title: Feminizing Sertoli cell tumor associated with Peutz-Jeghers syndrome.
abstract: Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by the association of mucocutaneous pigmentation and multiple gastrointestinal hamartomatous polyps and with an increased risk of developing gonadal sex tumors besides other malignancies. We describe a 7 1/2 year-old boy with PJS and bilateral gynecomastia. He has had buccal pigmentation since 1.5 years and had been operated for rectal polyp excision at 3.5 years. On physical examination, his height was at the 90th percentile, and his height age and bone age were 9 and 10 1/2 years, respectively. Increased melanotic buccal pigmentation of the lips and bilateral gynecomastia were noticed. Both of the testes were firm, non-tender and smooth on the surface, and each measured 8 ml. Hormonal measurements were all in the prepubertal range. Testis ultrasonography showed bilateral hyperechogenic areas within the glands. When he was operated for invagination and an ileum segment full of polyps was resected, bilateral testicular biopsies were also performed. Histopathological evaluation of the testes revealed bilateral multicentric benign Sertoli cell tumors. The aromatase inhibitor testolactone was started to slow skeletal maturation. On the basis of this and previous reports, PJS associated with sex-cord tumors is increasingly recognized in males as well as in females.
authors: Division of Pediatric Endocrinology, Hacettepe University, Ihsan Dogramaci Children's Hospital, Ankara, Turkey. ayfer@gen.hun.edu.tr
authors: Ayfer
authors: A
authors: Alikasifoglu
authors: E Nazh
authors: EN
authors: Gonc
authors: Zuhal
authors: Z
authors: Akcoren
authors: Gulsev
authors: G
authors: Kale
authors: Arbay O
authors: AO
authors: Ciftci
authors: M Emin
authors: ME
authors: Senocak
authors: Nursen
authors: N
authors: Yordam
mesh_terms: D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D002648:Child; D006177:Gynecomastia / Q000209:etiology; D006728:Hormones / Q000097:blood; D006801:Humans; D007082:Ileum / Q000473:pathology / Q000601:surgery; D007417:Intestinal Polyps / Q000473:pathology / Q000601:surgery; D008297:Male; D010580:Peutz-Jeghers Syndrome / Q000473:pathology* / Q000601:surgery; D010859:Pigmentation Disorders / Q000209:etiology; D012707:Sertoli Cell Tumor / Q000150:complications* / Q000473:pathology; D013736:Testicular Neoplasms / Q000150:complications* / Q000473:pathology; D013738:Testolactone / Q000627:therapeutic use
chemical_list: D018931:Antineoplastic Agents, Hormonal; D006728:Hormones; D013738:Testolactone