Gastrointestinal stromal tumors presenting as omental masses--a clinicopathologic analysis of 95 cases.
Gastrointestinal stromal tumors (GISTs), generally KIT-positive and KIT/PDGFRA mutation-driven mesenchymal neoplasms, most commonly originate from the stomach or small intestine, but in rare examples they involve the omentum. In this study, we analyzed 95 GISTs surgically designated as the omental masses. These tumors occurred in 49 males and 46 females with a median age of 60 years (range: 27 to 88 y). They formed single (n=51) or multiple masses (n=39); 5 cases were equivocal in this respect. Of the single tumors, 21 had no evidence of gastrointestinal tract involvement, 25 were attached to stomach, and 3 were attached to small intestine. Clinicopathologic parameters and prognosis of the 2 former groups were similar. Single tumor cases showed a median mitotic count of 2/50 HPFs and median tumor size was 14 cm. Their histologic features were similar to gastric GISTs in 22 cases, and to small intestinal GISTs in 6 cases. These tumors were KIT positive 38/41, CD34 positive 20/33, 8 had PDGFRA mutations, and 6 had KIT exon 11 mutations. The median survival was 129 months (range: 0 to 397 mo) and 14 patients were alive at the end of follow-up. Multiple tumor cases showed median mitotic count of 14/50 HPFs and the main tumor median size was 16 cm. The histologic features were similar to small intestinal GISTs in 21 cases and to gastric GISTs in 7 cases; small intestinal attachment or history of a previous small intestinal GIST were noted in 5 cases, whereas no tumor was attached to stomach. The multiple GISTs were KIT positive 23/24, CD34 positive 7/21, and 5 had KIT exon 11 mutations, 3 had KIT exon 9 mutations, and 2 had PDGFRA mutations. The median survival was for 8 months and all patients died. Omental GISTs are clinicopathologically heterogenous. Patients with solitary tumors usually have gastric GIST-like morphology and a better prognosis than those with multiple tumors, whose tumor usually has small intestinal GIST-like histology. Omental GISTs unattached to gastrointestinal tract often resemble gastric GISTs suggesting that they may be gastric GISTs directly extending or parasitically attached into the omentum, whereas multiple omental GISTs more often resemble small intestinal GISTs suggesting that they may be metastatic or detached from this source. KIT positive Cajal cells were not found in normal omental tissues failing to support the presence of these ancestral cells for GIST in the omentum.
Miettinen
Markku
M
Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, 6825 16th Street, N.W., Building 54, Rm. G090, Washington, DC 20306-6000, USA. miettinen@afip.osd.mil
Sobin
Leslie H
LH
Lasota
Jerzy
J
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018952:Antigens, CD34 / Q000378:metabolism; D014408:Biomarkers, Tumor / Q000378:metabolism; D004273:DNA, Neoplasm / Q000032:analysis; D005260:Female; D046152:Gastrointestinal Stromal Tumors / Q000235:genetics / Q000378:metabolism / Q000401:mortality / Q000473:pathology*; D006801:Humans; D007414:Intestinal Neoplasms / Q000473:pathology; D007421:Intestine, Small / Q000473:pathology; D008297:Male; D008637:Mesenchymoma / Q000235:genetics / Q000378:metabolism / Q000401:mortality / Q000473:pathology*; D008875:Middle Aged; D009154:Mutation; D009378:Neoplasms, Multiple Primary; D009852:Omentum / Q000378:metabolism / Q000473:pathology*; D010534:Peritoneal Neoplasms / Q000235:genetics / Q000378:metabolism / Q000401:mortality / Q000473:pathology*; D011379:Prognosis; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics / Q000378:metabolism; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000235:genetics; D013274:Stomach Neoplasms / Q000473:pathology; D015996:Survival Rate
D018952:Antigens, CD34; D014408:Biomarkers, Tumor; D004273:DNA, Neoplasm; D019009:Proto-Oncogene Proteins c-kit; D020796:Receptor, Platelet-Derived Growth Factor alpha