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Surg Today. 2017 Oct;47(10):1259-1267. doi: 10.1007/s00595-017-1500-3. Epub 2017 Mar 1.

The treatment of desmoid tumors associated with familial adenomatous polyposis: the results of a Japanese multicenter observational study.

Author information

1
Department of Gastrointestinal and Pediatric Surgery, Division of Reparative Medicine, Institute of Life Sciences, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu, Mie, 514-8507, Japan. yasinoue@clin.medic.mie-u.ac.jp.
2
Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University, Saitama, Japan.
3
Department of Surgery, National Defense Medical College, Saitama, Japan.
4
Center for Minimally Invasive Surgery, Tokyo Medical and Dental University, Tokyo, Japan.
5
Department of Surgery, Tokyo Metropolitan Cancer and Infectious Diseases Center, Komagome Hospital, Tokyo, Japan.
6
Gastroenterological Center, Department of Gastroenterological Surgery, Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan.
7
Department of Surgery, Hyogo College of Medicine, Hyogo, Japan.
8
Digestive Disease Center, Showa University Northern Yokohama Hospital, Kanagawa, Japan.
9
Department of Gastroenterological and Transplant Surgery Applied Life Sciences, Institute of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan.
10
Division of Colorectal Surgery, National Cancer Center Hospital, Tokyo, Japan.
11
Department of Surgical Oncology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
12
Department of Surgical Oncology, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan.

Abstract

PURPOSE:

Familial adenomatous polyposis (FAP)-associated desmoid tumor (DT) is sometimes life threatening. However, the optimal treatment for DTs has not been established. The aim of this study was to analyze the outcomes of surgical and pharmacological treatments for DT in Japanese FAP patients.

METHODS:

We retrospectively reviewed the data of 303 patients who underwent colectomy for FAP between 2000 and 2012. We analyzed 41 patients with DTs in which the location was apparent. The selection of treatment for intra-abdominal DTs was also evaluated according to Church's classification.

RESULTS:

Surgery was frequently used to treat extra-abdominal DTs. Multimodal treatments, including surgery, and the administration of non-steroidal anti-inflammatory drugs, hormonal therapy, and chemotherapy were widely used for intra-abdominal DTs. The most effective pharmacological treatment was cytotoxic chemotherapy, which was associated with a response rate of 45.5% and a disease control rate of 72.7%. After a median follow-up period of 53.0 months, the 5-year DT-specific survival rate in patients with stage IV disease was 71.4%; in contrast, the rate in patients with other stages was 100%. Four-stage IV patients died of DT due to uncontrollable rapid progression. No cytotoxic chemotherapy was administered; however, incomplete resection was performed in three cases.

CONCLUSION:

Our findings will provide clues that may help physicians in selecting the optimal strategy for this rare disease.

KEYWORDS:

Chemotherapy; Desmoid tumor; Familial adenomatous polyposis; Pharmacological treatment

PMID:
28251376
DOI:
10.1007/s00595-017-1500-3
[Indexed for MEDLINE]

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