Dermatofibrosarcoma Protuberans of the Neck: A Case Report

Tzu-I Wu,Chia-Jung Lee,Tzu Hsuan Luo,Yih-Jeng Tsai,Hsing-Mei Wu

doi:10.4236/ijohns.2021.102009

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.

Highlights

Dermatofibrosarcoma protuberans (DFSP) is an uncommon locally aggressive cutaneous soft tissue sarcoma of the dermis layer of the skin [1] with an incidence of 0.8 - 4.5 cases per million per year [2] [3]
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

Summary

Introduction

Dermatofibrosarcoma protuberans (DFSP) is an uncommon locally aggressive cutaneous soft tissue sarcoma of the dermis layer of the skin [1] with an incidence of 0.8 - 4.5 cases per million per year [2] [3]. Due to its slow growing nature similar to nodules that appear as hypertrophic scars or benign soft tissue tumors without any definite symptoms, it is difficult to diagnose pre-operatively. Dermatofibrosarcoma protuberans (DFSP) is rare soft tissue sarcoma but has a locally aggressive nature. Most cases are of low grade and have a rare metastasis rate, all DFSP variants have a tendency to show local recurrence. 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. Due to the low incidence rate, slow-growing nature, and non-alarming initial presentation features, diagnostic delay or even misdiagnosis is not uncommon

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