Gui Hongxing, Lhospital Elliott, Staddon Arthur P, Nagda Suneel N, Zager Eric L, Zhang Paul J L, Brooks John S
1 Department of Pathology and Laboratory Medicine, Pennsylvania Hospital of the University of Pennsylvania Health System, Philadelphia, PA, USA.
2 Division of Hematology/Oncology, Department of Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
Int J Surg Pathol. 2019 May;27(3):328-335. doi: 10.1177/1066896918802030. Epub 2018 Oct 1.
Sclerosing rhabdomyosarcoma (RMS) is a rare subtype of RMS with unique prominent stromal hyalinization and a pseudovascular architecture. It overlaps morphologically with spindle cell RMS and poses both diagnostic and therapeutic challenges because of its rarity and aggressive clinical course. In this article, we report a case of sclerosing RMS arising from a prior craniotomy site, which demonstrated both sclerosing and spindle cell components. A literature review of RMS with sclerosing morphology identified 122 cases. Our review documents the following: sclerosing RMS occurs in both childhood and adult populations, has a predilection for the head and neck areas, and has a worse prognosis in adults. Sclerosing RMS harbors a high frequency of MYOD1 mutations, conferring a poor clinical outcome. Sclerosing RMS and spindle RMS likely represent a morphologic spectrum of one entity.
硬化性横纹肌肉瘤（RMS）是RMS的一种罕见亚型，具有独特的显著间质玻璃样变和假血管结构。它在形态学上与梭形细胞RMS重叠，由于其罕见性和侵袭性临床过程，带来了诊断和治疗方面的挑战。在本文中，我们报告了一例起源于既往开颅手术部位的硬化性RMS病例，该病例同时表现出硬化性和梭形细胞成分。对具有硬化形态的RMS进行文献回顾，共识别出122例病例。我们的综述记录如下：硬化性RMS发生于儿童和成人人群，好发于头颈部区域，成人患者预后较差。硬化性RMS具有高频的MYOD1突变，导致临床结局不佳。硬化性RMS和梭形RMS可能代表同一实体的形态学谱系。
