[The Characteristics and Treatment Outcomes for Desmoid Tumors Associated with Familial Adenomatous Polyposis].

Abstract

INTRODUCTION:

The characteristics of desmoid tumors(DTs)associated with familial adenomatous polyposis(FAP)and relationships between the development of DTs and the sites of APC germline mutation have not closely been examined Japan.

PATIENTS AND METHODS:

This retrospective study was performed to address these issues by examining patients with FAP who underwent proctocolectomy between 1981 and 2015.

RESULTS:

The cumulative 2-year incidence of DT development was 50%. The DTs developed in the abdominal wall only in 2, in the abdominal wall plus intra-abdominally in 4, and intra-abdominally in 2. Clinical stages according to the Church's classification included Stage I in 3, Stage II in 2, Stage III in 1, and Stage IV in 2. Among 31 patients with a confirmed pathogenic germline APC mutation, patients with mutation in 3' site of codon 1400 (n=7)tended to develop DTs more frequently than those with mutation in 5' site of codon 1400(n=24)(p=0.08). The cumulative 5-year survival rate in patients with DT development was 73.3%. Including 2 patients undergoing initial colectomy at other institutions, the therapeutic efficacy in 4 patients with severe intraabdominal DTs(Stage III /Stage IV )who were given chemotherapy comprising doxorubicin plus dacarbazine(DOX plus DTIC)revealed partial response in 3 and complete response in 1. Febrile neutropenia was recorded in 2 of these patients.

CONCLUSION:

The frequency of DTs development and genotype-phenotype relationship of FAP patients seems to concur with those reported from Western countries. Since the DOX plus DTIC therapy for severe DTs is valid but toxicity is high, the development of less toxic regimens are warranted.

• Authors: Kunihiko Amano, Noriyasu Chika, Tetsuya Ito, Azusa Yamamoto, Satoshi Hatano, Toru Ishiguro, Minoru Fukuchi, Youichi Kumagai, Keiichiro Ishibashi, Erito Mochiki, Takeo Iwama, Hidetaka Eguchi, Koji Okazaki, Shigehisa Inokuma, Hideyuki Ishida
• Journal: Gan to kagaku ryoho. Cancer & chemotherapy (Gan To Kagaku Ryoho) Vol. 44 Issue 12 Pg. 1449-1451 (Nov 2017) ISSN: 0385-0684 [Print] Japan
• PMID: 29394664 (Publication Type: Journal Article)

Topics

• Adenomatous Polyposis Coli (complications, surgery)
• Adolescent
• Adult
• Aged
• Antineoplastic Combined Chemotherapy Protocols (therapeutic use)
• Colectomy
• Female
• Fibromatosis, Aggressive (drug therapy, etiology, surgery)
• Humans
• Male
• Middle Aged
• Retrospective Studies
• Treatment Outcome
• Young Adult