Pleomorphic Rhabdomyosarcoma with an Impressive Response to Chemotherapy: Case Report and Review of the Literature

Abstract

Our case report emphasizes that multiagent systemic therapy according to pediatric protocols should be considered in adult patients with pleomorphic RMS.

Temozolomide combined with irinotecan caused regression in an adult pleomorphic rhabdomyosarcoma patient-derived orthotopic xenograft (PDOX) nude-mouse model

“…The combination of vincristine, actinomycin D, cyclophosphamide (VAC) is sometimes used to treat RMS and will be used for future experiments comparing VAC with TEM+IRN [ 21 ]. The results of the present study suggests that potential powerful therapy can be identified for many adult PRMS patients.…”

Pleomorphic rhabdomyosarcoma metastasis to small intestine causing intussusception

“…Rhabdomyosarcoma (RMS) is a malignant mesenchymal tumor that originates from skeletal muscle cells. It mostly occurs in children and adolescents, [1] accounting for about 5% of all pediatric cancers and 50% of all soft tissue sarcomas. [2] Among the 3 major histotypes of RMS: embryonal, alveolar, and pleomorphic subtypes, embryonal RMS is mostly diagnosed in children under 10 years old, [2] alveolar RMS predominantly in adolescents and young adults [2] while pleomorphic RMS (PRMS) almost exclusively affects adults aged over 45.…”

“…[2] Among the 3 major histotypes of RMS: embryonal, alveolar, and pleomorphic subtypes, embryonal RMS is mostly diagnosed in children under 10 years old, [2] alveolar RMS predominantly in adolescents and young adults [2] while pleomorphic RMS (PRMS) almost exclusively affects adults aged over 45. [1] The histotypical variants of RMS are associated with differential prognosis with embryonal RMS having the most favorable outcome. [2] In addition, older age predicts disease metastasis and poor survival, [2] likely due to the increased incidence of unfavorable histotype, poor chemosensitivity, and decreased tolerance of patients towards chemotherapies.…”

“…[2] In addition, older age predicts disease metastasis and poor survival, [2] likely due to the increased incidence of unfavorable histotype, poor chemosensitivity, and decreased tolerance of patients towards chemotherapies. [1]…”

“…Pleomorphic RMS represents the most aggressive subtype of RMS, with a 5-year overall survival of 27% to 44%, [1] however, the diagnostic criteria for it has been controversial, [1,3] leading to frequent misdiagnosis of this lethal malignancy. Metastasis is often found at diagnosis, commonly to lungs, lymph nodes, and bone marrow.…”