1. Combined Sclerosing and Spindle Cell Rhabdomyosarcoma in Previous Craniotomy Site: A Case Report and a Review of the Literature.
- Author
-
Gui, Hongxing, Lhospital, Elliott, Staddon, Arthur P., Nagda, Suneel N., Zager, Eric L., Zhang, Paul J. L., and Brooks, John S.
- Subjects
RHABDOMYOSARCOMA ,CRANIOTOMY ,CELL morphology ,CASE studies ,DERMATOFIBROMA - Abstract
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. [ABSTRACT FROM AUTHOR]
- Published
- 2019
- Full Text
- View/download PDF