Pediatric blood & cancer, 2019-11, Vol.66 (11), p.e27935-n/a
2019
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- Autor(en) / Beteiligte
- Titel
- Congenital spindle cell rhabdomyosarcoma
- Ist Teil von
- Pediatric blood & cancer, 2019-11, Vol.66 (11), p.e27935-n/a
- Ort / Verlag
- United States: Wiley Subscription Services, Inc
- Erscheinungsjahr
- 2019
- Quelle
- Wiley-Blackwell Journals
- Beschreibungen/Notizen
- Spindle cell and sclerosing rhabdomyosarcoma (ssRMS) is a rare variant of rhabdomyosarcoma, which includes three distinct subtypes. In infants, these tumors are commonly associated with recurring fusions involving VGLL2 or NCOA2 and have a favorable prognosis. We present four cases of ssRMS and 16 additional cases from the literature, which show that these patients present with localized disease and have an excellent prognosis regardless of surgical margin or lack of radiation therapy. Molecularly defined spindle cell rhabdomyosarcoma in infants is likely a biologically distinct entity which may not require the aggressive multimodal treatment used for other subtypes of rhabdomyosarcoma.
- Sprache
- Englisch
- Identifikatoren
- ISSN: 1545-5009eISSN: 1545-5017DOI: 10.1002/pbc.27935Titel-ID: cdi_proquest_journals_2293986361
- Format
- –
- Schlagworte
- Amputation, Antineoplastic Combined Chemotherapy Protocols - therapeutic use, Chemotherapy, Adjuvant, Combined Modality Therapy, congenital, Cyclophosphamide - administration & dosage, Dactinomycin - administration & dosage, Extremities - pathology, Female, Foot Diseases - congenital, Foot Diseases - drug therapy, Foot Diseases - genetics, Foot Diseases - surgery, Hematology, Humans, Infant, Newborn, Infant, Premature, Infant, Premature, Diseases - drug therapy, Infant, Premature, Diseases - genetics, Infant, Premature, Diseases - surgery, Infants, Male, NCOA2, Nuclear Receptor Coactivator 2, Oncogene Proteins, Fusion - genetics, Oncology, pediatric, Pediatrics, Prognosis, Radiation, Radiation therapy, Remission Induction, Rhabdomyosarcoma, Rhabdomyosarcoma, Embryonal - congenital, Rhabdomyosarcoma, Embryonal - drug therapy, Rhabdomyosarcoma, Embryonal - genetics, Rhabdomyosarcoma, Embryonal - surgery, Soft Tissue Neoplasms - congenital, Soft Tissue Neoplasms - drug therapy, Soft Tissue Neoplasms - genetics, Soft Tissue Neoplasms - surgery, TEA Domain Transcription Factors, Thigh, Thoracic Neoplasms - congenital, Thoracic Neoplasms - drug therapy, Thoracic Neoplasms - genetics, Thoracic Neoplasms - surgery, Thoracic Wall - pathology, Tumors, VGLL2, Vincristine - administration & dosage