Dermatofibrosarcoma Protuberans of the Neck: A Case Report

Abstract

Background: Dermatofibrosarcoma protuberans (DFSP) is rare soft tissue sarcoma but has a locally aggressive nature. Although most cases are of low grade and have a rare metastasis rate, all DFSP variants have a tendency to show local recurrence. Wide excision with negative margins is the treatment of choice. Aim: To report a case of DFSP who presented with an asymptomatic slow growing tumor similar to etiologies such as hypertrophic scars or other benign soft tissue tumors. Case Presentation: A 68-year-old male presented with a large soft tumor located at the left posterior neck. Local excision was done under the preoperative impression of a benign tumor such as lipoma or sebaceous cyst. However the diagnosis of DFSP was made upon histological examination and the patient underwent another surgery to achieve free resection margins under general anesthesia as well as adjuvant radiotherapy. Conclusion: DFSP is a malignant tumor that is diagnosed histopathologically. Due to the low incidence rate, slow-growing nature, and non-alarming initial presentation features, diagnostic delay or even misdiagnosis is not uncommon.

Dermatofibrosarcoma Protuberans is Possible Without Skin Involvement

“…Local recurrences often occur requiring more than one surgical procedure, as the tumor is spread intra-and/or subcutaneously and is often very difficult to distinguish clinically. [3][4][5] In histopathology, the tumor is located in the dermis and consists of uniform fusiform cells, densely grouped, with an elongated nucleus. The mass should be excised with a wide surgical margin to maintain an adequate safety distance.…”

“…Developing a multidisciplinary approach is an expertise that could achieve for the treatment of DFSP lesions. [1][2][3][4][5][6][7]…”