AI Article Synopsis
- Sclerosing rhabdomyosarcoma (RMS) is a rare and aggressive type of cancer characterized by unique features like stromal hyalinization and a pseudovascular structure, making it challenging to diagnose and treat.
- A case study described a sclerosing RMS that developed from a previous craniotomy site, showing both sclerosing and spindle cell characteristics.
- A review of 122 reported cases revealed that sclerosing RMS affects both children and adults, is commonly found in the head and neck regions, has a worse prognosis in adults, and frequently involves MYOD1 mutations that lead to poor outcomes.
Article 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.
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| http://dx.doi.org/10.1177/1066896918802030 | DOI Listing |
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