ELECTRONIC IMAGE OF THE MONTH An Unusual Cause of Upper Gastrointestinal Bleeding Q2
Tricia Murdock,* Allen Lee,‡ and Rebecca Wilcox* *Department of Pathology and Laboratory Medicine and ‡Department of Medicine, Fletcher Allen Health Care, University of Vermont, Burlington, Vermont
web 4C/FPO
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52
36-year-old man was admitted after a single episode 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). Subsequent endoscopic ultrasound confirmed a 5.5-cm hyperechoic, submucosal mass (Figure B, 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 C, 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-Karim1 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 adults1,2 but is now known to
A
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.3,4 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.5 However, their immunohistochemical profiles are distinctly different. CFT does not express C-Kit, DOG-1, or CD34.4 Desmoids, inflammatory myofibroblastic tumors, and gastric schwannomas are also diagnostic considerations, but again, immunohistochemistry can be used to exclude these diagnoses.
References 1.
Rosenthal NS, Abdul-Karim FW. Childhood fibrous tumor with psammoma bodies: clinicopathologic features in two cases. Arch Pathol Lab Med 1988;112:798–800.
Clinical Gastroenterology and Hepatology 2014;-:-–SCO 5.2.0 DTD YJCGH53766_proof 15 May 2014 3:51 pm ce
53 54 55 56 57 58 59 60 61 62 63 64 65 66 67 68 69 70 71 72 73 74 75 76 77 78 79 80 81 82 83 84 85 86 87 88 89 90 91 92 93 94 95 96 97 98 99 100 101 102 103 104
ELECTRONIC IMAGE OF THE MONTH, continued 105 106 107 108 109 110 111 112 113 114 115 116 117 118 119 120 121 122 123 124 125 126 127 128 129 130 131 132 133 134 135 136 137 138 139 140 141 142 143 144 145 146 147 148 149 150 151 152 153 154 155 156 157 158 159 160
2.
Fetsch JF, Montgomery EA, Meis JM. Calcifying fibrous pseudotumor. Am J Surg Pathol 1993;17:502–508.
3.
Nascimento AF, Ruiz R, Hornick JL, et al. Calcifying fibrous “pseudotumor”: clinicopathologic study of 15 cases and analysis of its relationship to inflammatory myofibroblastic tumor. Int J Surg Pathol 2002;10:189–196.
4.
Agaimy A, Bihl MP, Tornillo L, et al. Calcifying fibrous tumor of the stomach: clinicopathologic and molecular study of seven cases with literature review and reappraisal of histogenesis. Am J Surg Pathol 2010;34:271–278.
5.
Miettinen M, Sobin LH, Lasota J. Gastrointestinal stromal tumors of the stomach: a clinicopathologic, immunohistochemical, and molecular genetic study of 1765 cases with long-term follow-up. Am J Surg Pathol 2005;29:52–68.
Conflicts of interest The authors disclose no conflicts. © 2014 by the AGA Institute 1542-3565/$36.00 http://dx.doi.org/10.1016/j.cgh.2014.03.023
e2 SCO 5.2.0 DTD YJCGH53766_proof 15 May 2014 3:51 pm ce
Q1
161 162 163 164 165 166 167 168 169 170 171 172 173 174 175 176 177 178 179 180 181 182 183 184 185 186 187 188 189 190 191 192 193 194 195 196 197 198 199 200 201 202 203 204 205 206 207 208 209 210 211 212 213 214 215 216