Overcoming Challenges of Recurrent Abdominal Wall Dermatofibrosarcoma Protuberans in a Low-resource Setting: Case Report From Ethiopia

Summary:

Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive soft-tissue sarcoma with high recurrence rates. Management of large, recurrent DFSP of the abdominal wall is particularly challenging in low-resource settings due to limitations in specialized care and reconstructive options. A 31-year-old man presented with a recurrent, extensive, fungating abdominal wall mass 8 years after previous surgery. A computed tomography scan revealed an extensive sarcomatous mass, and both fine needle aspiration cytology and incisional biopsy confirmed DFSP. Wide local excision with a 5-cm margin was performed, resulting in a full-thickness abdominal wall defect. Reconstruction was accomplished using a left external oblique myocutaneous flap and a combined pedicled superficial inferior epigastric artery flap with a groin flap (chimeric flap), after intra-abdominal coverage with omentum and mesh. Donor sites were resurfaced with split-thickness skin grafts. A minor wound dehiscence on postoperative day 8 was managed with bedside suturing. The patient was subsequently followed-up and, at the ninth postoperative month, demonstrated an optimal reconstructive outcome with no evidence of recurrence. He was then linked to the oncology unit for ongoing surveillance and care. In resource-limited settings, even extensive recurrent abdominal wall DFSP can be successfully managed with wide excision and strategic regional flap reconstruction when microsurgical options are unavailable.

Dermatofibrosarcoma protuberans (DFSP) is a rare, low-to-intermediate-grade soft-tissue sarcoma of dermal origin, characterized by infiltrative growth and a high rate of local recurrence but low metastatic potential.^1,2 It typically affects young to middle-aged adults and commonly involves the trunk, including the abdominal wall.³

The cornerstone of DFSP management is wide local excision with clear margins. However, recurrent or extensive abdominal wall DFSP poses significant reconstructive challenges, particularly in low-resource settings where oncological and microsurgical capabilities are limited.⁴ Additionally, delays in presentation, suboptimal initial excisions, and poor access to oncological services compound the difficulty in achieving optimal outcomes in such environments.^5–7 We present a case of recurrent, extensive abdominal wall DFSP in Ethiopia successfully managed with wide local excision and reconstruction using regional myocutaneous and chimeric flaps.

CASE REPORT

A 31-year-old man presented with a progressively enlarging anterior abdominal wall mass with a 7-month history. He had undergone a prior surgical excision of a similar mass 8 years earlier. Unfortunately, operative records, histopathology reports, tumor size, surgical margin status, and postoperative follow-up information were unavailable. On examination, a large, ulcerating, fungating, hemorrhagic abdominal wall mass was noted (Fig. 1).

Fig. 1.

Preoperative photograph showing a large, ulcerating, and fungating anterior abdominal wall mass.

Contrast-enhanced computed tomography showed a 19.4 × 9.4 × 18.4 cm ill-defined, lobulated, heterogeneous, fungating soft-tissue mass at the anterior thoracoabdominal wall compressing the left lobe of the liver without intervening fat plane to level IV. No bone involvement was seen. Fine needle aspiration cytology and incisional biopsy both confirmed the diagnosis of DFSP.

Wide local excision with a 5-cm margin was performed under general anesthesia, resulting in a large full-thickness abdominal wall defect (Fig. 2). Although preoperative computed tomography demonstrated loss of the intervening fat plane between the tumor and the left lobe of the liver, intraoperative exploration revealed no gross hepatic invasion, and liver resection was not required. Histopathological examination confirmed DFSP with negative surgical margins. Immunohistochemical studies demonstrated CD34 positivity, further confirming the diagnosis of DFSP. The viscera were covered with omentum and a nonabsorbable mesh to provide intra-abdominal support.

Fig. 2.

Intraoperative image showing a full-thickness abdominal wall defect following wide local excision.

Reconstruction was achieved using a left external oblique myocutaneous flap from the upper abdominal wall and a combined pedicled superficial inferior epigastric artery (SIEA) flap and groin flap (chimeric flap) from the right lower abdominal region. Additional intraoperative photographs demonstrating flap design and boundaries are provided in Supplemental Digital Contents 1 and 2. (See figure, Supplemental Digital Content 1, which displays the intraoperative photograph demonstrating elevation of a left external oblique myocutaneous flap and a pedicled SIEA–groin chimeric flap in relation to the abdominal wall defect, https://links.lww.com/PRSGO/F350.) (See figure, Supplemental Digital Content 2, which displays the intraoperative photograph demonstrating elevation of a pedicled greater omental flap, https://links.lww.com/PRSGO/F351.) Donor sites of both flaps were resurfaced with split-thickness skin grafts from the thigh, which healed well. The flaps were inset tension-free with appropriate drains (Fig. 3). The patient was admitted for 2 weeks. On postoperative day 8, a minor wound dehiscence developed at the junction of the external oblique and chimeric flaps, which was sutured at the bedside under local anesthesia. Recovery was otherwise uneventful.

Fig. 3.

Intraoperative view immediately after abdominal wall reconstruction using a left external oblique myocutaneous flap and a right lower side SIEA–groin chimeric flap, with omentum and mesh providing intra-abdominal protection.

At 9 months of follow-up, no local recurrence was noted. The abdominal wall was functionally and cosmetically satisfactory (Fig. 4). The patient was linked to the oncology unit for further follow-up and long-term surveillance.

Fig. 4.

Postoperative photograph at the 9-month follow-up, demonstrating a well-healed abdominal wall with no evidence of recurrence and satisfactory functional and aesthetic outcomes.

DISCUSSION

DFSP is a rare but locally aggressive malignancy with a high tendency for local recurrence due to its infiltrative growth and microscopic extensions.^1–3 Wide local excision with negative margins is the mainstay of treatment.^2,4,8 In well-resourced settings, Mohs micrographic surgery or wide excision with histological control achieves excellent local control.¹

However, in low-resource settings, the management of DFSP faces multiple obstacles. Delayed presentation is frequent, largely due to poor public awareness, healthcare access limitations, and lack of early specialist referral. Suboptimal initial excisions performed without histopathological confirmation or adequate surgical margins are also common, increasing the likelihood of local recurrence, as was evident in this case. Furthermore, limited oncology services, absence of multidisciplinary tumor boards, and lack of radiotherapy facilities further compromise comprehensive care and long-term outcomes for patients with DFSP in such environments.^5–7

Abdominal wall reconstruction following large DFSP resection is complex. Although microsurgical flaps are considered ideal in high-resource settings,⁹ the combination of regional options—such as the external oblique myocutaneous flap and pedicled SIEA–groin chimeric flap—provides a safe and reliable alternative in environments lacking microsurgical capabilities. Several reconstructive options have been described for large full-thickness abdominal wall defects, including free tissue transfer, tensor fasciae latae flaps, anterolateral thigh flaps, and rectus abdominis flaps. However, many of these techniques require microsurgical expertise, specialized equipment, prolonged operative times, and advanced postoperative monitoring, which may not be readily available in low-resource settings.

In the present case, the external oblique myocutaneous flap was selected because of its proximity to the defect, reliable vascular supply, and ability to provide substantial soft-tissue coverage. The pedicled SIEA–groin chimeric flap offered additional well-vascularized tissue while avoiding microvascular anastomosis. The combination of these regional flaps allowed tension-free coverage of the extensive defect with acceptable donor-site morbidity. Although free flaps may provide greater versatility and larger tissue volume, the selected regional flaps represented a practical and reliable reconstructive option in the present setting.

The availability of microsurgical services remains a significant challenge in low-resource settings due to factors, including the high cost of specialized equipment, limited access to trained microsurgeons, and inadequate perioperative support infrastructure, such as intensive care units and advanced monitoring. Additionally, prolonged operative times and the need for meticulous postoperative care make microsurgical procedures difficult to implement consistently in these environments. Therefore, reliance on regional flaps that do not require microvascular anastomosis but still achieve effective reconstruction is a pragmatic and necessary approach, as demonstrated in this case.

This case highlights the potential to achieve satisfactory outcomes through the strategic use of local and regional flaps for reconstruction in low-income settings. It also underscores the critical importance of referring patients to oncology services for ongoing surveillance, given that DFSP can recur even years after excision. To improve management and outcomes, initial excisions of soft-tissue tumors should be performed with adequate surgical margins and histopathological confirmation. Early referral to specialized centers is essential when DFSP is suspected. Moreover, strengthening oncology services and establishing multidisciplinary tumor boards in low-resource countries are crucial steps toward enhancing sarcoma care and patient prognosis.¹⁰

CONCLUSIONS

In resource-limited settings, managing recurrent large abdominal wall DFSP presents significant challenges but can be effectively addressed through wide local excision combined with regional flap reconstruction when microsurgical options are unavailable. Continued long-term surveillance remains essential because of the risk of late recurrence. Early diagnosis, adequate surgical margins during initial excision, and timely referral to specialized centers are essential to reduce morbidity and recurrence.

DISCLOSURE

The authors have no financial interest to declare in relation to the content of this article.