Lessons from genomic profiling: towards a molecular-based classification of ovarian Sertoli-Leydig cell tumour.

Currently available molecular data support a dichotomous classification of Sertoli-Leydig cell tumours (SLCTs) based on DICER1 mutational status. This correspondence suggests a possible roadmap towards a molecular-based classification of SLCT.

Outcomes in ovarian Sertoli-Leydig cell tumor: A report from the International Pleuropulmonary Blastoma/DICER1 and Ovarian and Testicular Stromal Tumor Registries.

OBJECTIVE: Sertoli-Leydig cell tumors (SLCTs) are rare sex cord-stromal tumors, representing <0.5% of all ovarian tumors. We sought to describe prognostic factors, treatment and outcomes for individuals with ovarian SLCT. METHODS: Individuals with SLCT were enrolled in the International Pleuropulmonary Blastoma/DICER1 Registry and/or the International Ovarian and Testicular Stromal Tumor Registry. Medical records were systematically abstracted, and pathology was centrally reviewed when available. RESULTS: In total, 191 participants with ovarian SLCT enrolled, with most (92%, 175/191) presenting with FIGO stage I disease. Germline DICER1 results were available for 156 patients; of these 58% had a pathogenic or likely pathogenic germline variant. Somatic (tumor) DICER1 testing showed RNase IIIb hotspot variants in 97% (88/91) of intermediately and poorly differentiated tumors. Adjuvant chemotherapy was administered in 40% (77/191) of cases, and among these, nearly all patients received platinum-based regimens (95%, 73/77), and 30% (23/77) received regimens that included an alkylating agent. Three-year recurrence-free survival for patients with stage IA tumors was 93.6% (95% CI: 88.2-99.3%) compared to 67.1% (95% CI: 55.2-81.6%) for all stage IC and 60.6% (95% CI: 40.3-91.0%) for stage II-IV (p < .001) tumors. Among patients with FIGO stage I tumors, those with mesenchymal heterologous elements treated with surgery alone were at higher risk for recurrence (HR: 74.18, 95% CI: 17.99-305.85). CONCLUSION: Most individuals with SLCT fare well, though specific risk factors such as mesenchymal heterologous elements are associated with poor prognosis. We also highlight the role of DICER1 surveillance in early detection of SLCT, facilitating stage IA resection.

A sub-hepatic nodule in a young female: Chase the case.

Sertoli-Leydig cell tumours (SLCTs) are rare, mixed sex-cord stromal tumours composed of varying proportions of both Sertoli and Leydig cells, which account for <0.5% of all ovarian tumours. The cytomorphologic features of SLCTs are not well described in literature. Herein, we describe the cytomorphologic features of an SLCT at an uncommon metastatic site in a young female. Sertoli-Leydig cell tumours (SLCTs) are rare, mixed sex-cord stromal tumours composed of varying proportions of both Sertoli and Leydig cells, which account for <0.5% of all ovarian tumours. The cytomorphologic features of SLCTs are not well described in literature. Herein, we describe the cytomorphologic features of an SLCT at an uncommon metastatic site in a young female.

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.

Arrenoblastoma

Presentamos un caso de Arrenoblastoma (tumor de células de Sertoly-Leydig) en una paciente de 16 años de edad que concurrió con un cuadro clínico de masculinización, lo que ocurre en el 75% de estos tumores. El objetivo de este reporte es presentar un caso de neoplasia rara entre los tumores ováricos y analizar las características clínico-patológicas

Tumor virilizante do ovário: a respeito de um caso clínico / Virilizing tumor od the ovary: a case report

A case of androblastoma, functioning, intermediary type, is related. The tumor was present on a 25 year old woman who presented charactheristics of defeminization and masculinization. The hormonal count of androgen was normal. The 17-OHCS were elevated. The patient was submited to total abdominal hysterectomy, bilateral salpingo-ooferectomy and omentectomy. Some endocrine aspects of this tumor are commented

Tumor de células de Sertoli-Leydig do ovário: apresentação de um caso e revisão da literatura / Ovary tumor by Sertoli-Leydig cells tumor: a case report and review of the literature

Os autores relatam um caso de tumor de células de Sertoli e Leydig (TCSL), estádio Ia, no ovário direito de uma paciente com 10 anos de idade. O tumor era não funcionante e as manifestações iniciais foram dor e aumento do volume abdominal. Ao exame físico palpava-se uma massa endurecida na região hipogástrica. A criança não apresentava sinais de virilização ou puberdade precoce. O tratamento foi realizado com cirurgia: salpingo-ooforectomia direita e biópsia em cunha do ovário esquerdo. Histologicamente o tumor era bem diferenciado e não apresentava elementos heterólogos. A paciente apresenta há 26 meses sobrevida livre de doença. Acreditamos que este seja o primeiro caso de TCSL de ovário em crianças com idade igual ou inferior a 10 anos, publicado na literatura médica brasileira indexada.

Tumor ovariano de células de Sertoli: relato de caso / Ovarian Sertoli cell tumor: a case report

A case of Sertoli cells ovarian tumor in a 55 year old patient is reported. Some comments are made on this type of tumor. The tumor reported was very differentiated and non functional. The surgical indication was made on the clinical data, and a bilateral ooforectomy was performed.

Tumor de Sertoli y Leydig: caso clínico / Sertoli-Leydig tumor: clinical case

We report a 16 year old girl presenting with secondary amenorrhea, a history of voice coarsening, hirsutism and a body mass index of 35 kg/m2. Pelvic ultrasound and CT scans showed a retro uterine dense mass. She was operated and a left ovarian tumor was excised. Pathological examination disclosed a Sertoli-Leydig tumor

Tumor de Sertoli-Leydig do ovário com padräo retiforme: apresentaçäo de um caso / Sertoli-Leydig tumor of the ovary with retiform pattern: case report

É apresentado um caso de Tumor de Sertoli-Leydig do ovário, ocorrendo numa criança de cinco anos, apresentando-se clinicamente como um quadro de abdome agudo por rutura espontânea da tumoraçäo. Chama-se a atençäo para a estrutura histológica do tumor com característico padräo retiforme, lembrando a imagem histológica da "rete testis"