Sex-Cord Tumor with Annular Tubules with Unusual Morphology in an Infant with Peutz-Jeghers Syndrome. (4th March 2022)

• Record Type: Journal Article
• Authors: Maity, Priyanka; Das, Nandini; Chatterjee, Uttara; Basak, Dhananjay
• Is Part Of: Fetal and pediatric pathology. Volume 41:Number 2(2022)
• Page Start: 306
• Page End: 311
• Publication Date: 2022-03-04
• Subjects: Peutz-Jeghers syndrome -- sex-cord tumor with annular tubules -- sex-cord tumors -- Sertoli cell tumors; Pathology, Molecular -- Periodicals; Pediatrics -- Periodicals; Molecular biology -- Periodicals; Pediatric pathology -- Periodicals; Fetal Diseases -- pathology -- Periodicals; Infant, Newborn, Diseases -- pathology -- Periodicals; Pediatrics -- Periodicals; 618.92007
• DOI: 10.1080/15513815.2020.1786200
• Languages: English
• ISSNs: 1551-3815

Abstract

Abstract: Background Peutz-Jeghers syndrome (PJS) is characterized by hamartomatous gastrointestinal polyposis, mucocutaneous pigmentation and cancer predisposition. The clinical features of PJS manifest in first two decades of life; however, neonatal presentation is uncommon. Case report: We present a five day old girl with PJS that presented with obstructive hamartomatous polyps in the sigmoid colon. At colostomy closure at six months, an incidental ovarian sex-cord tumor with annular tubules (SCTAT) was detected. It showed predominantly a solid pattern with limited tubule formation and was composed of lipid-rich cells. She had no hormonal symptoms. Conclusion: SCTAT can occur as young as six months of age in PJS, and may show histologic overlap with lipid-rich Sertoli cell tumors.