Cancer Res Treat.  2014 Oct;46(4):366-373. 10.4143/crt.2013.185.

Clinical Characteristics and Adequate Treatment of Familial Adenomatous Polyposis Combined with Desmoid Tumors

• Jung WB
• Kim CW
• Kim JC

• KMID: 2380374
• DOI: http://doi.org/10.4143/crt.2013.185

Abstract

PURPOSE
The objective of this study was to examine the clincopathologic characteristics and outcomes of familial adenomatous polyposis (FAP) patients with and without desmoid tumors (DTs), including the risk factors for progression of FAP-related DTs.
MATERIALS AND METHODS
We reviewed the medical records and database of all patients with FAP who were treated between January 1993 and December 2011.
RESULTS
Of 75 FAP patients, 18 (24%) were FAP with DTs. Seventeen of these had intra-abdominal DTs and one had intra- and extra-abdominal DTs. We divided the patients into two groups according to type of resection; the R0 or R1 resection group, referred to as the curative resection group (eight patients), and the R2 resection/palliative operation/medical treatment group, referred to as the palliative resection group (10 patients). Two patients in the curative resection group and two patients in the palliative group had progressed to tumor growth by the follow-up (p=0.800). In univariate analysis, DT diagnosis before or simultaneously with FAP diagnosis (DTs unrelated to surgical trauma) was a significant risk factor for tumor progression at final follow-up.
CONCLUSION
A multidisciplinary approach to DT treatment is needed, including nonsteroidal antiinflammatory drugs, anti-estrogens, cytotoxic agents, and surgery. However, the role of surgery in resectable and complicated tumors may be limited. DT unrelated to surgical trauma has a relatively poor prognosis.

Keyword

Familial adenomatous polyposis; Aggressive fibromatosis; Risk factors

MeSH Terms

Adenomatous Polyposis Coli*
Cytotoxins
Diagnosis
Fibromatosis, Aggressive*
Follow-Up Studies
Humans
Medical Records
Prognosis
Risk Factors
Cytotoxins

Figure

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  Fig. 1. Overall survival curves of familial adenomatous polyposis (FAP) with and without desmoid tumors.

Reference

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