1/13. Primary hepatic low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue type: a case report and review of the literature.Primary hepatic lymphoma, mostly diffuse large B-cell lymphoma, is a rare disease. We describe an extremely rare case of low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) type occurring in the liver. A 61-year-old man with a history of hepatitis a presented with early gastric cancer and a liver mass. Needle biopsy of the liver tumor suggested low-grade B-cell lymphoma by histology and polymerase chain reaction of the immunoglobulin heavy chain gene. The tumor (3.4 x 2.8 x 2.4 cm) was completely resected from the anterior segment of the right lobe of the liver. Atypical lymphoid cells of small to intermediate size proliferated in the tumor, and lymphoepithelial lesions were recognized. Immunohistochemically, lymphoma cells were positive for CD20 and negative for CD5, CD10, and cyclin d1. Staging procedures showed no lymphoma lesion other than the liver tumor. Thus, the patient was diagnosed with low-grade hepatic marginal zone B-cell lymphoma of the MALT type. The patient has been followed up for 1.5 years since surgical resection with no recurrence. The clinicopathologic characteristics and management of this rare disease are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/13. Case presentation of gastrinoma combined with gastric carcinoid with the longest survival record -- zollinger-ellison syndrome: pathophysiology, diagnosis and therapy.BACKGROUND: zollinger-ellison syndrome is a very rare disease caused by tumor with gastrin producing cells accompanied by hypergastrinemia leading to gastric hypersecretion and peptic ulcers and their complications.CASE STUDY: female case of gastrinoma (zollinger-ellison syndrome; Z-E) with a record of 38 yrs of survival. Acute gastro-duodenal ulcers started at 28 yr of age and Z-E was diagnosed by using gastrin assays. Basal and maximal acid outputs and ratio of basal/maximal outputs were away over normal limits. Because of ulcer recurrence and complications, patient was subjected to several gastric surgeries but refused total gastrectomy. She was also treated with many H2-receptor (R) antagonists and proton-pump inhibitors (PPI), each new drug being initially highly effective but then showing declining efficacy except when PPI, lansoprazole was used. The gastrin level rose in the course of disease from initial high value of 2000 pg/mL to the extreme 4500 ng/mL at present. During the last 2 yrs, metastasis mainly to liver developed and they were successfully treated by synthetic octapeptide derivative of somatostatin and, as a result, metastatis partly reduced and plasma gastrin drasticly decreased. biopsy taken from liver metastasis showed the presence of typical gastrinoma cells with gastrin and chromogranin, while that from oxyntic mucosa revealed the ECL-cell hyperplasia with carcinoid tumors and unexpected gastric atrophy.CONCLUSIONS: This phenomenal case described in this article might be the new proven evidence needed by gastroenterologists to overturn the traditional treatment using total gastrectomy as a treatment of choice to the partial gastrectomy combined with proton pump inhibitors.- - - - - - - - - - ranking = 0.5keywords = rare disease (Clic here for more details about this article) |
3/13. pseudomyxoma peritonei: report of an unusual case.pseudomyxoma peritonei, a rare disease, is characterized by accumulation of mucinous material in the peritoneal cavity and is commonly caused by ovarian tumors or appendicular lesions. This report presents necropsy findings of an unusual case of pseudomyxoma peritonei caused by dissemination of mucinous adenocarcinoma of the stomach, hitherto unreported in literature.- - - - - - - - - - ranking = 0.5keywords = rare disease (Clic here for more details about this article) |
4/13. Endoscopic mucosal resection in the management of gastric carcinoid tumors.BACKGROUND AND STUDY AIMS: Gastric carcinoid tumors are a rare disease. Previously, total gastrectomy was regarded as the treatment of choice. However, differences in biological malignancy have recently led to the increased use of endoscopic mucosal resection (EMR) for treatment. We studied the outcome of EMR in patients with gastric carcinoids who were treated at our hospital and discuss the indications for endoscopic treatment. patients AND methods: Between 1986 and 1999 we carried out gastric mucosal resection in five patients with gastric carcinoid tumors. The procedure used for EMR was either strip biopsy or endoscopic aspiration mucosectomy. RESULTS: The carcinoid tumors measured 10 mm or less in four of the five patients. Two patients had type A gastritis, and all had hypergastrinemia. There was no evidence of recurrence during follow-up (range 6 - 66 months; mean 32.6 months). CONCLUSION: EMR is useful in the management of type 1 gastric carcinoids as classified by Rindi (hypergastrinemia; tumor diameter of 10 mm or less).- - - - - - - - - - ranking = 0.5keywords = rare disease (Clic here for more details about this article) |
5/13. Primary neutrophil-rich, CD30-positive anaplastic large cell lymphoma of the stomach: case report and review of the literature.Primary T-cell lymphoma of the stomach is a rare disease, most gastric lymphomas being of B-cell type. Here we describe a unique case of primary neutrophil-rich CD30-positive anaplastic large cell lymphoma (ALCL) of the stomach that was treated and cured by combined chemotherapy. According to our literature review, only 7 cases of primary gastric ALCL have been previously reported, none of them being of the neutrophil-rich subtype. Although very peculiar in its histological presentation, which may simulate an inflammatory or carcinomatous process, the natural history as well as the clinical features of this unusual gastric lymphoma does not differ from the other reported cases of gastric ALCL.- - - - - - - - - - ranking = 0.5keywords = rare disease (Clic here for more details about this article) |
6/13. Follicular lymphoma of the stomach: immunohistochemical and molecular genetic studies.The gastrointestinal (GI) tract is the most common site for the development of extranodal non-Hodgkin's lymphomas, with most cases having a diffuse architectural pattern. Follicular lymphoma (FL) of the stomach is a very rare disease, and little is known about the immuno-histochemical and molecular genetic characteristics of this type of lymphoma. Despite their rarity, FLs are an important consideration in the differential diagnosis of follicular hyperplasia of mucosa-associated lymphoid tissue (MALT), a relatively frequently encountered lesion with the potential to cause significant diagnostic difficulty, especially when assessment is based on scanty biopsy material. We report a 57-year-old woman with gastric FL, and describe the results of immunohistochemical and molecular genetic studies.- - - - - - - - - - ranking = 0.5keywords = rare disease (Clic here for more details about this article) |
7/13. Inflammatory myofibroblastic tumor of the stomach with peritoneal dissemination in a young adult: imaging findings.Inflammatory myofibroblastic tumors are lesions that most often affect young adults and children. These tumors have been found in numerous extrapulmonary sites but rarely in the stomach. It is unknown whether this process is reactive or neoplastic. They are infiltrative lesions and often extend through the gastric wall, sometimes reaching adjacent organs including the esophagus, duodenum, peritoneal cavity, spleen. pancreas, and liver. These features mimic malignancy on endoscopy and radiology. We report the ultrasound, color Doppler ultrasound, and helical computed tomographic findings of a gastric inflammatory myofibroblastic tumor with peritoneal dissemination in a young adult. To our knowledge, this is the first report of color Doppler ultrasound and helical computed tomographic findings of this rare disease entity.- - - - - - - - - - ranking = 0.5keywords = rare disease (Clic here for more details about this article) |
8/13. Menetrier's disease in korea: report of two cases and review of cases in a gastric cancer prevalent region.Menetrier's disease is a rare disease of the stomach generally described as hypertrophic gastropathy associated with hypoproteinemia. Gastric resection is still the most definitive treatment for the disease, but the appropriate extent of resection has not been determined. One of the major factors that would determine the extent of gastric resection in Menetrier's disease is its malignant potential. We present two recent cases of Menetrier's disease treated in our institution and review cases of the disease reported in korea where the incidence of gastric cancer is one of the highest in the world.- - - - - - - - - - ranking = 0.5keywords = rare disease (Clic here for more details about this article) |
9/13. A case of primary gastric CD30-positive anaplastic large-cell lymphoma.Gastric CD30-positive anaplastic large-cell lymphoma is a very rare disease. It is sometimes difficult to distinguish it from undifferentiated carcinoma, sarcoma and so on. We report here on a case of primary gastric anaplastic large-cell lymphoma. A 50-yr-old woman complained of epigastric pain and severe chest pain for 1 week. The gastroendoscopic examination revealed geographic mucosal irregularities with shallow ulceration at the antrum. She underwent a total gastrectomy. The gross finding of the resected stomach was an 8 x 4.5 cm sized ulceroinfiltrative lesion at the pyloric antrum along the lesser curvature. The microscopic examination revealed diffuse and solid proliferations of large atypical cells with pleomorphic nuclei. Immunohistochemically, the tumor cells were positive for CD30, vimentin and CD3, and this was a finding compatible with anaplastic large-cell lymphoma. To the best of our knowledge, this is the first such reported case in korea.- - - - - - - - - - ranking = 0.5keywords = rare disease (Clic here for more details about this article) |
10/13. Primary gastric small cell carcinoma: report of a case and review of the literature.A 52-year-old man suffering from a pure-type primary gastric small cell carcinoma was treated with surgery and combination chemotherapy. The small cell carcinoma, approximately 6.5 cm in diameter, was situated in the posterior wall of the antrum and there were no distant metastases. Total gastrectomy and regional lymph node dissection was carried out. Histological examination revealed a solid pattern of proliferation of small cells with hyperchromatic, round nuclei and scant cytoplasm. Neoplastic cells infiltrated into the subserosal layer with severe lymphatic and vascular invasion. Regional lymph node cells were mostly replaced by tumor cells that stained positive for Grimelius, neuron-specific enolase (NSE), and synaptophysin. Accumulations of electron-dense core granules in the small neoplastic cells were seen by electron microscopy. Following surgery, the patient was treated with adjuvant chemotherapy consisting of cisplatin and etoposide. The patient is alive and recurrence free 3 years after surgical operation. We review 107 published cases of primary gastric small cell carcinoma, an extremely rare disease first reported in 1976. Small cell carcinoma is an aggressive, malignant tumor. Intensive chemotherapy is essential for patient survival even when curative surgical resection is carried out.- - - - - - - - - - ranking = 0.5keywords = rare disease (Clic here for more details about this article) |
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