Case of pleomorphic rhabdomyosarcoma arising on subcutaneous tissue in an adult patient: Review of the published works of 13 cases arising on cutaneous or subcutaneous tissue

Abstract

Rhabdomyosarcoma (RMS) is a malignant mesenchymal tumor usually occurring in children, young adults, and predominantly arising in the head and neck or deep soft tissue of the extremities. Pleomorphic RMS (PRMS) arising on subcutaneous tissue in an adult patient's trunk is extremely rare. We present a case of primary PRMS occurring on the back of a 50-year-old man. The tumor was diagnosed by histological and immunohistochemical findings of the resected specimen. One month after the surgery, axillary lymph node metastases were found. Subsequently, the patient received three cycles of combined chemotherapy. The rest of those axillary lymph nodes were reduced in size and there was no local recurrence 6 months postoperatively. PRMS is a very rare but aggressive tumor. Thus, it is important to confirm the diagnosis and provide adequate treatments.

Primary cutaneous pleomorphic rhabdomyosarcoma in the periocular region

“…1 This case represents the first description of a periocular cutaneous PRMS in an adult in the English literature, and therefore adds to the base of information regarding the primary cutaneous form of this disease. Eleven of the 13 described cases underwent surgical excision of the tumour, with six patients receiving adjuvant chemotherapy and five patients adjuvant radiotherapy.…”

“…[1][2][3] PRMS arising in the dermis and subcutis is extremely rare, with few cases having been reported in the literature. Primary cutaneous PRMS is a rare variant of adult sarcoma, arising most commonly in the head and neck, or the soft tissues of the extremities.…”

“…1 PRMS is most commonly found within the paediatric population, and is readily described within the orbit. Rhabdomyosarcoma can broadly be split into four main histological sub types: pleomorphic, embryonal, alveolar and spindle cell/sclerosing.…”

“…Eleven of the 13 described cases underwent surgical excision of the tumour, with six patients receiving adjuvant chemotherapy and five patients adjuvant radiotherapy. 1,2 The pathogenesis of the inner retinal infarction is unclear. 1 This case represents the first description of a periocular cutaneous PRMS in an adult in the English literature, and therefore adds to the base of information regarding the primary cutaneous form of this disease.…”

“…Huw Oliphant BSc MBBCh,1 Mark Taylor FRCPath 2 and Saul Rajak PhD FRCOphth 1The Sussex Eye Hospital, and 2 Department of Pathology, Brighton and Sussex University Hospitals, Brighton, UK…”