Recurrent pleomorphic dermal sarcoma of the temporal bone: Case report and literature review

Abstract

Background: Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) lie on a diagnostic spectrum and have been previously classified as benign (BFH) or malignant fibrous histiocytoma (MFH) and undifferentiated pleomorphic sarcoma (UPS). We report a Case of recurrent PDS of the temporal bone and systematically review literature regarding this tumor spectrum presenting in the auricle and temporal bone. Case presentation: A 71-year-old male presented with AFX of the left tragus five years after undergoing radiotherapy for recurrent squamous cell carcinoma of the left auricle. He underwent total auriculectomy, lateral temporal bone resection, and right anterolateral thigh myocutaneous free flap reconstruction (ALTFF). The tumor recurred in the infratemporal fossa requiring re-excision and left ALTFF. Adjuvant radiotherapy was deferred by the patient. There are 104 reported cases presenting in the auricle, periauricular skin, or temporal bone (93 AFX, 7 MFH, 2 BFH, 1 UPS, 1 PDS). All were treated with surgery; 3 cases of MFH were treated with adjuvant radiotherapy or chemotherapy. Conclusion: PDS remains a clinical challenge due to its wide diagnostic spectrum and unpredictable nature. Multimodal treatment should be considered in aggressive or recurrent cases.

Keywords

• Atypical fibroxanthoma
• Pleomorphic dermal sarcoma
• Cutaneous pathology
• Head and neck reconstruction