Department of Surgery, General Hospital, Port-of-Spain, Trinidad, West Indies.
Published: November 2020
AI Article Synopsis
Article Abstract
A case of dermatofibrosarcoma protuberans (DFSP) in a 22-year old female associated with blunt trauma of the mid-upper back is presented in the current study with a review of the literature. DFSP is a rare slow growing sarcoma of the soft tissue most commonly seen on the trunk and upper extremities with a low to intermediate grade malignant potential, high chance of local invasion and a high local recurrence rate. The literature search revealed that both non-congenital mutation as well as trauma serve a role in the development of this dermal neoplasm, but the exact mechanism by which trauma may predispose to development of DFSP is unknown. However, it seems intuitive that chronic inflammation and stimulation of the immune system at a local level may trigger the immunopathologic changes that could lead to the malignant transformation of dermal cells. Future research into the relationship between trauma and DFSP on a cellular level is required, as there appears to be a direct link present.
Triple primary malignancies are rare, involving the occurrence of three distinct, unrelated cancers in a single patient, requiring a personalized, multidisciplinary treatment approach. A 69-year-old male was diagnosed with dermatofibrosarcoma protuberans (DFSP), lung adenocarcinoma, and papillary thyroid carcinoma. After amputation for DFSP, following staging and genetic evaluation, a multidisciplinary tumor board designed a tailored treatment plan, adjuvant chemotherapy for lung adenocarcinoma while monitor regularly the thyroid carcinoma via ultrasound.
Background: Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive, soft-tissue sarcoma. The treatment is surgical and includes wide local excision (WLE) or Mohs micrographic Surgery (MMS). There is no consensus regarding the preferred type of surgery.
Purpose: To identify candidate susceptibility genes for dermatofibrosarcoma protuberans (DFSP).
Methods: All individuals with DFSP from the International Sarcoma Kindred Study ( = 3767 individuals with sarcoma diagnoses from Australia, Europe, New Zealand, and United States) and cohorts that were not ascertained based on sarcoma status or other phenotypes (Geisinger MyCode, = 170,503 individuals, United States; UK Biobank, = 469,789 individuals, United Kingdom) were evaluated for germline pathogenic or likely pathogenic (P/LP) variants in 156 cancer genes.
Results: There were 92 unrelated individuals with DFSP across the 3 cohorts.
