Desmoid tumors

Last updated: July 11, 2022

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Desmoid tumors are slow-growing, mostly benign but locally aggressive tumors caused by the proliferation of fibroblasts. They are often associated with familial adenomatous polyposis and can arise from any part of the body, most commonly the extremities, abdominal wall, and the abdominal cavity. Individuals may be asymptomatic until the tumors grow large enough to compress adjacent structures (e.g., bowel obstruction). Diagnosis includes imaging and requires biopsy with immunohistochemistry for confirmation. Treatment is based on active surveillance with MRI.

Epidemiological data refers to the US, unless otherwise specified.

Clinical findings may vary widely according to age, tumor size, and location.

  • Painless mass
  • Nerve or muscle compression may cause localized pain or tenderness
  • Clinical features of bowel obstruction (e.g., abdominal pain, distention, nausea, vomiting)

The differential diagnoses listed here are not exhaustive.

  • Desmoid tumors are locally aggressive and associated with a high local recurrence rate but lack metastatic potential.
  • Unpredictable clinical course (from spontaneous regression to progression and stable chronic disease)
  • Desmoid tumors may compress vital organs and disrupt their function with potentially fatal consequences.
  1. Bonvalot S, Desai A, Coppola S, et al. The treatment of desmoid tumors: a stepwise clinical approach. Annals of Oncology. 2012; 23 : p.x158-x166. doi: 10.1093/annonc/mds298 . | Open in Read by QxMD
  2. Alman B, Attia S, Baumgarten C, et al. The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients. Eur J Cancer. 2020; 127 : p.96-107. doi: 10.1016/j.ejca.2019.11.013 . | Open in Read by QxMD
  3. Kasper B, Ströbel P, Hohenberger P. Desmoid Tumors: Clinical Features and Treatment Options for Advanced Disease. Oncologist. 2011; 16 (5): p.682-693. doi: 10.1634/theoncologist.2010-0281 . | Open in Read by QxMD

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