The MEN syndromes continue to be the focus of considerable interest and research. Since successful treatment requires early diagnosis, proper screening and follow-up of patients at risk is important. In the individual at risk for developing MEN IIa, annual screening should include measurement of the basal and stimulated plasma CT levels, and determination of plasma levels of calcium, PTH, and CEA. Twenty-four hour urine excretion rates of norepinephrine, epinephrine, metanephrine, dopamine, and VMA should also be obtained. It is our recommendation that this screening be continued through the third decade of life. Patients having thyroidectomy for MTC need to be tested annually for recurrent MTC and the development of adrenal medullary disease. All patients at risk for developing MEN IIb should be evaluated in a similar fashion. Recently, several groups using DNA linkage analysis have mapped the gene for MEN IIa to chromosome 10, although the exact location of the gene is yet to be determined. Preliminary linkage studies have mapped the gene for MEN I to chromosome 11. The identification of the genes for MEN I and MEN II will greatly simplify the diagnosis of the disease and perhaps also the therapy of affected patients.
Inherited medullary thyroid carcinoma: a final monoclonal mutation in one of multiple clones of susceptible cells
Management of occult medullary thyroid carcinoma: evidenced only by serum calcitonin level elevations after apparently adequate neck operations
Prognostic factors in medullary thyroid carcinomas. Survival in relation to age, sex, stage, histology, immunocytochemistry, and DNA content
Medullary thyroid carcinoma: prognosis of familial versus sporadic disease and the role of radiotherapy
Pathophysiological study of diarrhoea in a patient with medullary thyroid carcinoma. Evidence against a secretory mechanism and for the role of shortened colonic transit time
Occult thyroid pathology in the young adult: an autopsy study of 138 patients without clinical thyroid disease
The cytochemistry and ultrastructure of polypeptide hormone-producing cells of the APUD series and the embryologic, physiologic and pathologic implications of the concept
Study of a kindred with pheochromocytoma, medullary thyroid carcinoma, hyperparathyroidism and Cushing's disease: multiple endocrine neoplasia, type 2
A comparison of pentagastrin injection and calcium infusion as provocative agents for the detection of medullary carcinoma of the thyroid
Multiple mucosal neuromata with endocrine tumours: a syndrome allied to von Recklinghausen's disease
Syndrome of bilateral pheochromocytoma, medullary thyroid carcinoma and multiple neuromas. A possible regulatory defect in the differentiation of chromaffin tissue
Serum calcitonin after pentagastrin stimulation in patients with bronchogenic and breast cancer compared to that in patients with medullary thyroid carcinoma
The prognostic and biological significance of cellular heterogeneity in medullary thyroid carcinoma: a study of calcitonin, L-dopa decarboxylase, and histaminase
Familial endocrine tumors; report of two unrelated kindred affected with pheochromocytomas, one also with multiple thyroid carcinomas
Oncogenic RET receptors display different autophosphorylation sites and substrate binding specificities.
Autoimmune Polyendocrine Syndromes
This feed focuses on a rare genetic condition called Autoimmune Polyendocrine Syndromes, which are characterized by autoantibodies against multiple endocrine organs. This can lead to Type I Diabetes.