Purpose <p>Desmoid tumors are rare neoplasms of mesenchymal origin. These tumors are locally aggressive but they lack metastatic potential. Treatment involves a multimodal strategy, usually involving surgical excision with or without reconstructive procedure, is of paramount importance. Adjuvant therapies, including chemotherapy, radiotherapy, and hormonal therapy, are now being followed. The rarity of the disease, coupled with its heterogeneous clinical manifestations and the wide array of therapeutic modalities, poses substantial challenges to clinical decision-making, thereby creating a therapeutic dilemma in the development of an individualized and optimal treatment strategy.</p> Methods <p>A 47-year-old postmenopausal woman presented with a slow-growing, painful abdominal mass with bleeding and foul-smelling discharge. She had a prior history of abdominal mass removal and myomectomy. Examination revealed a 20×15×15 cm ulcero-proliferative lesion with surrounding skin induration over the anterior abdominal wall. Imaging revealed multiple anterior abdominal wall tumors without intra-abdominal invasion. Core needle biopsy confirmed desmoid tumor. Preoperative therapy including tamoxifen and sulindac. Wide local excision with split-thickness skin grafting was performed. Histopathology showed spindle cells in collagenous stroma with R1 margins and no lymph node involvement. Immunohistochemistry revealed SMA positivity and low Ki67 index. Postoperative radiotherapy was administered. Patient was followed for a period of one year.</p> Discussion <p>Desmoid tumors may occur sporadically or as part of inherited syndromic conditions. Desmoid tumors have a varied presentation depending on the anatomical location. It is classified into three distinct categories based on anatomical location: intra-abdominal, abdominal wall-associated, and extra-abdominal.</p> Conclusion <p>Desmoid tumors are rare, locally aggressive, non-metastatic neoplasms with significant morbidity. Their complex presentation demands individualized management. Though the disease has low mortality especially outside FAP syndrome, they can cause severe morbidity. Continued research and clinical trials are essential to improve therapeutic strategies and outcomes.</p>

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Anterior abdominal wall desmoid tumor: a rare surgical dilemma

  • Tharun Ganapathy Chitrambalam,
  • Maramreddy Lokesh Reddy,
  • S. Keerthana,
  • Ramprakash Ramanathan,
  • Sowmiya Jayachandran,
  • A. Ashwin Rangan

摘要

Purpose

Desmoid tumors are rare neoplasms of mesenchymal origin. These tumors are locally aggressive but they lack metastatic potential. Treatment involves a multimodal strategy, usually involving surgical excision with or without reconstructive procedure, is of paramount importance. Adjuvant therapies, including chemotherapy, radiotherapy, and hormonal therapy, are now being followed. The rarity of the disease, coupled with its heterogeneous clinical manifestations and the wide array of therapeutic modalities, poses substantial challenges to clinical decision-making, thereby creating a therapeutic dilemma in the development of an individualized and optimal treatment strategy.

Methods

A 47-year-old postmenopausal woman presented with a slow-growing, painful abdominal mass with bleeding and foul-smelling discharge. She had a prior history of abdominal mass removal and myomectomy. Examination revealed a 20×15×15 cm ulcero-proliferative lesion with surrounding skin induration over the anterior abdominal wall. Imaging revealed multiple anterior abdominal wall tumors without intra-abdominal invasion. Core needle biopsy confirmed desmoid tumor. Preoperative therapy including tamoxifen and sulindac. Wide local excision with split-thickness skin grafting was performed. Histopathology showed spindle cells in collagenous stroma with R1 margins and no lymph node involvement. Immunohistochemistry revealed SMA positivity and low Ki67 index. Postoperative radiotherapy was administered. Patient was followed for a period of one year.

Discussion

Desmoid tumors may occur sporadically or as part of inherited syndromic conditions. Desmoid tumors have a varied presentation depending on the anatomical location. It is classified into three distinct categories based on anatomical location: intra-abdominal, abdominal wall-associated, and extra-abdominal.

Conclusion

Desmoid tumors are rare, locally aggressive, non-metastatic neoplasms with significant morbidity. Their complex presentation demands individualized management. Though the disease has low mortality especially outside FAP syndrome, they can cause severe morbidity. Continued research and clinical trials are essential to improve therapeutic strategies and outcomes.