Recurrent abdominal wall dermatofibrosarcoma protuberans in a child: a challenging reconstruction
© Vallam et al. 2015
Received: 23 February 2015
Accepted: 29 June 2015
Published: 8 July 2015
Dermatofibrosarcoma protuberans is an uncommon low-grade soft tissue sarcoma with a high potential for recurrence as it has irregular finger like extensions.
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.
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.
KeywordsDermatofibrosarcoma protuberans Children Abdominal wall Surgery Reconstruction Anterolateral thigh flap
Dermatofibrosarcoma protuberans (DFSP) is an uncommon, low grade soft tissue sarcoma of fibroblast origin. Surgical excision with negative resection margins is crucial to prevent recurrences (Bichakjian et al. 2014). The resultant soft tissue and skin defect often require reconstruction. Anterior abdominal wall defects are technically challenging to reconstruct especially in children since large donor areas are not available due to the small frame of children.
We present our experience in managing a child with recurrent DFSP of the anterior abdominal wall.
The patient had an uneventful recovery and was discharged from the hospital on the eighth postoperative day. All surgical resection margins were negative. The closest margin was 2 mm microscopically although grossly it was 12 mm away. The patient received postoperative radiotherapy (5,040 cGy/28#). At first follow up after three months the flap is well healed and the patient is disease free.
DFSP is a locally aggressive tumor with a high potential for recurrence as it has irregular finger like extensions (Bichakjian et al. 2014). The closest margin in our case was grossly 12 mm away from the tumour, however, on microscopic examination the tumor was extending till 2 mm from the cut margin indicating microscopic spread beyond grossly visualized disease which is difficult to assess intra-operatively. Due to this penchant of DFSP for microscopic extension beyond the gross confines and prior recurrences a wide excision with 2 cm margin was planned.
Available options for reconstruction of large abdominal wall defects
Latissimus dorsi flap
Constant vascular anatomy with long pedicle and large diameter
Significant motor deficit at donor site
Donor defect can be closed primarily if skin paddle required is small—better cosmesis
Available skin paddle is small though muscle bulk is good
Anterolateral thigh flap
Long vascular pedicle with relatively large diameter
Donor site cosmesis is poor
Large skin paddle
Tensor fascia lata flap
Consistent, lengthy vascular pedicle
Donor site cosmesis is poor
No significant functional loss at donor site
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.
SQ performed the surgical resection with the assistance of KCV and MB. VS performed the microvascular reconstruction. KCV drafted the manuscript with the assistance of MB. All authors were involved in review of literature. All authors read and approved the final manuscript.
We would like to thank Mr. Mehboob Shah for meticulous data collection and compilation.
Compliance with ethical guideline
Competing interests The authors declare that they have no competing interests.
Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.
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