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At a glance

It is an autosomal dominant disorder that is characterized by infiltrative fibromatosis of the mesentery without colonic polyps, osteomas, and sebaceous cysts. It is known to predispose to desmoid tumor but its association with colon cancer is unknown.

Incidence and genetic inheritance

Incidence unknown. Autosomal dominant inheritance.

Clinical aspects

These are fibroproliferative disorders in which histologically benign tumors of musculoaponeurotic tissue arise and are often locally invasive. They occur more commonly in patients with familial adenomatous polyposis. The fibromatosis may be intraabdominal (mesenteric) or extraabdominal, such as paraspinal, limb, buttock, flank, head, and neck.

Anesthetic considerations

Patients receive multidisciplinary treatment, so they may present for surgery after having received chemotherapy or radiotherapy. Patients with tumors of the head and neck may require careful airway management planning according to the site of the tumor(s).

References

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Dormans  JP, Spiegel  D, Meyer  J,  et al: Fibromatoses in childhood: The desmoid/fibromatosis complex. Med Pediatr Oncol 37:126, 2001.  [PubMed: 11496351]
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Robanus-Maandag  E, Bosch  C, Amini-Nik  S,  et al: Familial adenomatous polyposis-associated desmoids display significantly more genetic changes than sporadic desmoids. PLoS One 6(9):e24354, 2011. https://doi.org/10.1371/journal.pone.0024354  [PubMed: 21931686]

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