Ovarian Sertoli-Leydig Cell Tumors: Epidemiological, Clinical and Prognostic Factors

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%) presented with 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 of Gynecology and Obstetrics (FIGO). A total of 2 (16.7%) patients received adjuvant treatment; 1 of them underwent 3 cycles of paclitaxel and carboplatin every 21 days, 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.

Ovarian Sertoli–Leydig cell tumours: A systematic review of relapsed cases

“…Despite the good prognosis of SLCT, it has been reported that the relapse rate ranges from 0% to 33.3% [2,4,[7][8][9]. In contrast to other SCST such as ovarian granulosa cell tumours, SLCT tend to relapse early, with a relapse rate of 95% within 5 years [6,10].…”

Un tumor de ovario inusual: tumor de Sertoli-Leydig

“…A causa de lo infrecuente de esta enfermedad, las estrategias de tratamiento están basadas en comunicaciones de casos y experiencias institucionales. La resección quirúrgica del tumor es la primer maniobra terapéutica; en tumores unilaterales y localizados la ooforectomía unilateral es de elección, en casos con mayor compromiso (compromiso bilateral, tumores de gran tamaño, compromiso de órganos vecinos o lesiones secundarias intra abdominales) el objetivo es la resección de toda la enfermedad macroscópica, intentando no dejar residuo y realizando en el mismo acto una exhaustiva estadificación, evaluando la presencia de enfermedad extraovárica; algunos informes recomiendan la evaluación del endometrio para descartar una neoplasia endometrial sincrónica 5 . La presentación de esta enfermedad en pacientes jóvenes, en edad reproductiva, fuerza a buscar una óptima resección, intentando preservar la función hormonal y reproductiva cuando es posible.…”

“…Se manifiestan habitualmente con signos y/o síntomas inespecíficos secundarios al crecimiento de una masa abdominal, pero una de sus características distintivas es la posibilidad de sintetizar hormonas con manifestación de síntomas y/o signos secundarios a hipersecreción androgénica (patrón del vello masculino, voz grave, hipertrofia del clítoris) o estrogénica (sangrados ginecológicos anormales, amenorrea) 4 . Es relevante el informe y discusión de estos casos por ser tumores infrecuentes, usualmente con una histopatología heterogénea (lo que dificulta su diagnóstico) y su presentación en pacientes jóvenes en edad reproductiva, lo que lleva a buscar una estrategia de tratamiento óptima para tratar la enfermedad, intentando conservar la función reproductiva y evitando comorbilidades 5 . Presentamos la experiencia con tres situaciones clínicas diferentes en pacientes con TSL con posterior discusión de la evidencia publicada.…”

Spectrum of DICER1 Germline Pathogenic Variants in Ovarian Sertoli–Leydig Cell Tumor

“…At premenopausal age, women experience oligomenorrhea or amenorrhea, while virilization can appear with hirsutism, acne, frontal hair thinning, male pattern baldness, deepening of the voice, increased musculature, increased libido, and clitoromegaly [ 4 , 5 , 6 , 7 ]. SLCTs can also present with abdominal pain and increased abdominal circumference, frequently with a palpable adrenal mass at physical examination [ 8 ]. From a prognostic evaluation of patients, an important feature is the SLCTs degree of differentiation.…”