Head‐and‐neck dermatofibrosarcoma protuberans: Survival analysis and Clinically relevant immunohistochemical indicators

Abstract

BackgroundHead and neck dermatofibrosarcoma protuberans (HNDFSP) is extremely rare and not entirely understood.ObjectiveTo investigate the clinicopathological features of HNDFSP and identify the expression of its clinically relevant indicators, with the expectation of improving the existing treatment strategies.MethodsA long‐term follow‐up of patients with HNDFSP who received treatment between 2000 and 2021 at Shanghai Ninth People's Hospital was conducted. The clinical, histological, and immunohistochemical data of the patients were retrieved and analyzed. The endpoint of the study was the incidence of significant disease‐related clinical events (recurrences or metastasis).ResultsA total of 49 patients with HNDFSP were included in the study, with males (92.7%) predominating than females (7.3%). Eighteen patients developed recurrent disease (36.8%) after surgery, and the median time of recurrence was 48 months (interquartile, 20–74 months). Metastasis occurred in two cases (4.1%). Two patients died during follow‐up, both with local recurrence, and one of them with intestinal metastasis. Post‐operation radiotherapy was administered to eight patients (16.3%) and the effect in local control was remarkable. Age, tumor size, and negative margins with sufficient safety width were the main independent factors affecting the disease‐free survival. Several potential targeted therapeutic indicators, including EZH2 (80.0%), EGFR (91.4%), PDGF (97.1%), PD‐L1 (77.1%), and VEGF (77.1%), were positively expressed in most tumor samples.ConclusionHNDFSP is rare, significantly challenging to control locally, and has a worse prognosis with current treatment strategies. Wide local excision and long‐term follow‐up are needed. Radiotherapy could improve the prognosis of patients with HNDFSP.

The Role of Postoperative Radiotherapy in the Management of Dermatofibrosarcoma Protuberans: A Multidisciplinary Systematic Review

“…Dai et al [ 1 ] conducted a retrospective study of forty-nine patients with head and neck dermatofibrosarcoma protuberans. They collected patients affected by HNDFSP who had received surgical treatment.…”

“…Dermatofibrosarcoma protuberans (DFSP) represents a superficial soft tissue sarcoma that involves the dermis, subcutaneous fat and, in rare cases, muscle and fascia [ 1 ].…”

Fibrosarcomatous Dermatofibrosarcoma Protuberans of the Head

“…Head DFSP, which account for less than 10% of all DFSP cases, can be challenging to recognize and manage. In the series by Dai, et al (2023) 1 , the similarity of the initial presentation of DFSP with other lesions of the head such as solitary brous tumors, peripheral nerve sheath tumors, or benign brosarcomas predisposes to risk of delays or errors in diagnosis, hence the emphasis on accurate histomorphological description of the biopsy and adequate immunohistochemistry.…”

“…Lung and bone metastases dictate poorer outcomes. Regular, periodic follow-up of patients to clinically assess post-operative sites, with active search for possible local invasion and/or distant metastasis are crucial 1,2 .…”

“…Dermato brosarcoma protuberans (DFSP) is a rare type of sarcoma that arises from the broblasts, and tends to become locally aggressive 1 . The tumor begins as a gradually enlarging palpable lesion over the skin, mostly affecting the trunk and extremities.…”