Dysgerminoma with Estrogen-Producing Functioning Stroma Presenting Precocious Puberty. (22nd April 2021)

• Record Type: Journal Article
• Authors: Nagase, Shunsuke; Ogura, Kanako; Ashizawa, Karin; Nakazawa-Tanaka, Nana; Urao, Masahiko; Fukunaga, Masaharu; Yamazaki, Yuto; Sasano, Hironobu; Matsumoto, Toshiharu
• Other Names: Kulka Janina Academic Editor.
• Is Part Of: Case reports in pathology. Volume 2021(2021)
• Publication Date: 2021-04-22
• Subjects: Pathology -- Periodicals; Pathology; Pathology; Electronic journals; Periodicals; Periodicals; Case Reports; Fulltext; Internet Resources; Periodicals; 616.07
• DOI: 10.1155/2021/5545645
• Languages: English
• ISSNs: 2090-6781

Abstract

Abstract : Dysgerminoma is a malignant ovarian germ cell tumor, and unlike sex-cord stromal tumors, endocrine manifestation is considered rare. Here, we report the first case of dysgerminoma presenting precocious puberty. The patient is a 7-year-old girl who presented with a breast development in Tanner stage 3. Serum estradiol (E2 ) was markedly elevated while luteinizing hormone (LH) and follicle-stimulating hormone (FSH) were suppressed below the detection limit. Microscopically, the right ovarian mass displayed nests of large polygonal cells and fibrous septa which were focally concentrated by theca-like plump spindle cells. Immunohistochemistry revealed that the spindle cells expressed various steroidogenic enzymes involved in estrogen biosynthesis including P450 aromatase. The tumor was diagnosed with pure dysgerminoma with estrogen-producing functioning stroma. After the operation, serum E2 declined below the detection limit; LH and FSH returned within the normal range. This case demonstrates that even a conventional dysgerminoma can present endocrine manifestation through functioning stroma.