Retrospective Study
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Oct 16, 2021; 9(29): 8710-8717
Published online Oct 16, 2021. doi: 10.12998/wjcc.v9.i29.8710
Clinical and imaging features of desmoid tumors of the extremities
Zhuo Shi, Xin-Ming Zhao, Jiu-Ming Jiang, Meng Li, Li-Zhi Xie
Zhuo Shi, Xin-Ming Zhao, Jiu-Ming Jiang, Meng Li, Department of Imaging Diagnosis, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China
Li-Zhi Xie, GE Healthcare China, Beijing 100176, China
Author contributions: Shi Z and Zhao XM designed this retrospective study, Shi Z, Zhao XM, and Jiang JM wrote this paper; Shi Z, Zhao XM, Jiang JM, Li M, and Xie LZ were responsible for sorting the data.
Institutional review board statement: The study was reviewed and approved by the Cancer Hospital of Peking Union Medical College Hospital, Chinese Academy of Medical Sciences Institutional Review Board (Approval No. 20/120-2316).
Informed consent statement: Patients were not required to give informed consent to the study because the analysis used anonymous clinical data that were obtained after each patient agreed to treatment by written consent.
Conflict-of-interest statement: None.
Data sharing statement: No additional data are available.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Xin-Ming Zhao, MD, Chief Physician, Department of Imaging Diagnosis, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College. No. 17 Panjiayuan Nanli, Chaoyang District, Beijing 100021, China. xinmingzh@sina.com
Received: June 17, 2021
Peer-review started: June 17, 2021
First decision: July 5, 2021
Revised: July 14, 2021
Accepted: August 13, 2021
Article in press: August 13, 2021
Published online: October 16, 2021
Processing time: 120 Days and 7.9 Hours
Abstract
BACKGROUND

Desmoid fibroma is a rare soft tissue tumor originating from the aponeurosis, fascia, and muscle, and it is also known as aponeurotic fibroma, invasive fibroma, or ligamentous fibroma.

AIM

To investigate the clinical and imaging features of desmoid tumors of the extremities.

METHODS

Thirteen patients with desmoid fibroma of the extremities admitted to our hospital from October 2016 to March 2021 were included. All patients underwent computed tomography (CT), magnetic resonance imaging (MRI), and pathological examination of the lesion. Data on the diameter and distribution of the lesion, the relationship between the lesion morphology and surrounding structures, MRI and CT findings, and pathological features were statistically analyzed.

RESULTS

The lesion diameter ranged from 1.7 to 8.9 cm, with an average of 5.35 ± 2.39 cm. All lesions were located in the deep muscular space, with the left and right forearm each accounting for 23.08% of cases. Among the 13 patients with desmoid fibroma of the extremities, the lesions were "patchy" in 1 case, irregular in 10, and quasi-round in 2. The boundary between the lesion and surrounding soft tissue was blurred in 10 cases, and the focus infiltrated along the tissue space and invaded the adjacent structures. Furthermore, the edge of the lesion showed "beard-like" infiltration in 2 cases; bone resorption and damage were found in 8, and bending of the bone was present in 2; the boundary of the focus was clear in 1. According to the MRI examination, the lesions were larger than 5 cm (61.54%), round or fusiform in shape (84.62%), had an unclear boundary (76.92%), showed uniform signal (69.23%), inhomogeneous enhancement (84.62%), and "root" or "claw" infiltration (69.23%). Neurovascular tract invasion was present in 30.77% of cases. CT examination showed that the desmoid tumors had slightly a lower density (69.23%), higher enhancement (61.54%), and unclear boundary (84.62%); a CT value < 50 Hu was present in 53.85% of lesions, and the enhancement was uneven in 53.85% of cases. Microscopically, fibroblasts and myofibroblasts were arranged in strands and bundles, without obvious atypia but with occasional karyotyping; cells were surrounded by collagen tissue. There were disparities in the proportion of collagen tissue in different regions, with abundant collagen tissue and few tumor cells in some areas, similar to the structure of aponeuroses or ligaments, and tumor cells invading the surrounding tissues.

CONCLUSION

Desmoid tumors of the extremities have certain imaging features on CT and MRI. The two imaging techniques can be combined to improve the diagnostic accuracy, achieve a comprehensive diagnosis of the disease in the clinical practice, and reduce the risk of missed diagnosis or misdiagnosis. In addition, their use can ensure timely diagnosis and treatment.

Keywords: Soft tissue desmoid tumor of the extremities; Clinical features; Imaging examination; Computed tomography; Magnetic resonance imaging

Core Tip: Generally, soft tissue desmoid tumors of the extremities show certain imaging features on computed tomography and magnetic resonance imaging. These two imaging techniques can be combined to comprehensively diagnose the disease, improve the diagnostic accuracy, reduce the risk of missed diagnosis or misdiagnosis, and ensure that patients are diagnosed and treated as soon as possible.