Gastrointestinal (GI) stromal tumors (GISTs), the specific KIT- or PDFGRA-signaling driven mesenchymal tumors, are the most common mesenchymal tumors of the GI tract. This study analyzed 1091 tumors originally classified as smooth muscle tumors of the small intestine (including jejunum or ileum and excluding duodenum), and found that 906 (83%) of these were GISTs. The GIST patients had 55:45 male-to-female ratio with a median age of 59 years (range, 13-94 years). Only 0.6% of tumors occurred before the age of 21 years and 13.6% before the age of 40 years. The tumors varied from 0.3 to 40 cm (median, 7.0 cm) and most commonly presented with GI bleeding or acute abdomen; 18% were incidentally detected. Histologically, the tumors were relatively monotypic with spindle cell (86%), epithelioid (5%), or mixed patterns (9%). Skeinoid fibers were present in 44% of cases, and their presence was associated with a favorable course. Most epithelioid tumors were malignant, and this morphology sometimes emerged from less cellular and less mitotically active spindle cell tumors, suggesting that it represented a transformation. KIT was immunohistochemically detected in 98%, CD34 in 40%, smooth muscle actin in 34%, desmin in 0.2%, and S-100 protein in 14% of the tumors tested. Outcome was strongly dependent on tumor size and mitotic activity, with an overall 39% tumor-related mortality, twice that for gastric GISTs. Only 10 cm and >5 mitoses/50 HPF metastasized. In stark contrast to corresponding gastric tumors, tumors >10 cm with mitotic activity 5/50 HPF had a high metastatic rate (>50%); tumors >5 cm < or = 10 cm with low mitotic rate had a 24% metastatic rate. The median survival times of patients with low mitotic rate tumors who died of disease decreased by increasing tumor size. KIT exon 11 mutations were detected in 90 cases, exon 9 mutation in 17 cases, and exon 17 mutation in 1 case; the presence of mutation or mutation type was not prognostically significant. There were no PDGFRA exon 12 or 8 mutations. Systematic data on prognosis of small intestinal GISTs of various size and mitotic activity categories can be helpful in management and surveillance of patients with these tumors.

Gastrointestinal stromal tumors of the jejunum and ileum: a clinicopathologic, immunohistochemical, and molecular genetic study of 906 cases before imatinib with long-term follow-up.

Papillary cystic and solid tumors of the pancreas: A pancreatic embryonic tumor? Studies of three cases and cumulative review of the world's literature

Pancreatic cystic neoplasms.

Solid and papillary neoplasm of the pancreas: radiological-pathological study of five cases and review of the literature.

Cystic pancreatic lesions vary from benign to malignant entities and are increasingly detected on cross-sectional imaging. Knowledge of the imaging appearances of cystic pancreatic lesions may help radiologists in their diagnostic reporting and management. In this review, we discuss the morphologic classification of these lesions based on a diagnostic algorithm as well as the management of these lesions.

Cystic pancreatic lesions: From increased diagnosis rate to new dilemmas.

Solid and papillary epithelial neoplasms of the pancreas are uncommon. These grow to a large size, are often palpable, and occur most often in young black women. The tumors have a characteristic histologic appearance and a low malignant potential. Heavy tumor calcification is an unusual finding. Two cases with radiologie-pathologic correlation are presented herein.

Solid and papillary epithelial pancreatic neoplasm: an unusual tumor.

Two cases of ileal leiomyomatous neoplasms with positive scintigraphic findings for Meckel's diverticulum are presented. The precise mechanism for the abnormal concentration of the Tc-99m pertechnetate is uncertain. Illustrations of the collating barium and computerized tomographic studies are included and the scanning technique utilized is reviewed.

Ileal leiomyosarcoma and leiomyoma. False-positive scintiscans for Meckel's diverticulum.

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Solid and papillary epithelial pancreatic neoplasm: an unusual tumor.

Ileal leiomyosarcoma and leiomyoma. False-positive scintiscans for Meckel's diverticulum.