Desmoid tumor natural history, complications, and prognosis
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Desmoid tumor Microchapters |
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Diagnosis |
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Treatment |
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Case Studies |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]Faizan Sheraz, M.D. [3]
Overview
If left untreated, desmoid tumor produces few symptoms early in the course. Common complications of desmoid tumor include progression to agressive fibromatosis, involvement of intra abdominal viscera, and metastasis. The prognosis varies with the associated disease of desmoid tumor. Desmoid tumor associated with FAP has the least favorable prognosis.
Natural history
- If left untreated, desmoid tumor produces few symptoms early in the course.
Complications
Common complications of desmoid tumor include:
- Progression to aggressive fibromatosis
- Involvement of intra abdominal organs
- Metastasis
Prognosis
- Desmoid tumors have become an important cause of morbidity and mortality due to increasing use of prophylactic colectomy in FAP patients[1][2][3][4][5][6]
- Desmoids when become massive and inoperable, can cause death in approximately 11% of FAP patients post-colectomy surgery
- Due to gradual progression to desmoids, 60% of FAP patients remain alive for 10 years after the diagnosis
- Pregnancy-associated desmoid fibromatosis is associated with overall good outcomes without any attributable obstetric complications
Reference
- ↑ Church JM (1998). "Mucosal ischemia caused by desmoid tumors in patients with familial adenomatous polyposis: report of four cases". Dis Colon Rectum. 41 (5): 661–3. PMID 9593254.
- ↑ Sagar PM, Möslein G, Dozois RR (1998). "Management of desmoid tumors in patients after ileal pouch-anal anastomosis for familial adenomatous polyposis". Dis Colon Rectum. 41 (11): 1350–5, discussion 1355-6. PMID 9823798.
- ↑ Penna C, Tiret E, Parc R, Sfairi A, Kartheuser A, Hannoun L; et al. (1993). "Operation and abdominal desmoid tumors in familial adenomatous polyposis". Surg Gynecol Obstet. 177 (3): 263–8. PMID 8395084.
- ↑ Kadmon M, Möslein G, Buhr HJ, Herfarth C (1995). "[Desmoid tumors in patients with familial adenomatous polyposis (FAP). Clinical and therapeutic observations from the Heidelberg polyposis register]". Chirurg. 66 (10): 997–1005. PMID 8529453.
- ↑ Arvanitis ML, Jagelman DG, Fazio VW, Lavery IC, McGannon E (1990). "Mortality in patients with familial adenomatous polyposis". Dis Colon Rectum. 33 (8): 639–42. PMID 2165452.
- ↑ Quintini C, Ward G, Shatnawei A, Xhaja X, Hashimoto K, Steiger E; et al. (2012). "Mortality of intra-abdominal desmoid tumors in patients with familial adenomatous polyposis: a single center review of 154 patients". Ann Surg. 255 (3): 511–6. doi:10.1097/SLA.0b013e31824682d4. PMID 22323009.