Embryonal and Alveolar Rhabdomyosarcoma of Parameningeal Sites in Adults: A Report of 13 Cases

Abstract

This study reports 13 adult parameningeal rhabdomyosarcomas. Ages ranged from 18 to 86 years. Nine lesions were alveolar, 3 were embryonal, and 1 could not be further classified. All cases were positive for desmin and/or myogenin and 1 tumor was strongly positive for cytokeratin. PAX3-FKHR or PAX7-FKHR fusion transcripts or FKHR breaks were identified in 5 cases confirming a diagnosis of alveolar rhabdomyosarcoma. Three cases were negative supporting a diagnosis of embryonal rhabdomyosarcoma. A total of 10 patients were treated with chemotherapy and radiation. Three patients are alive with no disease, 3 are alive with disease, 3 died of disease, and 4 patients are lost to follow-up. Adult sinonasal rhabdomyosarcoma is uncommon and should be considered in the differential of sinonasal neoplasms. Disease can occur in the elderly. Desmin and myogenin can aid in the diagnosis but cytokeratin reactivity can be seen and care must be taken not to diagnose carcinoma.

Adult Sinonasal Rhabdomyosarcoma With Spinal Metastasis: A Case Report and Review of the Literature

“…Sinonasal RMS has an incidence of 0.034 per 100,000 cases, predominantly reported in the pediatric population [ 8 ]. In adults, sinonasal RMS is rare and more commonly seen in patients younger than 35 years old, whereas our patient was a 51-year-old at the time of the diagnosis [ 9 , 10 ]. RMS in adults differs from pediatrics in presentation, histopathology, prognosis, and response to treatment.…”

Rabdomiosarcoma alveolar con expresión aberrante de biomarcadores epiteliales y neuroendocrinos

“…El rabdomiosarcoma alveolar (RMSa) se presenta en un 30% de todos los rabdomiosarcomas 3 . Se puede presentar en cualquier parte del cuerpo 4 , sin embargo la localización anatómica más frecuente son las extremidades, antebrazos y brazos, regiones perirrectal y perineal 5,6 . La mayoría de los RMSa son de aparición esporádica, de alto grado y pueden presentarse en un estadio diseminado avanzado afectando los pulmones, ganglios linfáticos y médula ósea 2 .…”

Sinonasal Rhabdomyosarcoma in a Child: Case Report and Review of the Literature

“…The most common in children, adolescent and adults young. Its preferred locations are the head and neck in 44% of cases [1].…”