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

被引：4

作者：

Serife, Kacar ^{[1
]}

Karampelas, Stavros ^{[2
]}

Hottat, Nathalie ^{[3
]}

Devalck, Christine ^{[4
]}

Vanden Houte, Katherina ^{[1
]}

机构：

[1] Univ Libre Bruxelles, Brugmann Univ Hosp Ctr, Dept Pathol, Brussels, Belgium

[2] Univ Libre Bruxelles, Brugmann Univ Hosp Ctr, Dept Gynecol Surg, Brussels, Belgium

[3] Univ Libre Bruxelles, Brugmann Univ Hosp Ctr, Dept Med Imaging, Brussels, Belgium

[4] Univ Libre Bruxelles, Pediat Oncol Reine Fabiola Childrens Univ Hosp, Brussels, Belgium

来源：

CASE REPORTS IN OBSTETRICS AND GYNECOLOGY | 2022年 / 2022卷

关键词：

HEPATOCYTIC DIFFERENTIATION; RETIFORM PATTERN; ANDROBLASTOMA; DIAGNOSIS; ELEMENTS;

D O I：

10.1155/2022/4759826

中图分类号：

R71 [妇产科学];

学科分类号：

100211 ;

摘要：

Ovarian Sertoli-Leydig cell tumors (SLCTs) are extremely rare ovarian sex-cord stromal tumors. Alpha-fetoprotein (AFP) production by SLCTs is a rare event generally linked to the presence of hepatocytes or intestinal mucinous epithelium as heterologous elements. We report here a case of a 15-year-old female complaining about abdominal pain, constipation, and spaniomenorrhea with high level of serum AFP leading to a clinical suspicion of malignant germ cell tumor. Final histopathological diagnosis was a moderately differentiated Sertoli-Leydig cell tumor of the ovary with alpha-fetoprotein-producing cells without hepatocytic or intestinal epithelium differentiation. NGS analysis showed mutation in DICER1 gene. SLCTs occur in patients at any age with a mean age of 25 years. The presence of alpha-fetoprotein-producing cells is an important tool in the differential diagnosis of germ cell tumors and challenging in this case of SLCT because of its rarity in this context. An adequate sampling and exhaustive immunohistochemical analyses are mandatory to make the correct differential diagnosis and confirm the presence of alpha-fetoprotein-producing cells and also define the differentiation because of therapeutic strategies between conservative surgery and/or chemotherapy.

引用

页数：8