The Desmoid Tumor Research Foundation

April 2022

Surgical Management of a Giant Desmoid Fibromatosis of Abdominal Wall With Vessels Invasion in a Young Man: A Case Report and Review of the Literature

Author Information

Zhao J (1, 2), Cheng F (3, 4), Yao Z (5), Zheng B (1, 2), Niu Z (1, 2), He W (1, 2).

(1) Department of Urology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, China. (2) Department of Urology, Shandong Provincial Hospital Affiliated to Shandong University, Jinan, China. (3) Department of Nephrology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, China. (4) Department of Nephrology, Shandong Provincial Hospital Affiliated to Shandong University, Jinan, China. (5) Department of Pathology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, China.

Publication

Front Surg

Summary

Background: Desmoid fibromatosis (DF) is a rare clonal proliferation of fibroblasts and myofibroblasts. It develops in the connective tissues and does not metastasize but may infiltrate adjacent structures. Because of the rarity of these tumors and the unpredictable natural history of the disease, well-defined and precise guidelines of the optimal treatment for DF have not been formulated. Case presentation: Here, we present a giant abdominal DF that invaded the right spermatic cord and iliac vessels. The lesion was excised with external iliac artery dissection; however, the vein was sacrificed. The abdominal wall defect was then repaired with a polypropylene mesh. The lesional cells are positive for β-catenin. Conclusions: In the past decades, there has been a change in the treatment of DF. The "wait and see" policy has been considered initially in most cases. Surgical intervention remains a valid option for symptomatic lesions. The optimal regimes of the tumor should not take the risk of making the patient more symptomatic than the lesion itself.

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