Multiple endocrine neoplasia type 2: clinical manifestations and management. 2010

Amber L Traugott, and Jeffrey F Moley

UI MeSH Term Description Entries
D010673 Pheochromocytoma A usually benign, well-encapsulated, lobular, vascular tumor of chromaffin tissue of the ADRENAL MEDULLA or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of EPINEPHRINE and NOREPINEPHRINE, is HYPERTENSION, which may be persistent or intermittent. During severe attacks, there may be HEADACHE; SWEATING, palpitation, apprehension, TREMOR; PALLOR or FLUSHING of the face, NAUSEA and VOMITING, pain in the CHEST and ABDOMEN, and paresthesias of the extremities. The incidence of malignancy is as low as 5% but the pathologic distinction between benign and malignant pheochromocytomas is not clear. (Dorland, 27th ed; DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1298) Pheochromocytoma, Extra-Adrenal,Extra-Adrenal Pheochromocytoma,Extra-Adrenal Pheochromocytomas,Pheochromocytoma, Extra Adrenal,Pheochromocytomas,Pheochromocytomas, Extra-Adrenal
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000310 Adrenal Gland Neoplasms Tumors or cancer of the ADRENAL GLANDS. Adrenal Cancer,Adrenal Gland Cancer,Adrenal Neoplasm,Cancer of the Adrenal Gland,Neoplasms, Adrenal Gland,Adrenal Cancers,Adrenal Gland Cancers,Adrenal Gland Neoplasm,Adrenal Neoplasms,Cancer, Adrenal,Cancer, Adrenal Gland,Cancers, Adrenal,Cancers, Adrenal Gland,Neoplasm, Adrenal,Neoplasm, Adrenal Gland,Neoplasms, Adrenal
D013964 Thyroid Neoplasms Tumors or cancer of the THYROID GLAND. Cancer of Thyroid,Thyroid Cancer,Cancer of the Thyroid,Neoplasms, Thyroid,Thyroid Adenoma,Thyroid Carcinoma,Adenoma, Thyroid,Adenomas, Thyroid,Cancer, Thyroid,Cancers, Thyroid,Carcinoma, Thyroid,Carcinomas, Thyroid,Neoplasm, Thyroid,Thyroid Adenomas,Thyroid Cancers,Thyroid Carcinomas,Thyroid Neoplasm
D051096 Proto-Oncogene Proteins c-ret Receptor protein-tyrosine kinases involved in the signaling of GLIAL CELL-LINE DERIVED NEUROTROPHIC FACTOR ligands. They contain an extracellular cadherin domain and form a receptor complexes with GDNF RECEPTORS. Mutations in ret protein are responsible for HIRSCHSPRUNG DISEASE and MULTIPLE ENDOCRINE NEOPLASIA TYPE 2. c-ret Protein,ret Proto-Oncogene Proteins,Proto-Oncogene Protein Ret,Proto-Oncogene Protein c-ret,Receptor Tyrosine Kinase RET,Proto Oncogene Protein Ret,Proto Oncogene Protein c ret,Proto Oncogene Proteins c ret,Proto-Oncogene Proteins, ret,Ret, Proto-Oncogene Protein,c-ret, Proto-Oncogene Protein,c-ret, Proto-Oncogene Proteins,ret Proto Oncogene Proteins
D018276 Carcinoma, Medullary A carcinoma composed mainly of epithelial elements with little or no stroma. Medullary carcinomas of the breast constitute 5%-7% of all mammary carcinomas; medullary carcinomas of the thyroid comprise 3%-10% of all thyroid malignancies. (From Dorland, 27th ed; DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1141; Segen, Dictionary of Modern Medicine, 1992) Carcinomas, Medullary,Medullary Carcinoma,Medullary Carcinomas
D018813 Multiple Endocrine Neoplasia Type 2a A form of multiple endocrine neoplasia characterized by the presence of medullary carcinoma (CARCINOMA, MEDULLARY) of the THYROID GLAND, and usually with the co-occurrence of PHEOCHROMOCYTOMA, producing CALCITONIN and ADRENALINE, respectively. Less frequently, it can occur with hyperplasia or adenoma of the PARATHYROID GLANDS. This disease is due to gain-of-function mutations of the MEN2 gene on CHROMOSOME 10 (Locus: 10q11.2), also known as the RET proto-oncogene that encodes a RECEPTOR PROTEIN-TYROSINE KINASE. It is an autosomal dominant inherited disease. MEN 2,MEN 2a,Neoplasia, Multiple Endocrine Type 2a,Neoplasms, Multiple Endocrine Type 2a,Sipple Syndrome,MEA 2a,MEA II,MEA IIa,MEN II,MEN IIa,MEN-2A Syndrome,MEN2a,Multiple Endocrine Neoplasia Type 2,Multiple Endocrine Neoplasia, Type IIa,Multiple Endocrine Neoplasms Type 2a,Pheochromocytoma And Amyloid-Producing Medullary Thyroid Carcinoma,MEN 2A Syndrome,MEN-2A Syndromes,Pheochromocytoma And Amyloid Producing Medullary Thyroid Carcinoma

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