Clinicopathologic study of calcifying fibrous tumor of the gastrointestinal tract: a case series.
Calcifying fibrous tumor (CFT) is a rare benign mesenchymal lesion known to arise at multiple body sites that may clinically mimic other more aggressive lesions in the gastrointestinal (GI) tract. In this study we describe the clinicopathologic findings of 28 GI tract CFTs. Tumors predominantly arose in middle-aged adults with a slight female predominance. The most commonly involved sites were small bowel and colon, followed by stomach and appendix. Tumors ranged from 0.3 to 9.3 cm (median 1.4 cm), and submucosa was the most commonly involved layer. All tumors were well circumscribed and unencapsulated. Microscopically, tumors were hypocellular and composed of spindle cells with abundant, haphazardly arranged hyalinized collagen. No necrosis and less than one mitosis per 10 HPF were identified in all cases. Calcification was present in most (81%) of the cases. All cases had lymphoplasmacytic inflammatory infiltrates either scattered throughout the lesion with occasional perivascular conglomeration or in the form of lymphoid aggregates. A lymphoplasmacytic cuff was usually present (81%). Immunostains showed variable CD34 immunoreactivity and variable numbers of IgG4-positive plasma cells. The lesional cells were negative for DOG-1, ALK-1, S100, C-kit, Sox10, Melan A, HMB45, desmin, CK7, and CK20, and showed cytoplasmic staining for β-catenin. Follow-up information was available in 5 cases with no recurrences reported to date (mean follow-up, 3 years). CFT is a rare benign tumor that can occur in part of the GI tract and should be distinguished from other mesenchymal tumors due to its low risk of recurrence.
Department of Pathology, The Johns Hopkins University, School of Medicine, Baltimore, MD, 21231 USA. Electronic address: mkherad1@jhmi.edu.
Maryam Kherad
MK
Pezhouh
Department of Pathology, George Washington University, Washington, DC, 20037 USA.
M Katayoon
MK
Rezaei
Department of Pathology, The Johns Hopkins University, School of Medicine, Baltimore, MD, 21231 USA.
Maryam
M
Shabihkhani
Department of Pathology, The Johns Hopkins University, School of Medicine, Baltimore, MD, 21231 USA.
Arunima
A
Ghosh
Department of Pathology, The Johns Hopkins University, School of Medicine, Baltimore, MD, 21231 USA.
Deborah
D
Belchis
Department of Pathology, The Johns Hopkins University, School of Medicine, Baltimore, MD, 21231 USA.
Elizabeth A
EA
Montgomery
Department of Pathology, The Johns Hopkins University, School of Medicine, Baltimore, MD, 21231 USA.
Lysandra
L
Voltaggio
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D002114:Calcinosis / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D018218:Neoplasms, Fibrous Tissue / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D011237:Predictive Value of Tests; D013997:Time Factors; D016896:Treatment Outcome; D047368:Tumor Burden; D055815:Young Adult
D014408:Biomarkers, Tumor
Benign; Calcification; Calcifying fibrous tumor; Gastrointestinal tract; Hypocellular; Mesenchymal lesion