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Recurrent abdominal wall dermatofibrosarcoma protuberans in a child: a challenging reconstruction.

Vallam KC, Bhagat M, Shankhdhar V, Qureshi SS - Springerplus (2015)

Bottom Line: Dermatofibrosarcoma protuberans is an uncommon low-grade soft tissue sarcoma with a high potential for recurrence as it has irregular finger like extensions.Large composite defect, involving more than half of the anterior abdominal wall, necessitate a free flap reconstruction.Complete surgical excision is essential for DFSP of the abdominal wall, which may result in large challenging defects.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatric Surgical Oncology, Department of Surgical Oncology, Tata Memorial Centre, Ernest Borges Road, Parel, Bombay, 400012 India.

ABSTRACT

Introduction: Dermatofibrosarcoma protuberans is an uncommon low-grade soft tissue sarcoma with a high potential for recurrence as it has irregular finger like extensions.

Case description: We report a case of a large, recurrent dermatofibrosarcoma protuberans in a child involving the anterior abdominal wall, which posed a challenge for reconstruction. Peritoneum sparing full thickness resection of the anterior abdominal wall, meshplasty and a free anterolateral thigh flap was performed for reconstruction of the defect.

Discussion and evaluation: Large composite defect, involving more than half of the anterior abdominal wall, necessitate a free flap reconstruction. Although these reconstructions are technically challenging in children, they are the only option available.

Conclusion: Complete surgical excision is essential for DFSP of the abdominal wall, which may result in large challenging defects. Free flaps remain the only option in this scenario and hence it is essential to have expertise for microvascular flap reconstruction.

No MeSH data available.


Related in: MedlinePlus

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Fig1: Clinical photograph.

Mentions: A 10-year old boy presented with a recurrent abdominal wall tumor. The patient had an incomplete surgery 3 years earlier and had undergone two surgeries subsequently for recurrent swellings. There was neither history of trauma or any family history of similar swellings. Clinically the mass was 8 × 6 cm involving the skin and subcutaneous tissue with an 11 cm horizontal scar with prominent hatch marks and a separate drain site scar (Figure 1). Computerised tomography scan revealed a hypodense, ovoid mass measuring 3.8 × 7.9 × 8.4 cm infiltrating the rectus sheath with no obvious intra-abdominal extension (Figure 2).Figure 1


Recurrent abdominal wall dermatofibrosarcoma protuberans in a child: a challenging reconstruction.

Vallam KC, Bhagat M, Shankhdhar V, Qureshi SS - Springerplus (2015)

Clinical photograph.
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4495091&req=5

Fig1: Clinical photograph.
Mentions: A 10-year old boy presented with a recurrent abdominal wall tumor. The patient had an incomplete surgery 3 years earlier and had undergone two surgeries subsequently for recurrent swellings. There was neither history of trauma or any family history of similar swellings. Clinically the mass was 8 × 6 cm involving the skin and subcutaneous tissue with an 11 cm horizontal scar with prominent hatch marks and a separate drain site scar (Figure 1). Computerised tomography scan revealed a hypodense, ovoid mass measuring 3.8 × 7.9 × 8.4 cm infiltrating the rectus sheath with no obvious intra-abdominal extension (Figure 2).Figure 1

Bottom Line: Dermatofibrosarcoma protuberans is an uncommon low-grade soft tissue sarcoma with a high potential for recurrence as it has irregular finger like extensions.Large composite defect, involving more than half of the anterior abdominal wall, necessitate a free flap reconstruction.Complete surgical excision is essential for DFSP of the abdominal wall, which may result in large challenging defects.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatric Surgical Oncology, Department of Surgical Oncology, Tata Memorial Centre, Ernest Borges Road, Parel, Bombay, 400012 India.

ABSTRACT

Introduction: Dermatofibrosarcoma protuberans is an uncommon low-grade soft tissue sarcoma with a high potential for recurrence as it has irregular finger like extensions.

Case description: We report a case of a large, recurrent dermatofibrosarcoma protuberans in a child involving the anterior abdominal wall, which posed a challenge for reconstruction. Peritoneum sparing full thickness resection of the anterior abdominal wall, meshplasty and a free anterolateral thigh flap was performed for reconstruction of the defect.

Discussion and evaluation: Large composite defect, involving more than half of the anterior abdominal wall, necessitate a free flap reconstruction. Although these reconstructions are technically challenging in children, they are the only option available.

Conclusion: Complete surgical excision is essential for DFSP of the abdominal wall, which may result in large challenging defects. Free flaps remain the only option in this scenario and hence it is essential to have expertise for microvascular flap reconstruction.

No MeSH data available.


Related in: MedlinePlus