女性化肾上腺皮质癌导致性早熟：病例报告及文献复习。

Feminizing adrenocortical oncocytoma presenting as precocious puberty: a case report and literature review.

机构信息

Department of Endocrinology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

Department of Pediatric Surgery, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

出版信息

J Pediatr Endocrinol Metab. 2022 Mar 3;35(6):827-830. doi: 10.1515/jpem-2021-0707. Print 2022 Jun 27.

DOI:10.1515/jpem-2021-0707

PMID:35245009

Abstract

OBJECTIVES

Feminizing adrenal tumors are rare in childhood. We present a case of a special category of adrenal tumor, an oncocytoma, causing isosexual peripheral precocity.

CASE PRESENTATION

A 4-year old girl presented with breast development and menstrual bleeding over a period of 3-4 months. Her SMR staging was breast stage 4, pubic hair stage 3. Her bone age was advanced (6 year 10 months), stimulated LH 0.7 IU/L, estradiol 206 pmol/L and DHEAS >27.1 micromol/L. CT scan revealed a right adrenal mass with features of atypical adrenal adenoma. Laparoscopic adrenalectomy was done and histopathology revealed oncocytoma. Lin-Weiss-Bisceglia criteria classified it as likely benign, borne out till a 2 year follow up.

CONCLUSIONS

Adrenal oncocytoma can be a cause of isosexual peripheral precocity in a young girl. Recognition and correct classification of this histological variant, which is more often benign, is important for prognostication and choice of therapy after surgery.

摘要

目的

儿童时期的女性化肾上腺肿瘤较为罕见。我们报告了一例特殊类型的肾上腺肿瘤——嗜酸细胞瘤，其导致同性性早熟。

病例介绍

一名 4 岁女孩出现乳房发育和月经出血，持续 3-4 个月。她的 SMR 分期为乳房 4 期，阴毛 3 期。骨龄提前（6 岁 10 个月），LH 刺激值为 0.7IU/L，雌二醇 206pmol/L，DHEAS ＞27.1 微摩尔/L。CT 扫描显示右侧肾上腺有肿块，具有非典型肾上腺腺瘤的特征。行腹腔镜肾上腺切除术，组织病理学显示为嗜酸细胞瘤。Lin-Weiss-Bisceglia 标准将其归类为可能良性，随访 2 年后仍为良性。