Clinicopathologic study of calcifying fibrous tumor of the gastrointestinal tract: a case series

Maryam Kherad Pezhouh, M. Katayoon Rezaei, Maryam Shabihkhani, Arunima Ghosh, Deborah A Belchis, Elizabeth A Montgomery, Lysandra Voltaggio

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)199-205
Number of pages7
JournalHuman Pathology
Volume62
DOIs
StatePublished - Apr 1 2017

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Gastrointestinal Tract
Neoplasms
MART-1 Antigen
Recurrence
Catenins
Desmin
Appendix
Plasma Cells
Mitosis
Stomach
Colon
Necrosis
Collagen
Immunoglobulin G
Staining and Labeling

Keywords

  • Benign
  • Calcification
  • Calcifying fibrous tumor
  • Gastrointestinal tract
  • Hypocellular
  • Mesenchymal lesion

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Clinicopathologic study of calcifying fibrous tumor of the gastrointestinal tract : a case series. / Pezhouh, Maryam Kherad; Rezaei, M. Katayoon; Shabihkhani, Maryam; Ghosh, Arunima; Belchis, Deborah A; Montgomery, Elizabeth A; Voltaggio, Lysandra.

In: Human Pathology, Vol. 62, 01.04.2017, p. 199-205.

Research output: Contribution to journalArticle

Pezhouh, Maryam Kherad ; Rezaei, M. Katayoon ; Shabihkhani, Maryam ; Ghosh, Arunima ; Belchis, Deborah A ; Montgomery, Elizabeth A ; Voltaggio, Lysandra. / Clinicopathologic study of calcifying fibrous tumor of the gastrointestinal tract : a case series. In: Human Pathology. 2017 ; Vol. 62. pp. 199-205.
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abstract = "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.",
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