Gastric Calcifying Fibrous Tumor: An Easy Misdiagnosis as Gastrointestinal Stromal Tumor—A Systemic Review

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Abstract

Background and Objectives: Calcifying fibrous tumor (CFT) in the stomach is extremely rare and is easily misdiagnosed as a gastrointestinal stromal tumor (GIST). This study aims to determine the best method to differentiate between gastric CFT and GIST after a systemic review and meta-analysis. Materials and Methods: A systematic search of articles using electronic databases (MEDLINE, EMBASE, and LILACS) was conducted and resulted in 162 articles with 272 CFT cases published from January 1988 to September 2019. Results: Of these cases, 272 patients, 60 patients with gastric CFT (32 men and 28 women, mean age 49.2 years) were analyzed. The mean tumor size was 2.4 cm in patients with gastric CFT. Both endoscopic ultrasound (EUS) and computed tomography (CT) findings revealed well-defined (100% vs. 77.8%), heterogeneous (100% vs. 77.8%), iso-hypoechoic (71.4% vs. 33.3%), and calcified (85.7% vs. 77.8%) lesions, respectively. The majority of patients (53.3%) were symptomatic, with the most common symptom being abdominal discomfort (55.6%). None of the patients with gastric CFT showed recurrence after treatment, and most patients received nonendoscopic treatment (56%, n = 28/50). Both age and tumor size were statistically significant in patients with gastric CFT than GIST (49.2 vs. 65.0 years and 2.4 vs. 6.0 cm; both p < 0.001). The ratio of children among patients with CFT (5%) and GIST (0.05%) was also significantly different ( p = 0.037). The calcification rates of gastric CFT had significantly higher calcification rates than GIST on images of EUS and CT (85.7% vs. 3.6% and 77.8% vs. 3.6%; both p < 0.001). Conclusions: Compared with patients with GIST, patients with gastric CFT were younger, had smaller tumor size, and were symptomatic. Furthermore, gastric CFT was well-defined, heterogeneous in the third layer, and had high calcification rates on the images.

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Gastrointestinal stromal tumors--definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis.

M Miettinen, J. Lasota (2001)

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal (GI) tract. They are defined here as KIT (CD117, stem cell factor receptor)-positive mesenchymal spindle cell or epithelioid neoplasms primary in the GI tract, omentum, and mesentery. GISTs typically present in older individuals and are most common in the stomach (60-70%), followed by small intestine (20-25%), colon and rectum (5%), and esophagus (<5%). Benign tumors outnumber the malignant ones by a wide margin. Approximately 70% of GISTs are positive for CD34, 20-30% are positive for smooth muscle actin (SMA), 10% are positive for S100 protein and <5% are positive for desmin. The expression of CD34 and SMA is often reciprocal. GISTs commonly have activating mutations in exon 11 (or rarely exon 9 and exon 13) of the KIT gene that encodes a tyrosine kinase receptor for the growth factor named stem cell factor or mast cell growth factor. Ligand-independent activation of KIT appears to be a strong candidate for molecular pathogenesis of GISTs, and it may be a target for future treatment for such tumors. Other genetic changes in GISTs discovered using comparative genomic hybridization include losses in 14q and 22q in both benign and malignant GISTs and occurrence in various gains predominantly in malignant GISTs. GISTs have phenotypic similarities with the interstitial cells of Cajal and, therefore, a histogenetic origin from these cells has been suggested. An alternative possibility, origin of pluripotential stem cells, is also possible; this is supported by the same origin of Cajal cells and smooth muscle and by the common SMA expression in GISTs. GISTs differ clinically and pathogenetically from true leiomyosarcomas (very rare in the GI tract) and leiomyomas. The latter occur in the GI tract, predominantly in the esophagus (intramural tumors) and the colon and rectum (muscularis mucosae tumors). They also differ from schwannomas that are benign S100-positive spindle cell tumors usually presenting in the stomach. GI autonomic nerve tumors (GANTs) are probably a subset of GIST. Other mesenchymal tumors that have to be separated from GISTs include inflammatory myofibroblastic tumors in children, desmoid, and dedifferentiated liposarcoma. Angiosarcomas and metastatic melanomas, both of which are often KIT-positive, should not be confused with GISTs.

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Gastrointestinal stromal tumours: ESMO–EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up†

P G Casali, N Abecassis, S. Bauer … (2018)

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Gastrointestinal Stromal Tumors.

Margaret von Mehren, Heikki Joensuu (2018)

GI stromal tumors (GISTs) are neoplasms with a varying malignancy potential ranging from virtually indolent tumors to rapidly progressing cancers. GISTs occur throughout the intestinal tract, and most harbor an activating mutation in either KIT or platelet-derived growth factor A ( PDGFRA). Diagnosis is made using immunohistochemistry, but molecular testing with mutation analysis is paramount for selection of appropriate therapy. Most small GISTs are cured with surgery. Tyrosine kinase inhibitor (TKI) therapy has led to substantial improvements in survival, both for patients with localized GIST and those with advanced disease. Adjuvant therapy with imatinib benefits patients with a high risk of recurrence, with studies suggesting most benefit with at least 3 years of therapy. Neoadjuvant imatinib therapy should be considered for patients requiring extensive surgery, aiming at shrinking the tumor to allow organ preservation and less extensive surgery. The following three TKIs have been approved for the management of advanced disease: imatinib, sunitinib, and regorafenib; imatinib is usually the best tolerated of the three and the standard first-line treatment. TKIs benefit the majority of patients with advanced GIST but have no or limited efficacy in patients with the PDGFRA D842V mutation or patients with GIST lacking KIT and PDGFRA mutations. Surgery, the mainstay of primary tumor management, also plays a role in the advanced disease setting for selected patients, as do some other approaches such as palliative radiation therapy. Research continues to identify novel therapies, in particular effective agents to treat TKI-refractory disease.

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Journal ID (nlm-ta): Medicina (Kaunas)

Journal ID (publisher-id): medicina

Title: Medicina

Publisher: MDPI

ISSN (Print): 1010-660X

ISSN (Electronic): 1648-9144

Publication date (Electronic): 14 October 2020

Publication date Collection: October 2020

Volume: 56

Issue: 10

Electronic Location Identifier: 541

Affiliations

[1 ]Division of Rheumatology, Department of Internal Medicine, Taichung Armed Forces General Hospital, Taichung 41152, Taiwan; raymond42770@ 123456gmail.com

[2 ]Division of Rheumatology, Department of Internal Medicine, National Defense Medical Center, Taipei 11490, Taiwan

[3 ]Department of Pharmacy, China Medical University, Taichung 40402, Taiwan; hungyi@ 123456mail.cmu.edu.tw

[4 ]Department of Pharmacy, China Medical University Beigang Hospital, Yunlin County 65152, Taiwan

[5 ]Division of Pulmonary Medicine, Department of Internal Medicine, Chung Shan Medical University Hospital, Taichung 40201, Taiwan; cshy1292@ 123456csh.org.tw

[6 ]School of Medicine, Chung Shan Medical University, Taichung 40201, Taiwan

[7 ]Department of Radiology, Taichung Armed Forces General Hospital, Taichung 41168, Taiwan; lylmed066@ 123456gmail.com

[8 ]Department of Internal Medicine, Chung Shan Medical University Hospital, Taichung 40201, Taiwan

[9 ]Department of Internal Medicine, Chung Shan Medical University, Taichung 40201, Taiwan

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[* ]Correspondence: cgp8009@ 123456yahoo.com.tw ; Tel.: +886-933450449

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Meng-Ko Tsai https://orcid.org/0000-0003-2519-0501

Gwo-Ping Jong https://orcid.org/0000-0002-7786-5497

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Publisher ID: medicina-56-00541

DOI: 10.3390/medicina56100541

PMC ID: 7602413

PubMed ID: 33066662

SO-VID: 0f1e526b-710e-4bce-9c78-d05ce5c57536

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Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license ( http://creativecommons.org/licenses/by/4.0/).

Gastric Calcifying Fibrous Tumor: An Easy Misdiagnosis as Gastrointestinal Stromal Tumor—A Systemic Review

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Most cited references 46

Gastrointestinal stromal tumors--definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis.

Gastrointestinal stromal tumours: ESMO–EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up†

Gastrointestinal Stromal Tumors.

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