Desmoid tumor natural history, complications, and prognosis

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Desmoid tumor Microchapters

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Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Desmoid tumor from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

CT

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sara Mohsin, M.B.B.S.[2]Faizan Sheraz, M.D. [3]

Overview

Desmoids can develop virtually at any site of body causing wide range of clinical symptoms depending on size and tumor location or they can cause no symptoms at all. They may be accidentally picked up on a scan or a routine physical examination done for other medical reasons. They might grow aggressively or slowly or remian stable, hence, exhibiting wide range of behaviors in different individuals. Common complications of desmoid tumor include progression to agressive fibromatosis, involvement of intra-abdominal viscera that may lead to rupture of intestines, compression of the kidneys or ureters, rectal bleeding, compression of critical blood vessels such as the mesenteric vessels and the vena cava, amputation and loss of significant portions of foregut post-surgery. 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. Massive and inoperable desmoids have become an important cause of morbidity and mortality in FAP patients post-colectomy. Desmoid tumor associated with FAP has the least favorable prognosis due to high recurrence rate. Whereas, non-FAP-associated intra-abdominal fibromatosis has a low recurrence rate after surgical resection.

Natural history

Complications

Common complications of desmoid tumors include:

The progression of the intra-abdominal desmoid in patient 2. a CT scan before DOX/DTIC therapy showing a large mass in the abdomen and a desmoid tumor on the left abdominal wall. b CT scan of the same patient after DOX/DTIC therapy, showing tumor regression and perforation (as indicated by the white arrow, the gas was encapsulated by the tumor). c Follow-up CT scan in December 2015 Source: Li W. et al, Department of Oncology, Zhongshan Hospital, Fudan University, Shanghai, China

Prognosis

Recurrence of desmoid tumors

Prognostic stratification system for FAP-associated desmoids (Cleveland Clinic)

Prognostic stratification system for FAP-associated desmoids (Cleveland Clinic)
Tumor stage Characteristic features Five-year survival rate
Stage I 95%
Stage II 100%
Stage III 89%
Stage IV 76%

Reference

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