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
- Desmoid tumors can develop at virtually any site in the body
- They can have a wide range of clinical symptoms or no symptoms at all
- Many desmoid tumors are accidentally picked up on a scan or a routine physical exam done for other medical reasons
- If left untreated, desmoid tumor produces few symptoms early in the course
- Patients are asymptomatic at the time of diagnosis
- Patients can remain asymptomatic even after many years
- Patients can have a wide range of symptoms anytime depending upon the size and location of tumor
- On some occasions, desmoid tumors can shrink or become quiescent (deep sleep) without any therapy
- Some desmoids may be stable or grow very slowly over a period of months and years
- A small subset of desmoid tumors may be aggressive and grow rapidly
- Hence, desmoid tumors exhibit wide range of behaviors varying from person to person
Complications
- Desmoid tumors can progrss to aggressive fibromatosis
- Intra-abdomianl/pelvic desmoids can lead to:
Prognosis
- Five-year progression-free survival rate is 50% in patients with a primary or recurrent desmoid who are not treated initially with surgery, radiotherapy (RT), or systemic therapy
- 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 overall 11% of FAP patients post-colectomy surgery
- 18-31% motality in patients having desmoids with colorectal carcinoma
- 22% mortality in desmoid patients with periampullary carcinomas[7]
- Due to gradual progression to desmoids, 60% of FAP patients remain alive for 10 years after the diagnosis[6][8][9][10][11][12]
- Pregnancy-associated desmoid fibromatosis is associated with overall good outcomes without any attributable obstetric complications
- FAP-associated intra-abdominal fibromatosis has a high recurrence rate after surgical resection
- Non-FAP-associated intra-abdominal fibromatosis has a low recurrence rate after surgical resection[13]
Prognostic stratification system for FAP-associated desmoids (Cleveland Clinic)
- Following Prognostic stratification system for FAP-associated desmoids was proposed after a study was done in Cleveland Clinic:[6]
| Tumor stage | Characteristic features | Five-year survival rate |
|---|---|---|
| Stage I |
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95% |
| Stage II |
|
100% |
| Stage III |
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89% |
| Stage IV |
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76% |
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.
- ↑ 6.0 6.1 6.2 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.
- ↑ Seow-Choen F (2008). "The management of desmoids in patients with familial adenomatous polyposis (FAP)". Acta Chir Iugosl. 55 (3): 83–7. PMID 19069698.
- ↑ Soravia C, Berk T, McLeod RS, Cohen Z (2000). "Desmoid disease in patients with familial adenomatous polyposis". Dis Colon Rectum. 43 (3): 363–9. PMID 10733118.
- ↑ Goy BW, Lee SP, Eilber F, Dorey F, Eckardt J, Fu YS; et al. (1997). "The role of adjuvant radiotherapy in the treatment of resectable desmoid tumors". Int J Radiat Oncol Biol Phys. 39 (3): 659–65. PMID 9336146.
- ↑ Lynch HT, Fitzgibbons R (1996). "Surgery, desmoid tumors, and familial adenomatous polyposis: case report and literature review". Am J Gastroenterol. 91 (12): 2598–601. PMID 8946994.
- ↑ Lefevre JH, Parc Y, Kernéis S, Goasguen N, Benis M, Parc R; et al. (2008). "Risk factors for development of desmoid tumours in familial adenomatous polyposis". Br J Surg. 95 (9): 1136–9. doi:10.1002/bjs.6241. PMID 18581438.
- ↑ Schiessling S, Kihm M, Ganschow P, Kadmon G, Büchler MW, Kadmon M (2013). "Desmoid tumour biology in patients with familial adenomatous polyposis coli". Br J Surg. 100 (5): 694–703. doi:10.1002/bjs.9053. PMID 23334997.
- ↑ Wilkinson MJ, Fitzgerald JE, Thomas JM, Hayes AJ, Strauss DC (2012). "Surgical resection for non-familial adenomatous polyposis-related intra-abdominal fibromatosis". Br J Surg. 99 (5): 706–13. doi:10.1002/bjs.8703. PMID 22359346.