil, between October 2009 and August 2017. Results The median age of the patients was 31 years old (15-71 years old). A total of 9 patients (75.0%) presented symptoms: 8 (66.7%) presented with abdominal pain, 5 (41.7%) presented with abdominal enlargement, 2 (16.7%) presentedwith virilizing signs, 2 (16.7%) presented with abnormal uterine bleeding, 1 (8.3%) presented with dyspareunia, and 1 (8.3%) presented with weight loss. The median preoperative lactate dehydrogenase (LDH) was 504.5 U/L (138-569 U/L), alpha-fetoprotein (AFP) was 2.0 ng/ml (1.1-11.3 ng/ml), human chorionic gonadotropin (β-hCG) was 0.6 mUI/ml (0.0-2.3 mUI/ml), carcinoembryonic antigen (CEA) was 0.9 ng/ml (0.7-3.4 ng/ml), and cancer antigen 125 (CA-125) was 26.0 U/ml (19.1-147.0 U/ml). All of the tumors were unilateral and surgically treated. Lymphadenectomy was performed in 3 (25.0%) patients, but none of the three patients submitted to lymphadenectomy presented lymph node involvement. In the anatomopathological exam, 1 (8.3%) tumor was well-differentiated, 8 (66.7%) were moderately differentiated, and 3 (25.0%) were poorly differentiated. A total of 5 (55.6%) tumors were solid-cystic, 2 (22.2%) were purely cystic, 1 (11.1%) was cystic with vegetations, and 1 (11.1%) was purely solid, but for 3 patients this information was not available. The median lesion size was 14.2 cm (3.2-23.5 cm). All of the tumors were at stage IA of the 2014 classification of the International Federation ofGynecology andObstetrics (FIGO). A total of 2 (16.7%) patients received adjuvant treatment; 1 of themunderwent 3 cycles of paclitaxel and carboplatin every 21days, and the other underwent 4 cycles of ifosfamide, cisplatin and etoposide every 21 days. None of all of the patients had recurrence, and one death related to complications after surgical staging occurred. Conclusion Abdominal pain was the most frequent presentation. There was no ultrasonographic pattern. All of the SLCTs were at stage IA, and most of them were moderately differentiated. Relapses did not occur, but one death related to the surgical staging occurred.

Resumo Objetivo Descrever uma série de casos de tumores de células de Sertoli-Leydig (TCSLs) ovarianos. Métodos Revisão retrospectiva de 12 casos de TCSL tratados no Hospital de Câncer de Barretos entre outubro de 2009 e agosto de 2017. Resultados A mediana de idade foi 31 anos (15-71 anos). Um total de 9 pacientes (75,0%) apresentaram sintomas: 8 (66,7%) apresentaram dor abdominal, 5 (41,7%) apresentaram aumento abdominal, 2 (16,7%) apresentaram virilização, 2 (16,7%) apresentaram sangramento uterino anormal, 1 (8,3%) apresentou dispareunia, e 1 (8,3%) apresentou emagrecimento. A mediana de desidrogenase láctica (DHL) foi 504,5 U/L (138-569 U/L), alfafetoproteína (AFP) foi 2,0 ng/ml (1,1-11,3 ng/ml), gonadotrofina coriônica humana (β-hCG) foi 0,6 mUI/ml (0,0-2,3 mUI/ml), antígeno carcinoembrionário (CEA) foi 0,9 ng/ml (0,7-3,4) ng/ml, e antígeno cancerígeno 125 (CA-125) foi 26,0 U/ml (19,1-147,0 U/ml), todos pré-operatórios. Todos os tumores foram unilaterais e tratados cirurgicamente. Realizou-se linfadenectomia em 3 (25,0%) pacientes, por em, nenhuma das tr^es apresentou acometimento linfonodal. No exame anatomopatológico, 1 tumor (8,3%) era bem diferenciado, 8 (66,7%) eram moderadamente diferenciados, e 3 (25,0%) eram pouco diferenciados. Um total de 5 (55,6%) tumores eram sólido-císticos, 2 (22,2%) eram puramente císticos, 1 (11,1%) era cístico com vegetações, e 1 (11,1%) era puramente sólido, mas para 3 pacientes estas informações não estavam disponíveis. A mediana da dimensão da lesão foi 14,2 cm (3,2-23,5 cm). Todos os tumores eram estádio IA de acordo com a classificação de 2014 da Federação Internacional de Ginecologia e Obstetrícia (FIGO, na sigla em inglês). Duas (16,7%) pacientes receberam adjuvância; uma realizou 3 ciclos de paclitaxel e carboplatina a cada 21 dias, e a outra 4 ciclos de ifosfamida, cisplatina e etoposide a cada 21 dias. Dentre todas as pacientes, nenhuma apresentou recidiva e houve um óbito relacionado a complicações após estadiamento cirúrgico. Conclusão Dor abdominal foi a apresentação mais frequente. Todos os TCSLs eram estádio IA e a maioria era moderadamente diferenciada. Não ocorreram recidivas, mas ocorreu um óbito relacionado ao estadiamento cirúrgico.

Diagnosis of an indistinct Leydig cell tumor by positron emission tomography-computed tomography

A 51-year-old perimenopausal female patient presented with hirsutism and voice thickening which was started approximately one and a half years ago. Her initial hormone assay revealed elevated plasma testosterone, 5a-dihydrotestosterone, and dehydroepiandrosterone (DHEA) levels and therefore androgen-secreting tumor was first suspected. However, the lesion was inconspicuous on transvaginal sonography, abdominal-pelvic computed tomography (CT) scan, and pelvic magnetic resonance (MRI) imaging. Consequently, 18F-fluorodeoxyglucose (FDG) positron emission tomography-CT was performed, which localized the lesion as a focal FDG uptake within the right adnexa. Total laparoscopic hysterectomy with bilateral salpingo-oophorectomy was performed, and although visible gross mass lesions were not observed intraoperatively, pure Leydig cell tumor was pathologically confirmed within the right ovary. Plasma testosterone, 5a-dihydrotestosterone, and DHEA levels were normalized postoperatively. Clinical signs of virilization were also significantly resolved after 3-months of follow-up.

CT findings of ovarian sertoli-leydig cell tumor / 实用放射学杂志

Objective To study the CT findings of ovarian sertoli-leydig cell tumor (SLCT),to improve the understanding of SLCT imaging features.Methods CT images of 6 cases were reviewed retrospectively.CT plain scan was performed in 6 patients;CT enhancement scan was performed in 4 patients.Results All tumors were unilateral.On non-enhanced CT,5 cases showed cystic-solid masses with clear margin,and 1 case showed solid mass.After contrast administration,the cystic walls,septum and solid portions showed progressively obvious enhancement.Of these 6 cases, 3 cases showed increased testosterone levels,2 cases had high prolactin level,and 1 case was with raised AFP in plasma.Positive expressions of immunohistochemical markers were as following:Inhibin-α (6 cases),Vimentin (4 cases),Calretinin (2 cases), CD99 (1 case), Ki-67 (1 case), CK20 (1 case).Conclusion CT findings of SLCT have some certain characteristics, especially a unilateral, cystic-solid and well-defined mass, and marked solid part enhancement on contrast CT.Combined with clinical findings, especially increased plasma testosterone levels, SLCT should be included in the differential diagnosis of regional tumors.

A novel clinicopathological analysis of early stage ovarian Sertoli-Leydig cell tumors at a single institution

OBJECTIVE: To evaluate the clinical and pathologic characteristics of patients who were diagnosed with ovarian Sertoli-Leydig cell tumors (SLCTs) in a single institution. METHODS: The medical records of 11 patients who were pathologically diagnosed with SLCTs beginning in 1995 in a single institute was reviewed. RESULTS: The median patient age was 31 years (range, 16 to 70 years). Patient International Federation of Gynecology and Obstetrics stages were IA, IC, and IIB in 3 (27.3%), 6 (54.5%), and 2 (18.2%) patients, respectively. Six patients (54.5%) had grade 3 tumors, 3 patients (27.3%) had grade 2 tumors, and 1 patient (9.1%) had a grade 1 tumor. Four patients without children underwent fertility-sparing surgery, and 7 patients had full staging surgery, including a hysterectomy and bilateral salpingo-oophorectomy, with a laparoscopic approach used in 3. Eight patients underwent pelvic lymph node dissection, and 8 patients were administered adjuvant chemotherapy consisting of bleomycin, etoposide, and cisplatin in 6 cases, a modified bleomycin, etoposide, and cisplatin regimen in 1 case, and a combined paclitaxel and cisplatin regimen in 1 case. Two patients died of disease and were re-diagnosed with Sertoli form endometrioid carcinoma. The other patients remain alive without recurrence at the time of reporting. CONCLUSION: Our findings suggest that regardless of tumor stage or grade, ovarian SLCT patients have a good prognosis. Close observation and unilateral salpingo-oophorectomy would be beneficial for women who still wish to have children, while hysterectomy and bilateral salpingo-oophorectomy with adjuvant chemotherapy would be the optimal treatment in other cases. Furthermore, meticulous pathologic diagnosis is needed to develop a precise treatment strategy.

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 13x12 cm sized hypoechoic solid mass in pelvis and a 2.5x2 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 de células de Sertoli-Leydig: a propósito de un caso

Se comunica el caso de una paciente de 53 años con presencia de tumor de Sertoli-Leiydig de ovario, estadio clínico I, quien recibió tratamiento quirúrgico. Dada la baja frecuencia de este tipo de tumores, se realiza una revisión de la evidencia disponible en la literatura, a fin de sugerir algunas pautas para el manejo de esta rara entidad.

The case of a fifty-three years old woman with a Sertoli-Leydig cell tumor stage I is reported; she was treated with surgery alone. A review of the literature on this rare type of tumor is done and recommendations on its treatment is considered.

A case of successful salvage chemotherapy of recurrent ovarian sertoli-leydig cell tumor

Sertoli-Leydig tumors tend to relapse early and due to their rarity, limited data are available regarding a role of chemotherapy in the management of Sertoli-Leydig cell tumors. We present a case of recurrent ovarian Sertoli-Leydig cell tumor whose salvage treatment was successful with paclitaxel and carboplatin chemotherapy.

Tumor de células de Sertoli calcificante, de células grandes: caso clínico / Large cell calcifying Sertoli cell tumor: Report of one case

Sertoli Cell Tumors are less than 1 percent of all testicular tumors. We report a 14-year-old male presenting with a left testicular mass. Orchiectomy was carried out and the pathological study informed a large cell calcifying Sertoli cell tumor (LCCSCT). Its association with the Carney complex and Peutz-Jeghers Syndrome was subsequently discarded. Surgical excision was completed with a radical orchiectomy. After eight months of follow up, there is no evidence of tumor relapse.

Sertoli-Leydig Cell Tumor of the Ovary in a 4 year-old Girl: A Case Report

Sertoli-Leydig cell tumor is a rare sex-cord stromal tumor of the ovary. They make up less than 0.5 % of all ovarian tumors. We experienced a case of an ovarian Sertoli-Leydig cell tumor in a 4 year-old girl who presented with nausea, vomiting, and lower abdominal pain of 2 days' duration. On physical examination, there was mild tenderness in the right lower quadrant of the abdomen. Abdominal ultrasonography and computed tomography (CT) scan revealed a pelvic mass measuring 5 x 3 cm that appeared to arise from the right ovary. At exploratory laparotomy, a 6 x 5 x 3 cm solid right ovarian mass without torsion was found. A right salpingo-oophorectomy was performed. The postoperative course was uneventful. The child was discharged 5 days after surgery.

A case of Sertoli-Leydig cell tumor with mature cystic teratoma in a postmenopausal woman / 대한산부인과학회지

Sertoli-Leydig cell tumor is a gonadal tumor of the sex cord-stromal type. It is a rare tumor comprising 0.5% of all ovarian tumors and is best known for their frequent virilizing effects among the young women. They also have very rarely been reported in association with other ovarian neoplasms. We report such a tumor accompanied with a mature cystic teratoma at the same ovary in a 61-year-old postmenopausal woman without any virilization sign. We present it with brief review of literatures.

A case of Sertoli-Leydig cell tumor with mucinous heterologous element in a postmenopausal woman / 대한산부인과학회지

Ovarian Sertoli-Leydig cell tumors are rare sex cordstromal tumors, and these neoplasms account for less than 0.5% of all ovarian tumors. Those are more often encountered in young women between the ages of 20 and 30 years who usually become virilized. Recently, we experienced an unusual case of Sertoli-Leydig cell tumor with mucinous heterologous elements in a 71-year-old postmenopauseal woman. We present it with brief review of literatures.

A case of successful laparoscopic surgical staging in patient of Sertoli-Leydig cell tumor / 대한산부인과학회지

Sertoli-Leydig cell tumors are rare sex cord-stromal tumors. These tumors account for less than 0.5% of all ovarian tumors. Because these tumors appear predominantly in young women (between the age of 30 and 40) and are bilateral in less than 1% of cases, conservative removal of the tumor and adjacent fallopian tube is justifiable. Recently, we experienced a case of successful laparoscopic surgical staging in patient of Sertoli-Leydig cell tumor which was previously incompletely evaluated in local OBGY clinic. There are no solid data to suggest that usefulness and risk of laparoscopic surgical staging and adjuvant therapy with stage I disease of these tumors until now. So we present it with a brief review of literature.

A Sertoli-Leydig Cell Tumor in a Patient with Complete Androgen Insensitivity Syndrome: A Case Report

Complete androgen insensitivity syndrome (CAIS) is a rare disorder sometimes referred to as male pseudohermaphroditism. This syndrome is characterized by a 46,XY karyotype with internal bilateral testes, female external genitalia, absence or scantiness of pubic and axillary hair and normal breast development. A variety of gonadal tumors can develop in individuals with CAIS. A 57-year-old patient was admitted to urology with intermittent gross hematuria for one month. CT revealed a left ureteric mass, measuring 2.5 x 2 cm. Ureteroscopic biopsy was performed and the result was a diagnosis of high grade urothelial carcinoma. The patient underwent laparotomy which revealed slightly enlarged gonads in the pelvic cavity. However, a uterus and fallopian tubes were not identified. Left nephroureterectomy and bilateral gonadectomy were performed. The right gonad measured 3.9 x 2.5 x 2 cm and the cut surface showed a well circumscribed, firm, brownish gray colored 2 x 1.8 cm sized nodule. Microscopically, this nodule consisted of uniform and solid tubules containing immature Sertoli cells and Leydig cells which were both positive for alpha-inhibin. The left gonad measured 3.5 x 2 x 1.9 cm and showed atrophic testicular tissue.

Three Cases of Sertoli-Leydig Cell Tumor / 대한산부인과학회지

Sertoli-Leydig cell tumors of the ovary are one of the sex cord-stromal tumors. They are very rare, account for less than 0.5% of all ovarian tumor. These tumors are almost unilateral, and occur predominantly at premenopausal state and rarely at postmenopausal and prepubertal. The most specific symptoms are virilization due to hyperandrogenism but it presents only in 40-50% of the patients, and vast majority of the patients complain for non-specific abdominal symptoms and menstrual disorder including vaginal bleeding. Prognosis and treatment modalities for Sertoli-Leydig cell tumors remain controversial as they are rare and only a few studies have been published. Treatment varies with patients age, tumor stage, and differentiation from unilateral salpingo-oophrectomy and total hysterectomy concomitant with pelvic lymph node dissection, and in occasion, adjuvant chemotherapy. Prognosis is generally favorable with 5-year survival rate of 70-90%. Recurrence is rare. We have experienced three cases of Sertoli-Leydig cell tumor, each of them showed different clinical presentation and histologic findings, so we present them with a brief review of literature.

Two Cases of Sertoli-Leydig Cell Tumor of Ovary / 대한산부인과학회잡지

The Sertoli-Leydig cell tumor is a rare sex cord stromal tumor of the ovary. It is the most common type of all virillizing ovarian tumors, accounting for less than 0.5% of all primary ovarian neoplasm. Recently, we experienced two cases of intermediately differentiated Sertoli-Leydig cell tumor with amenorrhea and so we present it with brief review of literature.

Two Cases of Sertoli-Leydig Cell Tumor of Ovary / 대한산부인과학회잡지

The Sertoli-Leydig cell tumor is a rare sex cord stromal tumor of the ovary. It is the most common type of all virillizing ovarian tumors, accounting for less than 0.5% of all primary ovarian neoplasm. Recently, we experienced two cases of intermediately differentiated Sertoli-Leydig cell tumor with amenorrhea and so we present it with brief review of literature.