1. Combined Sclerosing and Spindle Cell Rhabdomyosarcoma in Previous Craniotomy Site: A Case Report and a Review of the Literature.
- Author
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Gui H, Lhospital E, Staddon AP, Nagda SN, Zager EL, Zhang PJL, and Brooks JS
- Subjects
- Aneurysm, Ruptured etiology, Aneurysm, Ruptured surgery, Biopsy, Brain diagnostic imaging, Brain pathology, Chemoradiotherapy, Adjuvant, Diagnosis, Differential, Head and Neck Neoplasms diagnostic imaging, Head and Neck Neoplasms etiology, Head and Neck Neoplasms therapy, Humans, Intracranial Aneurysm complications, Male, Middle Aged, Muscle Neoplasms diagnostic imaging, Muscle Neoplasms etiology, Muscle Neoplasms therapy, Rhabdomyosarcoma diagnostic imaging, Rhabdomyosarcoma etiology, Rhabdomyosarcoma therapy, Scalp, Sclerosis, Subcutaneous Tissue diagnostic imaging, Tomography, X-Ray Computed, Craniotomy adverse effects, Head and Neck Neoplasms pathology, Muscle Neoplasms pathology, Rhabdomyosarcoma pathology, Subcutaneous Tissue pathology
- 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.
- Published
- 2019
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