Department of Pathology, Children's Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China.
Central Laboratory, Children's Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China.
Pediatr Dev Pathol. 2020 May-Jun;23(3):230-234. doi: 10.1177/1093526619879601. Epub 2019 Oct 8.
Primary glomus tumors of the kidney are rare and have never been reported in children under 16 years of age. Tuberous sclerosis complex (TSC) is an extremely variable genetic condition that can affect virtually any organ in the body. Only a single case of glomus tumor associated with TSC was reported in 1964. In this article, we describe the clinical, radiologic, and pathological features of a primary renal glomus tumor in an 8-year-old girl with TSC. This tumor is large, has a deep location, and has infiltrative margins and numerous mitoses. However, there was no disease progression in a 16-month period of follow-up. To our knowledge, this is the second report of primary renal glomus tumor in childhood, the youngest one in the literature.
肾脏原发性血管平滑肌瘤非常罕见，在 16 岁以下的儿童中从未有过报道。结节性硬化症（TSC）是一种极其多样化的遗传疾病，几乎可以影响身体的任何器官。1964 年曾报道过一例与 TSC 相关的血管平滑肌瘤。本文描述了一例 8 岁 TSC 女孩的原发性肾血管平滑肌瘤的临床、影像学和病理学特征。该肿瘤体积较大，位置较深，呈浸润性边缘，有大量有丝分裂。然而，在 16 个月的随访中没有疾病进展。据我们所知，这是儿童期第二例原发性肾脏血管平滑肌瘤的报告，也是文献中报道的年龄最小的病例。
