1/20. Sclerosing mesenteritis seen clinically as pancreatic pseudotumor: two cases and a review.Sclerosing mesenteritis is an uncommon nonneoplastic inflammatory process in the mesentery that is seen as a pseudotumor, usually involving the small bowel mesentery, the mesenteric fat, and less commonly, the mesentery of the large bowel. We report two cases of sclerosing mesenteritis and review the literature on this rare disease. Both patients had pain, profound weight loss, and a mass on computed tomography (CT) scan of the abdomen. The provisional diagnosis was pancreatic neoplasm on the basis of clinical presentation and imaging studies. The diagnosis of sclerosing mesenteritis was established by histologic findings in biopsy material obtained at laparotomy in both cases. Interval histologic studies in one patient who had a high CA 19-9 level, progressive biliary ductal and partial duodenal compression, revealed a transitional histologic pattern from predominant inflammation and fat necrosis to predominant fibrosis. This may explain the varied descriptive terms used in the literature to describe this entity.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/20. Primary pancreatic lymphoma: clinicopathological analysis of 19 cases from japan and review of the literature.Primary pancreatic lymphoma (PL) is an extremely rare disease, and large-scale studies are rarely performed even in Western countries, in which all cases of PL reported to date were of the B-cell type. Little information regarding PL is available in japan. Nineteen cases of PL were collected through a nationwide study in japan, and paraffin-embedded specimens were processed for staining with hematoxylin and eosin and by immunohistochemical procedures. Clinicopathological findings were reviewed and compared with those reported in Western countries. The patient population consisted of 13 men and 6 women, ranging in age from 46 to 84 (average 62) years. abdominal pain was the most common presenting symptom. Tumors were located in the pancreatic head (12 cases), tail (4 cases) and body (2 cases), and ranged in size from 4 to 17 cm. Clinical stage was I(E) in 9 cases and II(E) in 10. Radical resection was performed in 10 cases and bypass operation in 1, followed by chemotherapy in 8. Immunophenotypically, 15 cases were B-cell and 4 were T-cell lymphomas. Seventy-three percent of B-cell tumors were diffuse large B-cell lymphomas. The 1-year actuarial survival rate for B-cell lymphomas (51.9%) was better than that of T-cell lymphomas (0%). However, in japan the incidence of T-cell PLs was higher, and, partly as a consequence of this, prognosis was poorer than in Western countries.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/20. Mucinous cystic lesions of the pancreas. Report of a patient submitted to distal pancreatectomy.Intraductal papillary neoplasm consists a variant of the mucin-producing neoplasms of the pancreas; these are rare diseases that can be divided into mucinous ductal ectasias and intraductal papillary neoplasms. Such neoplasms have a malignant potential, and their prognosis is good if they are early diagnosed and localized surgical resection. This paper describes a case of an intraductal papillary neoplasm localized in the tail of the pancreas and treated by distal splenopancreatectomy. The literature on this rare disease is also reviewed.- - - - - - - - - - ranking = 2keywords = rare disease (Clic here for more details about this article) |
4/20. Ampullary carcinoid tumors: rationale for an aggressive surgical approach.Two cases of ampullary carcinoid tumor are reported. These tumors are among the most rare of GI tract carcinoids and appear to have a distinct presentation and biological behavior from carcinoids arising in the duodenum. The existing literature is reviewed with attention to the implications for surgical management of this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/20. Small cell undifferentiated carcinoma of the pancreas. Report of a patient with tumor marker studies.BACKGROUND. Small cell undifferentiated carcinoma of the pancreas is a rare type of pancreatic neoplasm. methods. The authors report the clinical and pathologic aspects of a patient with this malignant lesion and an extensive serologic and immunohistochemical survey of potential ectopically produced hormones or tumor markers. RESULTS. Neuron-specific enolase (NSE) emerged as a tumor marker. CONCLUSIONS. NSE could be valuable in the diagnosis and management of other patients with this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/20. Primary pancreatic lymphoma presenting as acute pancreatitis.Primary pancreatic lymphoma is a rare disease and usually presents with nonspecific symptoms like abdominal pain, weight loss, or jaundice. Here we report a case of primary pancreatic lymphoma presenting as acute pancreatitis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/20. Curative resection of a small cell carcinoma of the pancreas: report of a case of long survival without chemotherapy.A 41-year-old asymptomatic man was, by chance, diagnosed as having a mass in the left upper quadrant of the abdomen by ultrasound. Computed tomography and magnetic resonance imaging showed a slightly enhanced heterogeneous mass, measuring about 5 cm in diameter, adjacent to the pancreas tail and spleen. On abdominal angiography the tumor was found to be fed by the splenic artery, and no encasement was observed. At operation the tumor was connected to the pancreas tail and attached to the spleen, and no metastasis was evident. As a result of these observations, a curative resection was performed. On histological examination the tumor cells had no ductal or architectural organization and were continuous to the normal pancreatic tissues. The cells were negative for Grimelius argylphile and the periodic acid schiff stain. immunohistochemistry indicated that the tumor cells were positive for keratin and epithelial membrane antigen, but negative for chromogranin a, vimentin, alpha1-antitrypsin and alpha1-antichymotrypsin. The tumor was diagnosed as a small cell carcinoma of the pancreas, which is a rare disease. In previously reported cases curative resection of the tumor had not been possible because of its rapid progression and metastasis, and the prognosis is generally very poor. In our case, however, curative resection of the tumor was done, and the patient is healthy with no signs of recurrence for 56 months after the operation and with no additional therapy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/20. A case of primary non-Hodgkin lymphoma of the pancreas.Non-Hodgkin pancreatic lymphoma is a rare disease. Its diagnosis is difficult without histological examination. Ultrasonographic, computed tomodensitometric, and endoscopic retrograde cholangio-pancreatographic findings are not pathognomonic. The better prognosis of these tumors, compared to adenocarcinoma, and their sensitivity to chemotherapy, implies the need for pathologic examination of every pancreatic tumor.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/20. A rare case of serous cystadenocarcinoma of the pancreas.Serous cystadenocarcinoma of the pancreas, a rare disease, developed in a 63-year-old Japanese woman. Pathologic examinations of the pancreatic tumor at the subtotal pancreatectomy showed it to be serous cystadenoma with focal atypical lesions. Three years after the operation, however, metastatic liver nodules were found, and the histologic characteristics of these lesions were quite similar to those of the pancreatic neoplasm. Both primary and metastatic tumors were composed of multiple cysts separated by fibrous septa. The epithelium of cysts was cuboidal and had clear cytoplasm, which had positive results for periodic acid-Schiff (PAS) and negative results for PAS with diastase, alcian blue, and mucicarmine. To the knowledge of the authors, serous cystic neoplasms of the pancreas have been uniformly benign in biologic behavior. Recently, however, serous cystadenocarcinoma of the pancreas has been reported as a new entity. The current case is the second reported case and might support the existence of serous cystadenocarcinoma of the pancreas.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/20. Primary pancreatic lymphoma. Report of five cases.Primary pancreatic lymphoma (PPL) is a very rare disease. We report five cases of PPL (4 men and 1 woman, mean age 65 years) diagnosed and treated at our Institution from 1987 to 1997. None of these patients had evidence of extrapancreatic disease and they were categorized as PPL involving pancreas only (stage IE, 3 patients) or pancreas and peripancreatic lymph nodes (stage IIE, 2 patients). The most common presenting symptoms were abdominal pain and weight loss. Imaging techniques showed a mass of the pancreatic head in all cases. The histological diagnosis (3 diffuse-large cell non-Hodgkin's lymphoma and 2 lymphoplasmacytic lymphoma/immunocytoma) was made by ultrasound-guided fine needle aspiration biopsy and tissue core fine-needle biopsy in three patients and by surgery in the remaining two patients. The three patients diagnosed by percutaneous biopsy were treated with chemotherapy as front-line therapy and two of them received also local radiotherapy; one of these patients is still alive in complete remission at 69 months, one died of an unrelated disease at 67 months and one died of lymphoma relapse at 88 months. Two patients underwent pancreaticoduodenectomy plus adjuvant chemotherapy; one of them died of recurrent cholangitis 8 months after surgery while the other one is still alive in complete remission after 160 months. This study shows that: 1) imaging techniques can suggest the suspicion of PPL but are unable to distinguish PPL from pancreatic adenocarcinoma; 2) histological diagnosis can be easily obtained by percutaneous US-guided tissue core biopsy; 3) surgery can be avoided both for diagnosis and therapy but the treatment of choice of PPL may only be evaluated on a larger series of patients.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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