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)

Medullary thyroid carcinoma, comprising approximately 7% of thyroid carcinoma, produces calcitonin, which can be monitored by immunoassay for diagnosis, at preclinical stages, and for persistent disease and its extent. It presents as sporadic and hereditary forms. The latter consists of the multiple endocrine neoplasia (MEN)-2A syndrome, which includes pheochromocytomas and hyperparathyroidism in some families, and the MEN-2B syndrome, which consistently includes mucosal neuromas and somatic features. The carcinoma, especially the MEN-2B variety, is more aggressive than well-differentiated thyroid carcinoma. After the presence and management of a possible pheochromocytoma is resolved, treatment is by total thyroidectomy, the MEN-2 syndromes always indicating bilateral involvement. Gross evidence of medullary thyroid carcinoma is associated with metastases to regional lymph nodes, justifying removal of lymph nodes in the central neck, anterior superior mediastinum, and lateral neck. At operation, attention is given to preservation of parathyroid glands but also to removal of hyperplastic parathyroid glands; subtotal parathyroidectomy usually is needed if clinical hyperparathyroidism is evident. Diagnosis at the preclinical stage, C-cell hyperplasia, permits total thyroidectomy. Lateral cervical lymph node dissection is determined by biopsy of midjugular lymph nodes. In this situation, serum calcitonin levels are usually normal after operation, indicating cure. However, for palpable medullary thyroid carcinoma, serum calcitonin levels are often elevated after appropriate neck surgery. In this event, scanning techniques are used to monitor patients, and reoperation is performed if localization of medullary thyroid carcinoma is achieved. The mediastinum is particularly observed for recurrence. Reoperation is justified for recurrence in the neck and mediastinum. Early diagnosis and monitoring permits long-term survival. In the future it is anticipated that diagnostic genetic techniques will provide definitive and early diagnosis in the hereditary form, permitting earlier treatment with assurance of cure.
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PMID:Surgical treatment of medullary carcinoma of the thyroid. 197 81

The features of two patients with multiple endocrine neoplasia type IIb are described. Patient 1, a 9-year-old boy with marfanoid features, presented with chronic constipation and failure to thrive since infancy. Patient 2, a 12-year-old boy with marfanoid features, presented with a five-year history of persistent cervical lymphadenopathy. In patient 1, the myenteric and submucosal nerve plexuses at all levels of the small and large intestines were comprised of diffusely disorganized, hyperplastic, mature ganglion cells and nonmyelinated nerve fibers. Nerve plexus dissection with morphometric analysis showed marked thickening of the myenteric plexus with a quantitative increase in neural tissue. Patient 2 had a submucosal neuroma of the tongue. Both patients had occult medullary thyroid carcinoma, and patient 2 had cervical lymph node metastases. Both neoplasms showed positive staining for cytokeratin, carcinoembryonic antigen, calcitonin, bombesin, chromogranin, serotonin, and Leu 7. Electron microscopy showed membrane-bound, intermediate-sized, dense-core neurosecretory granules in tumor cells. In patient 2, calcitonin-positive amyloid was present with localization of calcitonin by immunoelectron microscopy to cytoplasmic secretory granules and to extracellular amyloid fibrils. These cases illustrate the potential for missed or delayed diagnosis in multiple endocrine neoplasia syndromes.
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PMID:Pathological features of multiple endocrine neoplasia type IIb in childhood. 197 36

A 58-year-old male patient with rectal carcinoid tumor is presented. The tumor extensively involved the lymph nodes and liver, and multiple tumors were also recognized in the pancreas and thyroid. Grossly, it was uncertain whether the latter were metastases from the rectal carcinoid or all were coincident primary tumors involving multiple endocrine organs, so-called multiple endocrine neoplasia (MEN) syndrome. Histologic, histochemical and electron microscopic examinations of the tumors in both the pancreas and thyroid showed similar features to those of the rectal carcinoid. The neoplastic cells in all involved organs commonly expressed positive immunoreactivity for somatostatin, but negativity for carcinoembryonic antigen, calcitonin, calcitonin gene-related peptide, thyroglobulin, insulin, glucagon and pancreatic polypeptide. These immunohistochemical results confirmed that the tumors observed in multiple endocrine organs were indeed metastatic from the rectal carcinoid, rather than being a new combination of MEN syndrome. Some neuroendocrine tumors may develop widespread metastasis, sometimes creating problems with differentiation from multiple primary endocrine tumors. Immunohistochemistry may be of great help in setting this issue.
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PMID:Rectal carcinoid tumor metastasizing to the thyroid and pancreas. An autopsy case exploiting immunohistochemistry for differentiation from tumors involving multiple endocrine organs. 197 68

Medullary thyroid carcinoma (MTC) develops in all patients with multiple endocrine neoplasia type IIb (MEN IIb), a rare syndrome that either occurs sporadically or is inherited in an autosomal dominant pattern. The MTC in patients with MEN IIb has been reported to be biologically aggressive with onset at a young age and rapid progression as evidenced by widespread metastases and death, frequently in the teenage years. Seven children, aged 2 to 11 years (mean, 7 years), from three kindreds with MEN IIb were evaluated for evidence of tumor recurrence 3 to 10 years following thyroidectomy. In one child, age 11, a thyroid mass was palpable preoperatively. However, in the remaining six children (aged 2 to 10 years), the diagnosis of MTC was established by an increased concentration of plasma calcitonin (CT), either basally or following pentagastrin (Pg) stimulation. All patients underwent total thyroidectomy with removal of central lymph nodes from the neck. At the time of surgery, six children were found to have bilateral macroscopic MTC, five without and one with cervical metastases. One child (age 2 years) had C-cell hyperplasia, a premalignant precursor of MTC. Currently, five of the seven children are without evidence of recurrent disease clinically and have normal plasma CT levels (less than 0.3 ng/mL) following calcium (Ca) and Pg stimulation 3, 3, 10, 10, and 10 years after thyroidectomy. Two of the seven children have biochemical evidence of residual MTC.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Evaluation of children with multiple endocrine neoplasia type IIb following thyroidectomy. 197 72

The course of the disease in a patient with multiple endocrine neoplasia type IIa over a period of seven years is described. In spite of multiple diagnostic procedures, only a bone marrow biopsy was able to prove metastatic disease. All therapeutic measures, such as combination chemotherapy, sandostatin, bromocriptine, and 131Iodinemethylendiphosphonate-therapy were not effective in reducing tumor load or symptoms.
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PMID:[Metastatic C-cell carcinoma with bone marrow carcinosis in type IIa multiple endocrine neoplasia (MEN). Diagnostic and therapeutic problems]. 197 74

Recent linkage of the gene for multiple endocrine neoplasia type 2A and 2B to the centromeric region of chromosome 10 has provided new insight into the causes of medullary thyroid carcinoma and has provided tools to diagnose gene carriers status for this syndrome with greater than 90% certainty. This review focuses on how these advances influence the clinical management of both sporadic and hereditary medullary thyroid carcinoma and discusses how tests based on the genetic linkage studies will aid in the early diagnosis and treatment of this syndrome. In addition, the authors have focused on several controversial management questions regarding the type and extent of surgery for this thyroid tumor, the management of the patient with metastatic disease, and the approach to management of other manifestations of multiple endocrine neoplasia types 2A and 2B. This review attempts to provide a balanced overview of these complex issues.
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PMID:Changing concepts in the management of hereditary and sporadic medullary thyroid carcinoma. 197 73

In the last 15 years, 29 subjects (13 males, 16 females) aged 8-69 years, belonging to 3 kindreds with multiple endocrine neoplasia type 2 (MEN-2) were studied with rapid i.v. calcium gluconate infusions (2 mg Ca++/kg in 1 minute) in order to detect the presence of medullary thyroid cancer (MTC). Serum samples were obtained at 0, 2, 5, 7 and 10 minutes. Calcitonin was measured by radioimmunoassay using a commercial kit. On the basis of the results obtained in a control group of 28 healthy volunteers, hypercalcitoninemia (HCT) was defined as basal and stimulated values above 100 and 200 pg/ml, respectively, in either sex. In 2 patients with nodular goiter, HCT correctly predicted the malignant nature of the lesions. Out of 22 clinically normal subjects, 3 had HCT: one refused consent for surgery, but in the remaining 2 MTC was found, without extraglandular extension. Seven patients with surgically proven MTC have been followed; 4 have shown HCT: 3 women refused permission for exploratory surgery, and a 46 year old male with enlarged lymph nodes in the neck was found to have metastatic papillary thyroid cancer. Both calcitonin and thyroglobulin were immunohistochemically discovered in metastases and the original thyroid tumor (differentiated thyroid cancer, intermediate type). There were 2 false negative tests in patients with metastatic disease. This type of biochemical screening in members of MEN-2 kindreds allows early detection of MTC and its prevention through thyroidectomy in a preneoplastic stage (C-cell hyperplasia).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Multiple endocrine type 2 neoplasia in 3 families]. 198 20

Of 44 patients with the Zollinger-Ellison syndrome treated at our institution, nine appeared to have undergone "regression" of their gastrinomas. Six of the nine patients had sporadic gastrinomas and became permanently eugastrinemic following excision of nodal metastases and total gastrectomy (n = 4), antrectomy (n = 1), or pancreatoduodenectomy (n = 1) (mean survival, 13 years). The other three patients had Zollinger-Ellison syndrome as part of the multiple endocrine adenopathy type 1 syndrome and became temporarily eugastrinemic after total gastrectomy (mean survival, 11 years). Occult submucosal duodenal-wall microgastrinomas (mean size, 3.0 mm) were found to have been serendipitously excised in four patients. Long-term follow-up of these nine patients, as well as of six other patients described in the literature, demonstrates that excision of occult duodenal-wall gastrinomas provides a plausible explanation for the phenomenon of apparent regression of primary gastrinomas and the eugastrinemia that may follow total gastrectomy.
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PMID:Zollinger-Ellison syndrome. A new look at regression of gastrinomas. 202 33

This conference focuses on the controversies about managing thyroid cancer, emphasizing the possibility that the treatment of patients with potentially fatal thyroid cancer may be improved. Although the mortality rate from thyroid cancer is low, it is the highest among cancers affecting the endocrine glands (excluding the ovary). Exposure to radiation during childhood in the 1930s and 1940s increased the incidence of but not the mortality from thyroid cancer, because these tumors are mainly papillary cancers developing in young adults. These rates may change as the exposed cohort ages. Risk factors that increase mortality include older patient age and the growth characteristics of the tumor at diagnosis, the presence of distant metastases, and cell type (for example, the tall-cell variants of papillary cancer, follicular cancer [to be distinguished from the more benign follicular variant of papillary cancer], medullary cancer, and anaplastic cancer). Local metastases in lymph nodes do not seem to increase the risk for death from papillary cancer, but they do increase the risk for death from follicular and medullary cancer. In the latter, mortality is decreased by the early detection and treatment of patients with the familial multiple endocrine neoplasia syndrome 2a. There are excellent tumor markers for differentiated cancer of the parafollicular and of the follicular cells (serum calcitonin and serum thyroglobulin levels, respectively). Measuring the calcitonin level allows early diagnosis of familial medullary cancer, whereas measuring the thyroglobulin level, although useful only after total thyroidectomy, allows early recognition of recurrence or metastases of papillary or follicular cancer. Initial surgery, protocols for follow-up, and the use of radioiodine for the ablation of any residual thyroid and the treatment of metastatic cancer are discussed. Because these tumors resist currently available chemotherapy regimens, possible ways to increase the effectiveness of radioiodine therapy are considered as are new approaches to treatment.
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PMID:Thyroid cancer: a lethal endocrine neoplasm. 205 61

The measurement of plasma levels of human pancreatic polypeptide (hPP) has been reported to be clinically useful in predicting the existence of pancreatic islet cell neoplasms in patients with familial multiple endocrine neoplasia type 1 (FMEN-I) and the possible presence of metastatic disease in patients with islet cell tumors. However, these studies have not been prospective and involve small numbers of patients. In this study, fasting plasma samples from 36 patients with biopsy-proved islet cell tumors were analyzed for hPP by radioimmunoassay and compared with age-matched control subjects. Of 13 patients with FMEN-I who had islet cell tumors, 7 (54%) had elevated plasma hPP levels before surgery. After resection of all islet cell tumors, 4 of 12 patients evaluated after surgery still had elevated levels. Fifteen patients had islet cell tumors that were localized (seven insulinomas and eight gastrinomas), but none of these patients had elevated hPP levels, either before or after surgery. Nine patients, including one with FMEN-I, with metastatic islet cell tumors to the liver were studied; three with more advanced disease had elevated hPP levels before surgery. Each of the nine patients underwent resection of all gross disease and the three patients with elevated preoperative levels had normal postoperative hPP levels. Our results indicate that basal plasma levels of hPP were not clinically useful. The hPP levels did not reliably predict the presence of islet cell tumors in patients with FMEN-I, because 46% of patients with tumors did not have elevated plasma levels, and in those with elevated values hPP levels did not reliably predict the resection of all tumor. Plasma levels of hPP have no utility in patients with localized sporadically occurring islet cell tumors and limited utility (33%) in predicting the presence of metastatic islet cell tumors to the liver.
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PMID:The utility of circulating levels of human pancreatic polypeptide as a marker for islet cell tumors. 217 93


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