Aggressive fibromatosis

Aggressive fibromatosis
Classification and external resources
MR scan of pelvis - desmoid tumor
DiseasesDB	29794
eMedicine	article/1060887
MeSH	D018222

Aggressive fibromatosis is a rare condition marked by the presence of desmoid tumors, which are benign, slow-growing tumors without any metastatic potential. However, Aggressive Fibromatosis is locally aggressive. Despite their benign nature, they can damage nearby structures causing organ dysfunction. Most cases are sporadic, but some are associated with familial adenomatous polyposis (FAP). Approximately 10% of individuals with Gardner's syndrome, a type of FAP with extracolonic features, have desmoid tumors.^[1]

Histologically they resemble low-grade fibrosarcomas,^[2] but they are very locally aggressive and tend to recur even after complete resection. There is a tendency for recurrence in the setting of prior surgery; in one study, two-thirds of patients with desmoid tumors had a history of prior abdominal surgery.^[3]

Risk factors for desmoid disease amongst FAP patients include female gender, a 3' APC mutation, a positive family history and a history of previous abdominal surgery. ^[4]

Classification

Desmoid tumors may be classified as extra-abdominal, abdominal wall, or intra-abdominal (the last is more common in patients with FAP). It is thought that the lesions may develop in relation to estrogen levels or trauma/operations.

A 3' APC mutation is the most significant risk factor for intra-abdominal desmoid development amongst FAP patients.^[5]FAP patients presenting with an abdominal wall desmoid pre-operatively are at an increased risk of developing an intra-abdominal desmoid post-operatively.^[6]

Treatment

Treatment may consist of watching and waiting, complete surgical removal, amputation of infected limb, radiation therapy, antiestrogens and NSAIDs, or chemotherapy.

Current experimental studies are being done with Gleevec (Imatinib) for treatment of Desmoid tumors, and show promising success rates.

See also

• List of cutaneous conditions

References

1. ^ Nieuwenhuis MH, De Vos Tot Nederveen Cappel W, Botma A, et al (February 2008). "Desmoid tumors in a Dutch cohort of patients with familial adenomatous polyposis". Clin. Gastroenterol. Hepatol. 6 (2): 215–9. doi:10.1016/j.cgh.2007.11.011. PMID 18237870. http://linkinghub.elsevier.com/retrieve/pii/S1542-3565(07)01107-X. 
2. ^ "desmoid" at Dorland's Medical Dictionary
3. ^ Lynch HT, Fitzgibbons R (December 1996). "Surgery, desmoid tumors, and familial adenomatous polyposis: case report and literature review". Am. J. Gastroenterol. 91 (12): 2598–601. PMID 8946994. 
4. ^ Sinha A, Clark SK (2010). "Risk factors predicting desmoid occurrence in patients with familial adenomatous polyposis: a meta-analysis". Colorectal Dis.: no. doi:10.1111/j.1463-1318.2010.02345.x. PMID 20528895. 
5. ^ Sinha A, Clark SK (June 2010). "Risk factors predicting intra-abdominal desmoids in familial adenomatous polyposis: a single centre experience". Tech Coloproctol. 14 (2): 141–6. doi:10.1007/s10151-010-0573-4. PMID 20352275. 
6. ^ Sinha A, Clark SK (2010). "Surgical prophylaxis in familial adenomatous polyposis: do pre-existing desmoids outside the abdominal cavity matter?". Fam Cancer 9 (3): 407–11. doi:10.1007/s10689-010-9342-9. PMID 20428953. 

External links

• Aggressive (Desmoid) Fibromatosis: A Problem for the Sarcoma Team by Randall Burt, MD and David Viskochil, MD, PhD

• Desmoid Tumor Research Foundation

• [1]

See also

• Benjamin Alman