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.

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.

Tumor de células de sertoli esclerosante. Reporte de un caso / Sclerosing sertoli cell tumor. Case report

RESUMEN El tumor esclerosante de células de Sertoli del testículo es una entidad patológica infrecuente, con todo, que solo se han descrito 22 casos en la literatura científica. Son descritos como tumores unilaterales que en su mayoría se presentan entre la tercera y sexta década de vida, siendo carentes de malignidad, sin embargo, en ninguno de los casos reportados se han realizado seguimientos por periodos prolongados. Se presenta un caso clínico cuyo objetivo es socializar el primer caso de tumor esclerosante de células de Sertoli (SSCT), diagnosticado por el departamento de patología de la Universidad industrial de Santander, que se registra en Colombia. Después de tres años de seguimiento clínico el paciente no presenta recidiva, así como tampoco otras lesiones tumorales. Se considera la orquiectomia el tratamiento curativo.(AU)

SUMMARY Sclerosing tumor of Sertoli cells of the testis is an infrequent pathological entity, only 22 cases have been described in the scientific literature. They are described as unilateral tumors that mostly occur between the third and sixth decade of life, being devoid of malignancy, however, in none of the cases have been followed for prolonged periods. We present a clinical case whose objective is to socialize the first case of Sertoli cell sclerosing tumor (SSCT), diagnosed by the pathology department of the industrial university of Santander, which is registered in Colombia. After three years of clinical follow-up, the patient does not present recurrence, as well as other tumor lesions. Orchiectomy is considered the curative treatment.(AU)

Sertolioma unilateral como causa de morte súbita por hemorragia interna em ganso Anser cygnoides / Unilateral Sertolioma as a cause of sudden death by internal bleeding in a Anser cygnoides goose

Um ganso adulto macho (Anser cygnoides), da família Anseriformes, de idade desconhecida, proveniente de uma criação da Universidade Luterana do Brasil, foi encontrado morto, sem apresentar histórico clínico, e foi submetido à investigação post mortem no Setor de Patologia Veterinária do Hospital Veterinário. Com base nos achados de necropsia e no exame histopatológico, definiu-se como causa da morte do animal hemorragia interna em razão da ruptura de vasos sanguíneos em uma neoplasia no testículo direito (sertolioma), com metástase no fígado...

An adult male goose (Anser cygnoides) of unknown age, raised at the Lutheran University of Brazil, was found dead without showing clinical history and was submitted for post mortem investigation in the Department of Pathology of the Veterinary Hospital. From the necropsy and histopathological findings, the cause of death was defined as exsanguination due to intestinal hemorrhage from ruptured vessels in a tumor in the right testis, which also presented hepatic metastasis...

Tumor de Sertoli variante célula grande calcificante asociado a síndrome de peutz-jeghers / Large cell calcifying sertoli cell tumor associated with peutz-jeghers syndrome

El tumor de células de Sertoli calcificante de células grandes (TCSCCG) es una neoplasia testicular muy rara, con 60 casos descritos en todo el mundo; en Latinoamérica y Colombia son escasos los casos reportados en la literatura. Esta neoplasia se origina en el estroma gonadal/cordones sexuales, y, en general, tiende a mostrar un comportamiento biológico benigno. Se piensa que estos tumores tienen características de presentación diferente, dependiendo de si se presentan en el contexto de un síndrome congénito complejo, en cuyo caso tienden a ser bilaterales y multifocales, en contraposición a los no asociados a síndromes, que tienden a ser unilaterales y focales. El estudio de inmunohistoquímica es fundamental para el diagnóstico; en especial, para diferenciar estos tumores de neoplasias germinales. Se reportan en esta investigación tres casos de esta neoplasia, uno de ellos asociado al síndrome de Peutz-Jeghers, y haciendo énfasis en los criterios histológicos para definir malignidad.

The large cell calcifying Sertoli cell tumor (LCCSCT) is a very rare testicular tumor with 60 cases reported worldwide; in Latin America and Colombia, few cases are reported in the literature. This neoplasm originates in the gonadal stromal/sex cord, and, in general, tends to display benign biological behavior. It is thought that these tumors may display differing characteristics: depending upon whether they occur in the context of a complex congenital syndrome, in which case they tend to be bilateral and multifocal; as opposed to those not associated with syndromes, which tend to be unilateral and focal. Immunohistochemical study is essential for diagnosis, in particular, to differentiate these tumors from germ cell tumors. This study reports on three cases of this tumor--one associated with Peutz-Jeghers Syndrome—and emphasis is given to histological criteria to define malignancy.