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)

A retrospective study of 90 surgically treated patients with the Zollinger-Ellison syndrome seen from 1958 through 1990 was performed. Fifteen patients had Zollinger-Ellison syndrome as a manifestation of multiple endocrine neoplasia type I. Preoperative tumor localization was positive in 46% of 54 patients studied. Gastrinomas were identified in 66% of patients, 38% of the tumors being malignant. Postoperative eugastrinemia was achieved in 11% of patients after a variety of surgical procedures. Exploratory laparotomy provides the only chance for cure and identifies the significant prognostic factors associated with long-term patient survival: small tumor size, extrapancreatic primary, and absence of tumor metastases.
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PMID:The Zollinger-Ellison syndrome. A collective surgical experience. 135 63

Among 33 patients with endocrine pancreatic tumors due to multiple endocrine neoplasia type 1 (MEN-1), 19 (58%) patients had hypergastrinemia, 7 (21%) patients had hyperinsulinism, and 7 (21%) patients had clinically non-functioning lesions. At least one gross tumor was found in all patients undergoing pancreatic surgery, including those with negative localization studies prior to operation. The patients also had additional macroscopic tumors as well as numerous microadenomas, and the lesions frequently were positive for immunostaining with multiple hormones, mainly pancreatic polypeptide, insulin, glucagon, and somatostatin. Duodenal endocrine lesions were found in 4 of 5 investigated patients and stained with gastrin and somatostatin antibodies. Distal, mainly subtotal pancreatic resection, was performed in 18 patients, eventually combined with caput tumor enucleation or duodenotomy, while a few patients underwent only tumor enucleation or a Whipple procedure. The long-term outcome of operation was most favorable in patients with hyperinsulinism; only 1 patient had clinical recurrence. Patients with hypergastrinemia experienced only transitory lowering of serum gastrin values after pancreatic surgery and 47% of them had or developed metastases. Such tumor spread was seen in 57% of the patients with non-functioning lesions. Nine patients died from progressive tumor disease during follow-up. Consistent with previous studies, we found that surgery is indicated in MEN-1 patients with hyperinsulinism even if a lesion is not visualized by radiology. In addition, these indications should be extended to also include patients with only biochemical markers of disease, including elevations of gastrin, as these indicate the presence of gross tumors.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pancreatic tumors in multiple endocrine neoplasia type 1: clinical presentation and surgical treatment. 135 27

During examination before surgical correction of pes valgus a 20-year-old man reported having 3-5 pasty, foul smelling diarrhoeic motions per day for the past 3 years. He was noted to have rather thick lips and Marfan-like body build. Erythrocyte sedimentation rate was 18/34 mm, serum activity of GOT 22.5 U/l, GPT 35.7 U/l. Faecal weight was increased to 640 g/d, fat content to 12 g/d. Serum levels of the carcinoembryonic antigen (2494 ng/ml; normal: < 2.5) and of calcitonin (1,619,760 pg/ml; normal < 100) were elevated. Gastroscopy, partial coloscopy, colon-contrast imaging, ultrasonography and computed tomography of the neck and abdomen, as well as magnetic nuclear imaging of the neck were all normal. But laparoscopy revealed the liver to be infiltrated by small whitish nodules which immunohistologically proved to be metastases of a C-cell carcinoma. Total thyroidectomy was performed and the diagnosis of a C-cell carcinoma of the thyroid confirmed intra-operatively. After the operation the diarrhoea was stopped with codeine (9 mg/d). In case of tumour progression, therapy with octreotide, a somatostatin analogue, will be carried out. The concomitant occurrence of C-cell carcinoma, Marfan-like body build, thick lips and skeletal changes is typical of multiple endocrine neoplasia type 2B, which is caused by a chromosomal defect.
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PMID:[Type-2B multiple endocrine neoplasms with diffuse liver metastases as the cause of chronic diarrhea]. 135 89

Nineteen patients were surgically treated for hyperparathyroidism associated with multiple endocrine neoplasia type 1 syndrome. Fourteen patients (74%) had removal of three or more parathyroid glands at the first operation, and five (26%) by removal of 2 1/2 or fewer glands. Two patients had recurrent hypercalcemia during the mean follow-up period of 65 months. One had a recurrence 10 years after subtotal parathyroidectomy. Reexploration in this patient revealed enlargement of the remaining tissue in the neck and an enlarged supernumerary gland in the aorticopulmonary window. The other patient had persistent hypercalcemia after removal of two hyperplastic parathyroid glands until after another 1 1/2 more glands were removed. After reoperation the patient was normocalcemic for 10 years before hypercalcemia was again noticed. The patient subsequently died from renal carcinoma metastases, which might have been the cause of the hypercalcemia before death.
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PMID:Primary hyperparathyroidism in patients with multiple endocrine neoplasia type 1: experience by a single surgical team in Japan. 136 3

Islet cell neoplasia is a frequent occurrence in multiple endocrine neoplasia type 1 (MEN 1). Sixteen of 27 patients with MEN 1 developed functioning endocrine pancreatic tumor syndromes. Eleven of the 16 developed Zollinger-Ellison syndrome and each was evaluated by a combination of computed tomography and hepatic angiography to exclude hepatic metastasis and percutaneous transhepatic catheterization to localize the tumor. Seven of the 11 patients were found to have duodenal gastrinomas with multiple duodenal tumors in three patients. Four of the 11 patients had only pancreatic gastrinomas. In addition to the gastrinomas, other types of islet tumors in the pancreatic body or tail were found in nine of the 11 patients. None of the patients had hepatic metastases. Seven of the 11 patients were treated by distal pancreatectomy and since 1986 all patients have had duodenotomies as part of the surgical exploration. Postsurgical evaluation ranging from three months to 14 years indicates that 10 of 11 patients have normal basal gastrin levels. We conclude that duodenal gastrinomas are common in MEN 1 and can be managed successfully by appropriate operative intervention.
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PMID:Surgical treatment of the endocrine pancreas and Zollinger-Ellison syndrome in the MEN 1 syndrome. 136 4

In this surgical series of 100 pheochromocytomas (PH), where 91 cases were studied and followed up, the frequency of malignancy appears high (29%) on macroscopic criteria observed at the first operation (25 out 26 cases of malignant PH) or thanks to the occurrence of metastases (1 case out of 26 malignant PH). While some clinical signs are suggestive, there is no excretory profile predictive of malignancy. Isolated or predominant secretion of dopamine is not specific of malignancy event if it occurs more frequently and severely in malignant PH. Bilateral involvement, occurring in the setting of phacomatosis or of multiple endocrine neoplasia (MEN II), or in a sporadic case, is not a predictor for malignancy. Contrarily, extraadrenal involvement is a major feature indicative of malignancy. A CT scan which demonstrates possible locoregional invasion, and a MIGB scan revealing distant localizations raise the suspicion of malignancy. The preferred surgical approach is via a laparotomy, thus allowing the exploration of all possible abdominal chromaffin site and the search for intraabdominal metastases. Intraoperative MIBG scanning checks the completeness of the excision. Flow cytometric DNA ploidy studies have been performed in 25 of our patients. The follow up of benign or malignant PH, after excision "for cure" must be life-long: clinically, biologically and by MIGB scan. Persistence of the disease after surgery or recurrence with unresectable metastases can benefit from MIGB and chemotherapy.
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PMID:High incidence of malignant pheochromocytoma in a surgical unit. 26 cases out of 100 patients operated from 1971 to 1991. 147 48

Thirty-three bronchial carcinoid tumours operated on in a 22-year period are reviewed. They were histologically verified as typical carcinoids with Grimelius' argryophilic staining (25 cases), electron microscopy (6) and immunostaining for synaptophysin (4). Nineteen were endobronchial and 14 peripheral, intraparenchymal tumours. Lymph-node metastases were present at operation in two patients. Two tumours, in patients with Cushing's syndrome, were hormonally active (one secreting ACTH and the other ACTH and calcitonin). There was one case of multiple endocrine neoplasia syndrome, but none of carcinoid syndrome. During follow-up for 5-18 (mean 8.2) years five patients died of unrelated causes. Only one patient showed distant metastasis (after 15 years in salivary gland and a year later in mediastinal fat). Extensive clinical re-examination was performed on 20 patients, and six others were interviewed. All were well but one, who died of uterine cancer 8 weeks later. Typical bronchial carcinoid is concluded to be of low-grade malignancy and suitable for limited pulmonary resection.
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PMID:Bronchial carcinoid: a clinical follow-up study of 33 cases. 166 42

Flow cytometry of medullary thyroid carcinoma (MCT) was performed in a large family with the MEN-2A syndrome. Of 15 family members with MCT five patients (10-27 yr) were without lymph node metastases. Six patients had a normal pentagastrin test after operation. All patients are alive and free of symptoms of MCT 6-9 yr after total thyroidectomy and an ablative dose of 131-I. In 12 of the 15 patients with MCT flowcytometry of paraffin-embedded tissue could be performed. The majority of all tumors (n = 9) were classified as peridiploid. Metastatic tumor, 6 years after thyroidectomy, in one of the patients was diploid. Only two MCT were clearly aneuploid. In one patient the tumor was tetraploid. We conclude that the majority of the MCT patients in this family with the MEN-2A syndrome have no or limited ploidy aberrations in their tumors, which correlates well with the favourable prognosis of familial MCT.
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PMID:Nuclear DNA content of medullary thyroid carcinoma in a large family with the MEN-2A syndrome. 167 51

In patients with multiple endocrine neoplasia type 1 (MEN-1), gastrinomas are common and thought to occur predominantly in the pancreas. We describe eight patients with MEN-1 and hypergastrinemia (seven with the Zollinger-Ellison syndrome) in whom we searched for neuroendocrine tumors in the pancreas and duodenum. Tumors were found in the proximal duodenum in all eight patients: solitary tumors (diameter, 6 to 20 mm) in three patients and multiple microtumors (diameter, 2 to 6 mm) in the other five. Paraduodenal lymph-node metastases were detected in four patients. Immunocytochemical analysis revealed the presence of gastrin in all the duodenal tumors and in their lymph-node metastases. In contrast, no immunoreactivity for gastrin was present in the endocrine tumors found in the seven pancreatic specimens available for study, except for one tumor with scattered gastrin-positive cells. In four of the six patients whose duodenal gastrinomas were removed, serum gastrin levels returned to normal; in the other two patients gastrin concentrations decreased toward normal. We conclude that in patients with MEN-1 and the Zollinger-Ellison syndrome, gastrinomas occur in the duodenum, but the tumors may be so small that they escape detection.
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PMID:Gastrinomas in the duodenums of patients with multiple endocrine neoplasia type 1 and the Zollinger-Ellison syndrome. 197 64

The nuclear DNA content of paraffin-embedded parathyroid tumors from 49 patients with proven primary hyperparathyroidism was determined by flow cytometric analysis. The lesions included 14 primary and 11 locally recurrent or metastatic lesions from 16 carcinoma patients, 28 single adenomas from 28 patients, and 15 hyperplastic glands from five patients with familial multiple endocrine neoplasia type 1. No abnormal DNA stemline was found in any of the hyperplastic glands. One (3.6%) of the adenomas was aneuploid. There was no difference in ploidy patterns between the primary and recurrent lesions of the carcinomas and five (31%) of the carcinomas expressed aneuploidy. Four of the five patients with aneuploid carcinoma had recurrences including pulmonary metastases. One of them died of this disease 12 years after the initial operation, and all except one of the others are hypercalcemic even after removal of the successive recurrent or metastatic tumors. Of the 11 patients with diploid carcinoma, four had either local recurrence or pulmonary metastasis. Two of them are living with normocalcemia 3 and 6 years, respectively, after removal of the recurrent tumors and the others are alive with mild hypercalcemia. The remaining seven patients with diploid carcinoma, however, have no recurrence 2 to 5 years after the initial operation. Thus aneuploid parathyroid carcinomas are likely to show more malignant behavior than those with a diploid DNA pattern. All of the patients with adenoma and hyperplasia have been normocalcemic after a mean follow-up interval of 37 months. This study indicates that flow cytometric analysis of nuclear DNA content is a valuable adjunct to histologic examination in the diagnosis of parathyroid carcinoma and the prediction of the clinical outcome.
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PMID:Flow cytometric DNA analysis of parathyroid tumors with special reference to its diagnostic and prognostic value in parathyroid carcinoma. 196 27


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