Department of Pediatrics, State University of New York Downstate Health Sciences University, New York, USA.
Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Pediatr Blood Cancer. 2024 Oct;71(10):e31180. doi: 10.1002/pbc.31180. Epub 2024 Jul 28.
Peutz-Jeghers syndrome (PJS) is a childhood-onset cancer predisposition syndrome that is associated with oral freckling and gastrointestinal polyposis. Male patients with PJS are at risk for large-cell calcifying Sertoli cell tumors in childhood. These tumors are estrogen-producing and can cause symptoms of precocious puberty, gynecomastia, and growth acceleration. Here we discuss our experience with spontaneous resolution or stabilization of breast enlargement without medical intervention in three patients with PJS and gynecomastia. These cases indicate that a watchful waiting approach can be considered in the management of gynecomastia in male children with PJS.
皮杰特-杰格斯综合征（PJS）是一种儿童期发病的癌症易感性综合征，与口腔雀斑和胃肠道息肉病有关。患有 PJS 的男性患者在儿童时期有罹患大细胞钙化支持细胞瘤的风险。这些肿瘤会产生雌激素，并可能导致性早熟、男性乳房发育和生长加速等症状。在这里，我们讨论了我们在三名患有 PJS 和男性乳房发育症的患者中，无需医疗干预即可自发消退或稳定乳房增大的经验。这些病例表明，在管理患有 PJS 的男性儿童的男性乳房发育症时，可以考虑采取静观其变的方法。
