Dermatofibrosarcoma protuberans of the head and face showing predilection for the cheek region

Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive cutaneous sarcoma that most commonly affects the trunk and proximal extremities, but only infrequently involves the head and face. Despite its low incidence in this region, DFSP of the head and face presents disproportionate clinical challenges due to the complex anatomy, high misdiagnosis rate, and potential for significant functional and aesthetic impairment following surgical excision. Until now, the anatomic distribution of DFSP within the head and face has not been systematically characterized.

In a retrospective single-center study published in the Chinese Journal of Plastic and Reconstructive Surgery, 161 patients with histologically confirmed DFSP were analyzed, among whom 26 cases (16.1%) involved the head and face.

“Tumor locations were mapped according to well-defined facial fat compartments, including the cheek, scalp, forehead and temporal region, nasolabial and jowl, and otic areas,” shares lead author Zhu Zhu. “The cheek emerged as the dominant site, accounting for 65.38% of all head-and-face DFSPs, whereas no tumors were found in the nasal or orbital compartments.”

This striking predilection suggests that regional anatomic or biological factors influence tumor development.

Clinically, patients with cheek DFSP were significantly older than those with tumors in non-cheek locations (48.82 vs 37.22 years, p = 0.04), while sex, tumor size, depth, recurrence rate, and trauma history showed no statistically significant differences between groups.

“Histologically, most tumors were conventional DFSP (88.5%), but three cases were fibrosarcomatous DFSP (FS-DFSP), a more aggressive subtype associated with higher recurrence and metastatic risk. Notably, all FS-DFSP cases occurred in non-cheek regions, whereas none were observed in the cheek (P = 0.032),” Zhu says.

These findings point toward a potential biological role of facial fat compartments in DFSP pathogenesis.

“The cheek is one of the most adipose-rich regions of the face, and DFSP characteristically infiltrates subcutaneous fat in a honeycomb pattern,” Zhu explains further. “Experimental and clinical evidence suggests that adipose-derived cells, inflammatory mediators, growth factors, and metabolic substrates within fat tissue may create a permissive microenvironment that supports DFSP growth while restraining aggressive fibrosarcomatous transformation.”

In contrast, non-cheek regions, which contain less adipose tissue, may impose metabolic stress that favors tumor dedifferentiation toward FS-DFSP.

Overall, this largest-to-date cohort of head-and-face DFSP demonstrates a clear predilection for the cheek, particularly in older patients, with a lower frequency of fibrosarcomatous transformation. Recognizing this spatial and biological heterogeneity can improve early diagnosis, guide surgical planning, and support the development of fat-microenvironment-based therapeutic strategies for DFSP of the face.

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Contact the author: Zhaoqi Yuan, Department of Plastic and Reconstructive Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine. Shanghai 200011, China. 18818270132@sina.cn.

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