Squillaro Anthony I, Zhou Shengmei, Thomas Stefanie M, Kim Eugene S
1 Division of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, California.
2 Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Los Angeles, California.
Pediatr Dev Pathol. 2019 Jul-Aug;22(4):375-379. doi: 10.1177/1093526618819605. Epub 2018 Dec 21.
Precocious puberty in an infant is an alarming and infrequent finding, making the differential diagnosis difficult for practitioners. Precocious puberty secondary to a sclerosing stromal tumor (SST) of the ovary is rare. We present a case of a child that began precocious puberty at 3 months of age including development of breast buds, pubic hair, growth spurt, and menarche 5 days prior to presenting to pediatric endocrinology at 10 months. She underwent right salpingo-oophorectomy which demonstrated a soft tissue mass occupying almost the entire ovary with a tan-pink fleshy cut surface. Histological examination confirmed a variant of SST. This case represents an extremely young onset of precocious puberty secondary to a variant of SST without hormonal elevation.
婴儿性早熟是一个令人担忧且罕见的发现，这使得从业者很难进行鉴别诊断。继发于卵巢硬化性间质瘤（SST）的性早熟很罕见。我们报告一例患儿，该患儿3个月大时开始出现性早熟，包括乳腺芽发育、阴毛生长、生长加速，在10个月大就诊于儿科内分泌科前5天出现月经初潮。她接受了右侧输卵管卵巢切除术，术中发现一个软组织肿块几乎占据了整个卵巢，切面呈棕褐色至粉红色肉质。组织学检查证实为SST的一种变体。该病例代表了继发于SST变体且无激素升高的极其年幼的性早熟发病情况。
