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Head and neck dermatofibrosarcoma protuberans (HNDFSP) are extremely rare and not entirely understood. This study aimed to investigate the clinicopathological features of HNDFSP (hyalinizing clear cell carcinoma, not otherwise specified) and identify clinically relevant indicators for improved treatment strategies. The study included 49 patients who received treatment at Shanghai Ninth People's Hospital between 2000 and 2021. The study found that HNDFSP is rare, with a worse prognosis and significant challenges to controlling locally. The study identified age, tumor size, and negative margins as independent factors affecting disease-free survival. The study also found that radiotherapy could improve the prognosis of patients with HNDFSP. The study identified several potential targeted therapeutic indicators, including EZH2, EGFR, PDGF, PD-L1, and VEGF, which were positively expressed in most tumor samples. Wide local excision and long-term follow-up are necessary for patients with HNDFSP.
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