Volume 25, Issue 108, February, 2021

Dermatofibrosarcoma protuberans: Our experience of 37 cases, a Single institutional review at NCI, Egypt

Ahmed El-Sayed Fathalla¹, Bahaa El-Din Ahmedmahmoud²

¹Associate Professor of Surgical Oncology-National Cancer Institute-Cairo University, Egypt; Email:drasf1975@gmail.com
²Lecturer of Surgical Oncology-National Cancer Institute-Cairo University, Egypt; Email:bahaaahmed897@yahoo.com

ABSTRACT

Background: DFSP is a rare slow growing locally malignant skin tumor. It is the commonest cutaneous stromal tumor arising in young & middle-aged adults with rare occurrence in paediatrics. It arises as a firm slow-growing nodule that infiltrates deeply with in apparent finger projections beyond clinical margins. Commonly it recurs but rarely metastasizes unless there is a fibrosarcoma component diagnosed by open biopsy & IHC with CD34. Surgery is the main stay of treatment with wide clear margins of 2-3 cm or more beyond clinical borders & down to fascia. Mohs micrographic surgery plays a role especially when wide resection would result in poor cosmetic or functional outcome as on face or ears. Margins of first resection are the main factor to affect local recurrence rates & prognosis. With LNs metastases regional lymphadenectomy offers survival benefit. Lung metastases are rare & occur with multiple local failures after multiple compromised surgeries. Metastasectomy is justified in isolated oligo-metastatic resectable lesions mainly in lungs. CTH is rarely used & RT may be used adjuvant to surgery reducing recurrence when clear margins are not obtained. Imatinib may be considered an option for locally advanced & recurrent lesions. Aims: To study clinicopathological features of all cases of DFSP, presentations, diagnosis, surgical management, complications, reconstructive methods & outcome 'DFS' & 'OS'. Materials & Methods: A single institution prospective analysis of all cases presented to NCI-Cairo University with DFSP candidates for surgery over a period of 5 years from Jan 2015 until Dec 2019. 37 cases included. Data collected from patients archives then analyzed. Results: Mean age was 36.5ys ranging (6-65ys). Males predominated (40 cases, 54.1%). Most lesions were on the trunk (14 cases, 37.9%). Protruding type was the commonest (26 cases, 70.2%). All cases were denovo at initial presentation. 8 cases (21.6%) were on top of previously injured skin. Post resection least diameter ranged (0.5-5.5cm) with mean (2.01±1.36 SD) while largest diameter ranged (0.5-14cm) with mean (3.79±3.08 SD). +ve or close margins (<1cm) encountered in (10 cases, 27%). Local recurrence appeared in (11 cases, 29.7%) for 1st time, in (5 cases, 13.5%) for 2nd time & in (2 cases, 5.4%) for 3rd time. Local recurrence occurred in (12 cases, 32.4%) of primary clear margins & among (7 cases, 18.9%) of primary close margins even after re-resection. Median & mean survival times were 4ys & 4.1yrs respectively. Most patients survived 3 or 4 years (43.2% or 35.1%, respectively). The only significant survival parameter was tumor free margins above 4 cm as free-margin lesions survived longer than those with close-margin lesions (<1cm).

Keywords: Dermatofibrosarcoma protuberans, prognosis, NCI, EGYPT

Medical Science, 2021, 25(108), 492-500

PDF