Sun Hee Chang

Primary gastrointestinal clear cell sarcoma: report of 2 cases, one case associated with IgG4-related sclerosing disease, and review of literature

Clear cell sarcoma (CCS) is a distinctive soft tissue sarcoma that shows melanocytic differentiation. Primary gastrointestinal (GI) CCSs have been rarely reported, but to our knowledge, no association between GI CCSs and immunoglobulin G4 (IgG4)-related sclerosing disease has been described in the literature. We experienced 2 cases of CCS that arose in the small intestine and metastasized to the liver. Histologic features and immunophenotype were typical of CCS. One of them showed a unique peritumoral sclerosing inflammatory reaction, which was highly reminiscent of IgG4-related sclerosing inflammatory disease. Dense lymphoplasmacytic infiltration with extensive sclerosis and obliterative phlebitis was observed in the immediate vicinity of the primary and metastatic tumors, but not in the distant areas from the tumor. The average number of IgG4-positive plasma cells was more than 50 per high-power field. We report 2 cases of primary GI CCS with one case showing a unique peritumoral IgG4-related lymphoplasmacytic sclerosing inflammation.

Helicobacter heilmannii-associated Gastritis: Clinicopathologic Findings and Comparison with Helicobacter pylori-associated Gastritis

The aims of this study were to evaluate the clinicopathologic features of Helicobacter heilmannii-associated gastritis and to compare H. heilmannii-associated gastritis with H. pylori-associated gastritis. We reviewed 5,985 consecutive gastric biopsy specimens. All cases of chronic gastritis with Helicobacter infection were evaluated with the Updated Sydney System, and the grades of all gastritis variables were compared between H. heilmannii-associated gastritis and H. pylori-associated gastritis groups. There were 10 cases of H. heilmannii-associated gastritis (0.17%) and 3,285 cases of H. pylori-associated gastritis (54.9%). The organisms were superficially located within the mucous layer without adhesion to epithelial cells. Interestingly, in one case many intracytoplasmic H. heilmannii organisms were observed in parietal cells with cell damage. A case of low-grade mucosa-associated lymphoid tissue (MALT) lymphoma concomitant with H. heilmannii infection was detected. Compared to H. pylori-associated gastritis, H. heilmannii-associated gastritis showed less severe neutrophilic activity (p<0.0001), mononuclear cell infiltration (p=0.0029), and endoscopic findings of chronic gastritis devoid of erosion or ulcer (p=0.0309). In conclusion, we present the detailed clinicopathologic findings of H. heilmannii-associated gastritis compared to H. pylori-associated gastritis. H. heilmannii-associated gastritis is uncommon and milder than H. pylori-associated gastritis, however it may be noteworthy with respect to the development of MALT lymphoma.

IgG4-related sclerosing esophagitis: a case report

decided to initially use an OVESCO (over-the-scope clip) with an 11-mm diameter that partially occluded the fistula and worked as an anchor point for the posterior placement of a self-expandable covered metal stent (20-mm diameter and 8-cm length) (Figs. 2-4). At the end of the procedure, there was a good flow of contrast to the stomach, with no evidence of the fistulous tract (Figs. 5 and 6). At the 48-hour evaluation, there was proper positioning of the clip and stent and no evidence Figure 5. Stent and endoclip in situ.

Nodular lymphoid hyperplasia and histologic changes mimicking celiac disease, collagenous sprue, and lymphocytic colitis in a patient with selective IgA deficiency

Selective IgA deficiency is the most common primary immunoglobulin deficiency. The clinical manifestations of selective IgA deficiency, including gastrointestinal (GI) complications, are rare and typically milder than those seen with common variable immunodeficiency or X-linked agammaglobulinemia. We present a rare case of selective IgA deficiency that shows a number of interesting histological features in the GI tract, including diffuse nodular lymphoid hyperplasia involving the entire small and large intestine, celiac disease-like and collagenous sprue-like changes in the small intestine, as well as lymphocytic colitis pattern. However, this patient had no particular GI symptoms suggestive of celiac sprue or microscopic colitis. These findings suggest that the GI tract in patients with selective IgA deficiency can show peculiar histologic changes that mimic celiac disease, collagenous sprue, or lymphocytic colitis, which may be a pattern of injury related to infection or immunoglobulin immunodeficiency-associated autoimmune phenomena.

Idiopathic segmental ureteritis, misdiagnosed as ureteral cancer preoperatively : A case report with literature review

A 53‐year‐old man presented with right flank pain for 6 days. Computerized tomography revealed a 3 cm long segment of ureteral narrowing with wall thickening and hydronephrosis, suspicious for ureteral cancer. Under the clinical diagnosis of ureteral carcinoma a right nephroureterectomy was performed. The wall of the distal ureter, 2.5 cm from the bladder cuff, had a luminal‐narrowing, firm mass‐forming lesion with abrupt transition from the adjacent ureter. Histologically, the resected ureteral mass showed transmural fibrosing, chronic inflammation with numerous plasma cells, epithelioid granulomas, and obliterative phlebitis. Histological findings were consistent with idiopathic segmental ureteritis (ISU) with differential diagnoses of IgG4‐related sclerosing disease, including lymphoplasmacytic inflammatory pseudotumor (IPT) and idiopathic retroperitoneal fibrosis. IgG4 immunostaining in this case was barely positive, excluding the possibility of IgG4‐related IPT. Although the majority of luminal obliterated segmental lesions of the ureter are neoplastic in nature, non‐neoplastic inflammatory processes as seen in this case may occur in the ureter, causing diagnostic confusion with true neoplasms. Herein we report a rare case of ISU that was clinically misdiagnosed as malignancy preoperatively. ISU of the current case may be an IgG4‐unrelated subtype of IPT.

Xanthogranulomatous pancreatitis presents as a solid tumor mass: a case report.

Xanthogranulomatous inflammation (XGI) is a rare, idiopathic process in which lipid-laden histiocytes are deposited at various locations in the body. Although XGI has been reported to occur in various organs such as the gallbladder, kidney, bone, stomach, colon, appendix, lymph nodes, urachus, and urinary bladder and in soft tissues, xanthogranulomatous pancreatitis (XGP) is extremely rare. Herein, we report a case of XGP occurring in a 70-yr-old woman, who presented with abdominal pain for several months. On physical examination, mild epigastric tenderness was noted. Abdomen CT scan revealed a low attenuated mass in uncinate process of pancreas, suggesting malignant lesion. Whipples operation was performed and the final pathologic diagnosis was XGP. The patients post-operative course was uneventful, and no recurrence was found within 7 months of the operation. When a pancreatic mass does not show clinico-radiological features typical of common pancreatic neoplasms, XGP should be considered for a differential diagnosis.

Cytogenetic and molecular genetic study on granular cell glioblastoma: a case report.

Granular cell astrocytoma is a rare infiltrative malignant glioma with prominent granular cell change. Granular cell astrocytomas are biologically aggressive compared with conventional infiltrating astrocytomas of similar grades, but their genetic alterations are poorly known. We report a case of granular cell glioblastoma and its genetic and molecular features. Histologically, the tumor not only showed features typical of granular cell astrocytoma but also demonstrated frequent mitoses, pseudopalisading necrosis, and vascular endothelial hyperplasia, compatible with glioblastoma. Array-based comparative genomic hybridization and focused molecular genetic analyses demonstrated gain of chromosome 7; losses of chromosome 1p, 8p, 9p, 10, 13q, and 22q; amplification of epidermal growth factor receptor; and homozygous deletion of CDKN2A as well as MGMT promoter methylation. However, neither isocitrate dehydrogenase 1 mutation nor codeletion of 1p/19q was found. Our results indicate that granular cell glioblastomas, despite having its peculiar granular cell changes, share common molecular genetic features with conventional glioblastoma, especially the classical subtype.

Duodenal Somatostatinoma: A Case Report and Review

Somatostatinomas are rare functioning carcinoid tumors that usually arise in the pancreas and duodenum. They are seldom associated with typical clinical symptoms; their diagnosis is confirmed only by histological and immunohistochemical studies and the presence of specific hormones. Two distinct clinicopathological forms of somatostatinoma exist: duodenal and pancreatic somatostatinomas. Clinically, compared to pancreatic somatostatinomas, duodenal somatostatinomas are more often associated with nonspecific symptoms and neurofibromatosis, but less often with somatostatinoma syndrome or metastasis. Histologically, duodenal somatostatinomas frequently have psammoma bodies in the tumor cells. We report a case of duodenal somatostatinoma in 58-year-old man with vague epigastric pain and nausea. He did not have diabetes, steatorrhea, or cholelithiasis. Abdominal computed tomography showed a 25-mm mass in the duodenum and 25-mm nodule in the liver. Endoscopic retrograde cholangiopancreatography showed a duodenal submucosal tumor. Although the endoscopic biopsies were free of malignancy, the patient subsequently underwent Whipples operation for the duodenal mass. Examination revealed as a somatostatinoma using a special stain for somatostatin.

WITHDRAWN: Cytogenetic and molecular genetic study on glioblastoma arising in granular cell astrocytoma: a case report.

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A 69‐Year‐Old Woman with an Epidural Mass

A 69-year-old woman presented with a 2-month history of progressive bilateral leg weakness and gait difficulty. On examination, the patient had diffuse lower extremity weakness and hypesthesia of the left leg. Magnetic resonance imaging revealed a 2.9-cm, extradural, soft tissue mass at the T5–6 level, with spinal cord compression; the mass was hypointense on a T1-weighted image (Figure 1a) and hyperintense on a T2-weighted image (Figure 1b). The tumor was a relatively well-demarcated epidural mass with focal invasions into both facet joints. Intraoperative examination showed a neoplasm with clear and granular cell morphologies displaying solid, papillocystic, and follicular patterns. Postoperative 18F-fluoro-2-deoxy-D-glucose positron emission tomographycomputed tomography (CT) and neck CT failed to identify a primary tumor or any other lesions.