Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Oct 7, 2020; 26(37): 5597-5605
Published online Oct 7, 2020. doi: 10.3748/wjg.v26.i37.5597
Calcifying fibrous tumor of the gastrointestinal tract: A clinicopathologic review and update
Donald Turbiville, Xuchen Zhang
Donald Turbiville, Xuchen Zhang, Department of Pathology, Yale University School of Medicine, New Haven, CT 06510, United States
Author contributions: Turbiville D drafted the paper; Zhang X edited, revised and contributed with conceptual development.
Conflict-of-interest statement: The authors have no conflicts of interest to disclose.
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:
Corresponding author: Xuchen Zhang, MD, PhD, Department of Pathology, Yale University School of Medicine, 310 Cedar Street, PO Box 208023, New Haven, CT 06510, United States.
Received: July 6, 2020
Peer-review started: July 6, 2020
First decision: July 28, 2020
Revised: August 9, 2020
Accepted: September 12, 2020
Article in press: September 12, 2020
Published online: October 7, 2020

Calcifying fibrous tumor (CFT) is a rare mesenchymal lesion that has been documented throughout the gastrointestinal tract. Gastrointestinal CFTs may occur at virtually any age, with a predilection for adults and for females. They occur most commonly in the stomach and the small and large intestines. CFTs are most often found incidentally, cured by local resection, and have a low risk of recurrence. Histology shows three characteristic features: Spindle cell proliferations within a densely hyalinized stroma, scattered calcifications, and lymphoplasmacytic inflammation. CFTs are immunoreactive for CD34, vimentin and factor XIIIa, helping to distinguish them from other benign mesenchymal neoplasms. The differential diagnosis of CFTs includes sclerosing gastrointestinal stromal tumor, leiomyoma, schwannoma, solitary fibrous tumor, inflammatory myofibroblastic tumor, plexiform fibromyxoma, fibromatosis, sclerosing mesenteritis, and reactive nodular fibrous pseudotumor. The pathogenesis of CFTs remains unclear, but some have hypothesized that they may be linked to IgG4-related disease, inflammatory myofibroblastic lesions, hyaline vascular type Castleman disease, sclerosing angiomatoid nodular transformation of the spleen, or trauma.

Keywords: Calcifying fibrous tumor, Calcifying fibrous pseudotumor, Gastrointestinal tract, Mesenchymal lesion, Calcification, Pathology

Core Tip: Calcifying fibrous tumors (CFTs) are rare benign mesenchymal tumors of the gastrointestinal tract. Originally thought to be tumors of soft tissue sites, recent studies have shown the gastrointestinal tract to be a primary site for these tumors. CFTs present a diagnostic challenge due to histologic features that overlap with numerous stromal lesions. Understanding of the core clinical, histologic and immunophenotypic features of CFTs is important in making an accurate diagnosis. In this review we summarize and update the clinical and pathologic features of CFT as well as the differential diagnosis for this entity.