An Integrated Surgical Management for Giant Dermatofibrosarcoma Protuberans of Anterior Abdominal Wall

Swagata Brahmachari,Anubha Pandey,Mahendra Pratap Singh,Vandana Agarwal

doi:10.7759/cureus.17038

Abstract

Giant dermatofibrosarcoma protuberans (DFSP) is a very rare dermal sarcoma whose diagnosis and management are important because of the high local recurrence but low metastatic potential. Complete surgical excision of giant DFSP in a single stage is difficult but has a high cure rate.A 47-year-old man presented with a gradually increasing large (18 x 15 x 7 cm) DFSP in the epigastrium. A 3 cm circumferential wide local excision (WLE) with microscopic tumor-free margin confirmed by frozen section was performed. Immediate single staged tension-free primary closure of resultant defect was done on the principle of abdominal wall reconstruction (AWR) in ventral hernia repair. This technique of anterior component separation and bridge meshplasty is functional, avoids multiple surgeries, is cost-effective, and can be done in a resource-limited setting in developing countries.A multidisciplinary and integrated surgical approach to treat giant DFSP over epigastrium, by three-dimensional WLE and immediate AWR with anterior component separation technique (CST) and bridging meshplasty, can be of immense help in managing such rare cases in developing countries.

Highlights

Dermatofibrosarcoma protuberans (DFSP) are rare, cutaneous mesenchymal neoplasm with an annual global incidence of 0.8-4.5 cases per million persons. Hoffman named this rare clinical entity DFSP which accounts for 1-6% of soft tissue sarcomas and 18% of cutaneous soft tissue sarcomas [1]
It is composed of CD-34 positive, neoplastic spindle cells with high local recurrence and low metastatic potential [2]
Large-sized DFSP and delayed presentation are often due to rarity and misdiagnosis as a benign tumor because of its indolent evolution. It presents as a 1-6 cm-sized tumor, but rarely >20 cm have been reported [2]. Clinical awareness of this uncommon oncological entity is important as complete surgical excision with histologically negative margin has an excellent prognosis with a 99% five-year survival rate [3]

Summary

Introduction

Dermatofibrosarcoma protuberans (DFSP) are rare, cutaneous mesenchymal neoplasm with an annual global incidence of 0.8-4.5 cases per million persons. We present an integrated surgical approach to treat a giant DFSP over epigastrium by three-dimensional wide local excision (WLE) and immediate abdominal wall reconstruction (AWR) by anterior component separation technique (CST) with bridging meshplasty. A 15 x 14 cm large, lobulated, firm, non-tender tumor covered by shiny telangiectatic skin was present over the anterior abdominal wall of the epigastrium (Figure 1A). The resultant full-thickness abdominal wall defect of size 22 x 19 cm (Figure 1D) was reconstructed by modified anterior CST with bridging meshplasty. 1(B): MRI (chest and abdomen) showing large heterogenous mass (16.4 x 14.6 x 9.7 cm) on the anterior abdominal wall in the epigastric region, arising from rectus abdominis muscle, and the gastric antrum appears adherent to the deeper margin of the lesion. 2(D): Immunohistochemistry of tumor cells showing cluster of differentiation for CD 34 antigen (magnification 40X)

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