Desmoid tumor natural history, complications, and prognosis

Desmoid tumor Microchapters
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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

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

• 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

Common complications of desmoid tumors include:

• Progression to aggressive fibromatosis
• Surgical complications such as:
  • Amputation
  • Loss of significant portions of foregut
• Intra-abdomianl/pelvic desmoids can 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

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

• 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
• Desmoids when become massive and inoperable, can cause death in overall 11% of FAP patients post-colectomy surgery
  • 18-31% mortality in patients having desmoids with colorectal carcinoma
  • 22% mortality in desmoid patients with periampullary carcinomas
• 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
• 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

Recurrence of desmoid tumors

• Recurrence rate is 20% to 77% depending on the location, extent and completeness of the initial resection
• Abdominal wall desmoid tumors have a significantly lower recurrence rate of 20% to 30%
• Abdominal wall desmoids usually becomes evident within six months after excision or in connection with subsequent gestations or deliveries

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:

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/ureteric obstruction 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%