An Unusual Cause of Upper Gastrointestinal Bleeding

Abstract

36-year-old man was admitted after a single Aepisode of hematemesis and 2-day history of black tarry stools, dizziness, and sweats. He was afebrile and denied abdominal pain. Laboratory results were significant only for mild anemia (hemoglobin, 12.3 gm/dL). Coagulation laboratory results were normal. The patient underwent esophagogastroduodenoscopy where a large, broad-based mass with mucosal ulceration was identified in the gastric body (Figure A, B). Subsequent endoscopic ultrasound confirmed a 5.5-cm hyperechoic, submucosal mass (Figure C, arrow). Endoscopic biopsies and ultrasound-guided fine-needle aspiration were inconclusive, consisting solely of bland spindle cells and ulcer bed. The patient opted for removal of the mass through laparoscopic partial gastrectomy. The surgical pathology diagnosis of the resection specimen was gastric calcifying fibrous tumor (Figure D, H&E). Immunohistochemical staining, specifically S-100, CD34, DOG-1, C-Kit, desmin, and ALK, were all negative. Calcifying fibrous tumor (CFT) is a rare, benign mesenchymal tumor. It was originally described by Rosenthal and Abdul-Karim in 1988 as childhood fibrous tumor with psammoma bodies because the tumor is composed of dense, eosinophilic, hyalinized fibrous tissue admixed with bland fibroblastic spindle cells, scattered psammomatous and/or dystrophic calcifications, and a variable mononuclear lymphocytic infiltrate. CFT was originally described in soft tissues of children and young adults but is now known to have a wide anatomic distribution. It is rare in the gastrointestinal tract, with only 13 gastric and 3 intestinal cases documented to date. Within the gastric cases including our own, there were 8 men and 6 women with a mean age of 52 years and mean tumor size of 2.1 cm. On ultrasound, the lesion tends to be isoechoic, with hyperechoic foci and acoustic shadowing corresponding to the calcifications. Although this is not entirely clear, CFTs are thought to be a reactive process caused by abnormal tissue repair. However, because local recurrences of CFT are documented, this lesion is best classified as a CFT rather than its former designation of a pseudotumor. Recurrence has not been documented in the largest gastric case series to date. CFT has morphologic overlap with other spindle cell tumors, namely gastrointestinal stromal tumor. Both can arise in the posterior stomach wall with varying involvement of the muscularis propria. However, their immunohistochemical profiles are distinctly different. CFT does not express C-Kit, DOG-1, or CD34. Desmoids, inflammatory myofibroblastic tumors, and gastric schwannomas are also diagnostic considerations, but again, immunohistochemistry can be used to exclude these diagnoses.