Upper extremity pleomorphic dermal sarcoma in a patient with chronic myelomonocytic leukemia.

BACKGROUND: Pleomorphic dermal sarcoma is a potentially high-grade cutaneous spindle cell tumor that closely resembles atypical fibroxanthoma in the superficial, dermal aspects but with adverse pathological features. Chronic inflammation, as several autoimmune disorders are co-associated with chronic myelomonocytic leukemia.

CASE DESCRIPTION: We report here an 84-year-old male patient with swelling lump on the upper third of the left arm. Previously he suffered from a type I chronic myelomonocytic leukemia. Based on the initial ultrasound-guided biopsy of the lesion, the histopathological examination revealed an atypical fibroxanthoma. A wide local excision was performed and the diagnosis was revised to pleomorphic dermal sarcoma by the pathologist, based on the currently accepted criteria. Adjuvant radiotherapy was performed.

CONCLUSION: Differentiating between atypical fibroxanthoma and pleomorphic dermal sarcoma is pivotal. A partial sampling of the skin lesion poses a significant pitfall, as important diagnostic features cannot be assessed. Immunosuppression seems to be involved in the pathogenesis of chronic myelomonocytic leukemia and pleomorphic dermal sarcomas, because of the advanced patient age. HIPPOKRATIA 2019, 23(4): 181-185.