Dermatofibrosarcoma protuberans mimicking a benign lesion: case report on a rare diagnostic dilemma

Girish Mishra; Manali B. Gaudani

doi:10.18203/issn.2454-5929.ijohns20253813

Authors

Girish Mishra Department of ENT and Head and Neck Surgery, Pramukhswami Medical College, Bhaikaka University, Karamsad, Anand, Gujarat, India
Manali B. Gaudani Department of ENT and Head and Neck Surgery, Pramukhswami Medical College, Bhaikaka University, Karamsad, Anand, Gujarat, India

DOI:

https://doi.org/10.18203/issn.2454-5929.ijohns20253813

Keywords:

Dermatofibrosarcoma protuberans, Fine-needle aspiration cytology, Mohs micrographic surgery

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare, low- to intermediate-grade dermal sarcoma, representing less than 1% of head and neck malignancies. It is locally aggressive, with high recurrence rates but low metastatic potential. Parotid region involvement is exceptionally uncommon. A 35-year-old male presented with a recurrent swelling in the left pre-auricular region, one month after superficial parotidectomy. Histopathology of the earlier specimen had confirmed DFSP. Positron emission tomography (PET) revealed fluorodeoxyglucose (FDG)-avid nodular enhancement, and wide local excision (WLE) with reconstruction was performed. Margins were clear, and adjuvant radiotherapy was administered. DFSP in the parotid region is extremely rare and may mimic benign parotid tumors, leading to delayed or incorrect diagnosis. Complete surgical excision with negative margins remains the cornerstone of management, while radiotherapy can reduce recurrence risk in high-risk cases. Given its infiltrative nature and propensity for late recurrence, meticulous long-term follow-up is mandatory to ensure optimal outcomes.

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