An Integrative Morphologic and Molecular Approach for Diagnosis and Subclassification of Rhabdomyosarcoma.
Arch Pathol Lab Med
; 146(8): 953-959, 2022 08 01.
Article
en En
| MEDLINE
| ID: mdl-35051261
ABSTRACT
CONTEXT. Rhabdomyosarcoma, the most common soft tissue sarcoma of children, is currently classified into the following 4 subtypes embryonal rhabdomyosarcoma, alveolar rhabdomyosarcoma, spindle cell/sclerosing rhabdomyosarcoma, and pleomorphic rhabdomyosarcoma, based on recent molecular genetic knowledge and morphologic features. OBJECTIVE. To highlight the most recent advances of molecular genetic alterations, and to familiarize pathologists with most recent genotype and phenotype correlation in rhabdomyosarcoma. DATA SOURCES. Data were derived from the World Health Organization Classification of Soft Tissue and Bone Tumors, fifth edition, recently published literature (PubMed), and clinical practice experience. CONCLUSIONS. Current classification has been significantly impacted by genotype and phenotype correlation, especially with PAX-FOXO1 fusion-positive rhabdomyosarcoma versus fusion-negative rhabdomyosarcoma, and with the emergence of 3 distinct new subtypes of spindle cell/sclerosing rhabdomyosarcoma. Although all rhabdomyosarcomas were considered a single diagnostic entity in the past, they are now considered to be a group of histologically similar but biologically diverse entities because their clinical behavior and underlying molecular alterations dramatically differ. This review outlines recent molecular genetic developments, corresponding morphologic features, and current challenges faced by pathologists in daily practice.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Rabdomiosarcoma
/
Neoplasias de los Tejidos Blandos
/
Rabdomiosarcoma Embrionario
Tipo de estudio:
Diagnostic_studies
Límite:
Humans
Idioma:
En
Revista:
Arch Pathol Lab Med
Año:
2022
Tipo del documento:
Article