Combined Sclerosing and Spindle Cell Rhabdomyosarcoma in Previous Craniotomy Site: A Case Report and a Review of the Literature.
Int J Surg Pathol
; 27(3): 328-335, 2019 May.
Article
en En
| MEDLINE
| ID: mdl-30270691
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.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Asunto principal:
Rabdomiosarcoma
/
Neoplasias de los Músculos
/
Craneotomía
/
Tejido Subcutáneo
/
Neoplasias de Cabeza y Cuello
Tipo de estudio:
Diagnostic_studies
/
Etiology_studies
/
Prognostic_studies
Límite:
Humans
/
Male
/
Middle aged
Idioma:
En
Revista:
Int J Surg Pathol
Asunto de la revista:
PATOLOGIA
Año:
2019
Tipo del documento:
Article
País de afiliación:
Estados Unidos