Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Somatostatinomas are rare functioning neoplasms usually arising in the pancreas and duodenum. We report a case of somatostatinoma in a 42-year-old male with neither neurofibromatosis nor somatostatinoma syndrome. A large tumor in the descending duodenum had given rise to multiple lymph node metastases. An additional 31 duodenal somatostatinoma cases were also reviewed. Most originated in the descending part of the duodenum, with the ampulla and peri-ampullary area as the most common sites (60%). Frequent manifestations were abdominal pain (25%), jaundice (25%), or cholelithiasis (19%), the latter two reflecting obstruction of the bile duct by tumors. Only two cases showed a possible somatostatinoma syndrome (6%). The tumors with metastases, lymph nodes (10) and liver (2), were significantly larger than average than those without (2.91 +/- 1.49 cm vs 1.36 +/- 0.71 cm, P < 0.05). With a cut-off point of 2.0 cm, diagnostic accuracy for metastasis was 77.78% with 87.50% specificity and 63.64% sensitivity. The smallest tumor with metastases was 0.8 cm and the largest without metastases was 3.0 cm. These results indicate that duodenal somatostatinomas are malignant by nature and the risk of metastasis significantly increases with tumors larger than 2.0 cm.
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PMID:Duodenal somatostatinoma: a case report and review of 31 cases with special reference to the relationship between tumor size and metastasis. 1110 73

Gallbladder carcinoma is the most common malignancy of the biliary tract. There are still many controversies regarding the type of curative surgical treatment for each stage of the disease. The staging system used is the TNM classification of the International Union Against Cancer. Different patterns of spread characterize gallbladder cancer but the two main types are direct invasion and lymph node metastases; since only the depth of invasion can be easily recognized by imaging techniques, it becomes the main variable in choosing the appropriate surgical treatment. Most Tis and T1 tumours are incidentally discovered after cholecystectomy for cholelithiasis and no further therapy is requested; for pT1b tumours, relaparotomy with hepatic resection and N1 dissection is associated with a better survival. For T2 tumours, cholecystectomy with hepatic resection and dissection of N1-2 lymph nodes is the standard treatment, with a 5-year survival of 60-80%. The only chance of long-term survival for patients with a T3-T4 tumour is an extended operation combining an hepatic resection with an N1-2 dissection with or without excision of the common bile duct. A subset of patients with peripancreatic positive nodes or invasion of adjacent organs seems to benefit from a synchronous pancreaticoduodenectomy.
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PMID:Radical surgery for gallbladder cancer: current options. 1101 62

Primary carcinoma of the gallbladder is an uncommon, aggressive malignancy that affects women more frequently than men. Older age groups are most often affected, and coexisting gallstones are present in the vast majority of cases. The symptoms at presentation are vague and are most often related to adjacent organ invasion. Therefore, despite advances in cross-sectional imaging, early-stage tumors are not often encountered. Imaging studies may reveal a mass replacing the normal gallbladder, diffuse or focal thickening of the gallbladder wall, or a polypoid mass within the gallbladder lumen. Adjacent organ invasion, most commonly involving the liver, is typically present at diagnosis, as is biliary obstruction. Periportal and peripancreatic lymphadenopathy, hematogenous metastases, and peritoneal metastases may also be seen. The vast majority of gallbladder carcinomas are adenocarcinomas. Because most patients present with advanced disease, the prognosis is poor, with a reported 5-year survival rate of less than 5% in most large series. The radiologic differential diagnosis includes the more frequently encountered inflammatory conditions of the gallbladder, xanthogranulomatous cholecystitis, adenomyomatosis, other hepatobiliary malignancies, and metastatic disease.
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PMID:Gallbladder carcinoma: radiologic-pathologic correlation. 1125 93

We present an unusual case of a port site tumor from a colonic adenocarcinoma following laparoscopic cholecystectomy. A 66-year-old woman with a previous renal transplant underwent elective laparoscopic cholecystectomy for symptomatic cholelithiasis. Three months later, she re-presented with pain localized to the right lateral port wound. Subsequent investigation revealed an adenocarcinoma in the ascending colon with no evidence of local spread or liver metastases. The patient was enrolled in the ALCCAS trial (an Australasian multicenter prospective randomized clinical study comparing laparoscopic and conventional open surgical treatments of colon cancer in adults) and randomized to the laparoscopic arm. At laparoscopy, nodules of tumor were located at the sites of the previous right flank and umbilical trocar sites. The procedure was converted to an open hemicolectomy with excision of port sites. To our knowledge, only one other case of a colon carcinoma metastasizing to a port site following laparoscopic cholecystectomy has been reported. This case illustrates a number of points: (a) This was an advanced tumor at the time of diagnosis and was undoubtedly present at laparoscopic cholecystectomy. (b) This tumor was not manipulated at the time of laparoscopic cholecystectomy. (c) The patient was immunosuppressed at the time of laparoscopic cholecytstectomy. (d) The patient developed clinical recurrence in her laparotomy wound within 3 months of her open procedure. This case supports current arguments that the etiology of port site metastases is likely to be mulifactorial.
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PMID:An unusual case of port site seeding. 1144 29

Somatostatinomas are the rarest pancreatic endocrine tumors and can arise in the pancreas or duodenum. Duodenal somatostatinomas are less common than, and are distinguished from, their pancreatic counterparts by a frequent association with type I neurofibromatosis, the presence of psammoma bodies, the less frequent presence of metastatic disease, and the absence of somatostatinoma syndrome (diabetes mellitus, steatorrhea, and cholelithiasis). We report a case of somatostatinoma with metastases and psammoma bodies presenting with all three features of the syndrome in a patient with neurofibromatosis. Although several reports have documented portions of the syndrome in patients with duodenal somatostatinomas, to our knowledge, this is the first report of the complete syndrome associated with a duodenal lesion.
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PMID:Duodenal somatostatinoma presenting with complete somatostatinoma syndrome. 1160 61

A 43-year-old man complained that during the previous 2 years he had experienced a number of episodes of hepatic colic. After examination, we diagnosed a symptomatic cholelithiasis with a sclerosed and atrophic gallbladder. He underwent laparoscopic surgery. During the operation, we observed multiple peritoneal tumors that appeared to be metastases of a gallbladder cancer. The histological study demonstrated a benign chronic cholecystitis accompanied by multiple peritoneal cystic mesotheliomas, an extremely rare tumor in men. The etiology of cystic mesothelioma is still unclear. It has been suggested that they are really multiple inclusion cysts that result from a proliferative reaction within the peritoneal tissue; their continued proliferation might be caused by the continued persistence of an inciting factor. However, in our patient, the proliferation appeared to be related to an extensive peritoneal tissue reaction to the chronic gallbladder inflammatory process. We did not use sclerosing therapy because we had resected the gallbladder and most of the visible lesions laparoscopically; therefore, we had most likely eliminated the potential source of the inciting factor. Because it is very difficult during laparascopy to differentiate these benign quistic mesotheliomas from peritoneal metastases or tuberculous lesions, it is debatable whether the surgeon should continue or terminate the laparoscopic procedure in these ambivalent and potentially risky circumstances.
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PMID:Peritoneal cystic mesothelioma. 1196 60

The possibilities and the limits of transabdominal ultrasonography (US) in the diagnosis of bilio-pancreatic diseases are reviewed here in the light of the last 10 years' research. US remains the method of choice for the diagnosis of gallstones and is generally accepted as an initial imaging technique in gallstone complications, such as acute cholecystitis. Moreover the method can be useful for the detection of the biliary complications after laparoscopic cholecystectomy and after liver transplantation. US is still considered the first diagnostic procedure when stones are suspected in the common bile duct. The use of color Doppler can provide a differential diagnosis of gallbladder cancer with respect to other benign inflammatory or polypoid lesions. Color Doppler US allows to detect vascular complications of acute pancreatitis such as pseudoaneurysms. US is still considered useful for the initial screening of the pancreatic cancer. However, for staging other imaging techniques must be employed. With US useful informations are obtained in the diagnosis of cystic tumors of the pancreas and of pancreatic metastases. US is generally of little use for the diagnosis of endocrine tumors.
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PMID:The role of ultrasound in biliary and pancreatic diseases. 1257 83

The members of four generations of a family with Von Hippel-Lindau syndrome (VHL) have been followed by one of us (I.P.) for 30 years. The disease was proved in four members of this family, in three of them associated with pheochromocytoma. The grandmother (I-1) died at the age of 16 years two months after her first birth. The cause of death was not established. Her daughter (II-1) had 9 births with 5 children alive. Paresthesia and difficulties in walking followed by paraparesis and paraplegia were the first signs of the disease at the age of 58 years. The surgical treatment was performed because of an expansive lesion at the level of Th 3-4. Pathohistological examination was not done. It seems that a haemangioblastoma might be the cause of her disease. Diagnosis of pheochromocytoma was documented in a female patient (III-2) in 1972. Two years later she was successfully operated on. Pathohistological examination proved clinical diagnosis. She had also diabetes mellitus, cholelithiasis and cardiomyopathy. She died at the age of 56 years. A right-sided pheochromocytoma was diagnosed in a next female patient (III-4) at the age of 22 years. Her surgical treatment was successful. Retinal haemangioblastomatosis was established 7 years later in this patient. She was blind at the end of her life. Haemangioblastomatosis cerebelli was diagnosed soon, and she died at the age of 51 years. A 12- year old boy (IV-3) presented severe hypertension (36/24 kPa). Left-sided pheochromocytoma was removed in this patient one year later. Right-sided pheochromocytoma was operated on in the same patient at the age of 24 years. An elevated level of urinary dopamine was documented four years after the second operation. A malignant right-sided pheochromocytoma was operated on in the same patient 15 years later. At the same time metastases were found in the lower part of the right lung lobe. A 131-I-MIBG therapy could not be realized. He died at the age of 41. Pathohistological examinations proved the clinical diagnosis in this patient after all of three surgical treatments. MEN 2 syndrome was excluded by proper genetical analyses on the RET-protooncogen. Genetical analyses are in the course to identify the possible mutations of VHL-tumour-suppressor gene through the living members of the family. Multidisciplinary approach is mandatory in diagnosis, follow up and treatment of this specific group of patients. A collaboration among specialists of different fields of medicine (internal medicine, ophthalmology, neurology, radiology, urology, neurosurgery, biochemistry, pathology and genetics) is suggested.
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PMID:[The von Hippel-Lindau syndrome with pheochromocytoma]. 1258 97

This study was carried out to obtain extensive information on carcinoids (the carcinoid group) and related variant endocrinomas (the variant group) of the gallbladder, and to statistically analyze their characteristics from various clinicopathologic aspects. A total of 138 cases were collected from the international sources, 101 belonging to the carcinoid group and 37 to the variant group. The first group consisted of 81 cases of typical and 20 atypical carcinoids. Comparative evaluation was attempted mainly between the carcinoid and variant groups, and occasionally between the typical and atypical carcinoid series when statistical significance was suspected. The carcinoid group showed a statistically significant difference from the variant group by exhibiting a younger average age (61.7 years vs 69.7 years: P<0.01), a higher incidence of associated cholelithiasis (87.3% vs 56.0%: P<0.01), a higher incidence of small tumors 50 mm or less (85.2% vs 52.9%: P<0.01), a smaller average tumor-size (29.6 mm vs 58.7 mm: P<0.01), a lower rate of metastases (40.7% vs 70.6%: P<0.05), a higher immunoreactivity rate of chromogranin (100.0% vs 66.7%: P<0.01), a lower immunoreactivity rate of gastrin (23.8% vs 70.6%: P<0.01), and a higher five-year survival rate (60.4% vs 21.3%: P<0.0005). Significant differences in various clinicopathological aspects confirmed between the carcinoid group and the variant group suggested that endocrine carcinomas of these two groups perform a different clinical pattern, represented most clearly by postoperative outcomes. These groupings are decided on the basis of histologic patterns, namely, well to poorly differentiated endocrine carcinomas (typical to atypical carcinoids) and undifferentiated or anaplastic variants of other endocrine carcinomas. The basic criteria for such classification of these endocrine carcinomas based on international agreements are required.
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PMID:Primary endocrinomas (carcinoids and variant neoplasms) of the gallbladder. A statistical evaluation of 138 reported cases. 1272 16

The gallbladder is rarely the site of distant metastases and in most cases malignant melanoma is the primary tumor. We report a case of a 64-year-old man with a gallbladder metastasis secondary to a renal cell carcinoma. Renal cell carcinoma has a tendency toward metastatic disease, the most notable features of this tumor being its unusual pattern of metastatic disease. Pre-operative imaging studies are often futile in the differentiation between primary and secondary tumors of the gallbladder. Since primary tumors of the gallbladder often coexist with gallstones, a polypoid lesion in an acalculous gallbladder is more consistent with metastasis than a primary tumour. If feasible, surgical resection of the gallbladder is mandatory because it could guarantee better chances of survival for patients with metastatic renal carcinoma.
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PMID:Metastatic carcinoma of the gallbladder after a renal cell carcinoma. 1276 71


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