The Desmoid Tumor Research Foundation

December 2019

Clinicopathological and molecular characteristics of abdominal desmoid tumors in the Chinese population: A single-center report of 15 cases

Author Information

Wang J(1), Jia N(2), Lin Q(1), Huang Y(3), Li J(1), Jiang Q(1), Liu W(1), Xu J(1), Hou Y(4), Liu J(4), Li M(5), Lu W(1), Zhou Y(6), Zhang Y(1), Tong H(1).

(1)Dept of General Surg, Zhongshan Hosp, Fudan Univ, Shanghai, P.R. CHN. (2)Laboratory of Molecular Biology, Dept of Biochemistry, An Hui Medical Univ, Hefei, Anhui, P.R. CHN. (3)Endoscopy Ctr, Zhongshan Hosp, Fudan Univ, Shanghai, P.R. CHN. (4)Dept of Pathology, Zhongshan Hosp, Fudan Univ, Shanghai, P.R. CHN. (5)Dept of Dermatology, Xinhua Hosp, Shanghai Jiaotong Univ School of Medicine, Shanghai, P.R. CHN. (6)Dept of Oncology, Zhongshan Hosp, Fudan Univ, Shanghai, P.R. CHN.

Publication

Oncol Lett

Summary

The aim of the present study was to investigate the clinicopathological and molecular characteristics of abdominal DT in a Chinese population and to provide clues for selecting the optimal treatment strategy for different types of abdominal desmoid tumors (DT). The clinicopathological data of 15 consecutive patients with DT was collected. A review of the patient clinicopathological characteristics revealed that FAP-associated DT exhibited a higher rate of abdominal surgery history (P=0.011), with no significant differences in any other characteristics. Sequencing revealed that mutations in the APC, CTNNB1 and MUTYH genes were common in DT, and only one patient harbored no mutations in these genes. Survival analyses revealed that patients with FAP exhibited shorter time to progress TTP (P=0.030). Log-rank test demonstrated a tendency towards shorter TTP in the cases where an R2 resection was performed (P=0.072) and a tendency towards poor prognosis in the cases of DT associated with FAP (P=0.087). In conclusion, Abdominal DTs were prone to occur in patients with FAP with a history of abdominal surgery. Mutations in the APC, CTNNB1 and MUTYH genes were detected in patients with DTs. To the best of our knowledge, this is the first study of abdominal DT occurrence in patients with MUTYH-associated FAP. The prognosis of DT associated with FAP may be worse compared with that of sporadic DT.

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