Ovarian Sertoli-Leydig cell tumour with α-fetoprotein-producing intestinal glandular cells. Clinical case and short review of basic literature

Abstract

Sertoli Leydig cell tumor of the ovary, is a rare neoplasm from the group of sex cord-stromal tumors of the ovary, accounting for less than 1% of all ovarian tumors. Among the Sertoli Leydig cell tumors, we distinguish a separate group of tumors secreting α-fetoprotein (AFP). The young 24-year-old woman presented to the Clinical Department of Gynaecological Endocrinology at the University Hospital in Krakow due to secondary amenorrhea, hirsutism and worsening abdominal pain for several months. During the admission draws attention was drawn to the abnormal level of testosterone, AFP and the revised structure of the ovary in the ultrasound. After a preliminary diagnosis, expanded pelvic MRI was performed, which found an isolated tumor derived from Sertoli Leydig cells. The patient was enrolled to unilaterally remove the right ovary by laparotomy. Histopathological examination and immunohistochemical staining confirmed the diagnosis of Sertoli Leydig cells tumor, and in pathological examination we found glandular mucosa cells of the colon. Owing to scientific reports on the stromal tumors of the ovary, we decided to perform genetic testing and verify the patient's karyotype. In the follow-up 90 days after the surgery, levels of testosterone and AFP were correct. In case of Sertoli Leydig cell tumors, especially in young women of childbearing potential, special attention should be paid to Anti-Mullerian hormone testing before surgery, as well as genetic diagnostics to exclude disorders of sex development.

15-Year-Old Patient with an Unusual Alpha-Fetoprotein-Producing Sertoli-Leydig Cell Tumor of Ovary

“…We compare our case to these cases and to new case reports since this publication to understand the origin of AFP-producing cells ( Table 1 ) [ 6 , 8 – 11 ].…”

Tumor de células de Sertoli-Leydig: a propósito de un caso y revisión de la literatura reciente

“…El tumor de células de Sertoli-Leydig, también conocido como androblastoma, pertenece a los tumores del estroma de los cordones sexuales y presenta un patrón de diferenciación testicular [4][5][6] . Afecta a cualquier grupo etario, pero el 75% se diagnostican en mujeres menores de 30 años y solo el 10% en mayores de 50 años.…”

“…El lugar de recaída más frecuente es la cavidad abdominal 1,7,11 . En caso de sospecharse una recidiva, la prueba de imagen de elección es la TC 6 .…”

“…Tampoco existe evidencia sobre la estrategia de seguimiento tras la cirugía. En las guías de la National Comprehensive Cancer Network (NCCN) se recomienda llevar a cabo una exploración física y análisis de marcadores tumorales cada 2-4 meses durante los primeros 2 años desde la intervención, y luego cada 6 meses durante un mínimo de 10 años 6 .…”

“…La incidencia de los tumores de células de Sertoli-Leydig entre las neoplasias de ovario oscila entre menos del 0,5% y el 1%, según la literatura consultada [4][5][6] . Es el tumor virilizante más frecuente, y más de la mitad de las pacientes presentan sintomatología hormonal, como manifestaciones androgénicas, amenorrea secundaria o ciclos irregulares 7 .…”