Desmoid tumor natural history, complications, and prognosis

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Differentiating Desmoid tumor from other Diseases

Epidemiology and Demographics

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Natural History, Complications and Prognosis

Diagnosis

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Case Studies

Case #1

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
  • 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

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 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

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]
Prognostic stratification system for FAP-associated desmoids (Cleveland Clinic)
Tumor stage Characteristic features Five-year survival rate
Stage I
  • Asymptomatic
  • <10 cm maximum diameter
  • Not growing
 95%
Stage II
  • Mildly symptomatic (sensation of mass or pain but no restriction)
  • <10 cm in maximum diameter
  • Not growing
 100%
Stage III
  • Moderately symptomatic (sensation of mass, pain, restrictive but not hospitalized)
  • Bowel/uretericobstruction
  • 10 to 20 cm in size
  • Slowly growing
 89%
Stage IV
  • Severely symptomatic (sensation of mass, pain, restrictive and hospitalized)
  • Septic complications such as fistula and abscess
  • >20 cm in size
  • Rapidly growing
 76%

Reference

  1. ↑ 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.
  2. ↑ 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.
  3. ↑ 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.
  4. ↑ 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.
  5. ↑ 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. ↑ 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.
  7. ↑ Seow-Choen F (2008). "The management of desmoids in patients with familial adenomatous polyposis (FAP)". Acta Chir Iugosl. 55 (3): 83–7. PMID 19069698.
  8. ↑ 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.
  9. ↑ 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.
  10. ↑ 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.
  11. ↑ 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.
  12. ↑ 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.

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