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Descriptor English: Multiple Endocrine Neoplasia Type 2a
Descriptor Spanish: Neoplasia Endocrina Múltiple Tipo 2a
Descriptor neoplasia endocrina múltiple tipo 2a
Entry term(s) MEN 2
MEN 2a
NEM 2a
NEM tipo 2a
síndrome de Sipple
Scope note: Tipo de neoplasia endocrina múltiple caracterizada por una incidencia prácticamente en el 100 por ciento de los casos de carcinoma medular del tiroides, una incidencia del 50 por ciento de feocromocitoma, y una menor incidencia de adenomas paratiroideos asociados con hiperparatiroidismo. La afección se transmite siempre con carácter autosómico dominante. El estudio genético puede identificar desde la lactancia a los individuos con el rasgo. El tratamiento suele ser la extirpación de las glándulas paratiroideas aumentadas de volumen. (Holland et al., Cancer Medicine, 3d ed, pp1172-75)
Descriptor Portuguese: Neoplasia Endócrina Múltipla Tipo 2a
Descriptor French: Néoplasie endocrinienne multiple de type 2a
Entry term(s): MEA 2a
MEA II
MEA IIa
MEN 2
MEN 2A Syndrome
MEN 2a
MEN II
MEN IIa
MEN-2A Syndrome
MEN-2A Syndromes
MEN2a
Multiple Endocrine Neoplasia Type 2
Multiple Endocrine Neoplasia, Type IIa
Multiple Endocrine Neoplasms Type 2a
Neoplasia, Multiple Endocrine Type 2a
Neoplasms, Multiple Endocrine Type 2a
Pheochromocytoma And Amyloid Producing Medullary Thyroid Carcinoma
Pheochromocytoma And Amyloid-Producing Medullary Thyroid Carcinoma
Sipple Syndrome
Tree number(s): C04.588.322.400.505
C04.651.600.505
C04.700.630.505
C16.320.700.630.505
C19.344.400.505
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D018813
Scope note: 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.
Annotation: coordinate IM with specific endocrine/neoplasm pre-coordinates (IM) + specific histological type (IM) if pertinent
Allowable Qualifiers: BL blood
BS blood supply
CF cerebrospinal fluid
CH chemistry
CI chemically induced
CL classification
CN congenital
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SC secondary
SU surgery
TH therapy
UL ultrastructure
UR urine
VE veterinary
VI virology
Previous Indexing: Multiple Endocrine Neoplasia (1966-1994)
Public MeSH Note: 95; MEA II, MEN II, & SIPPLE SYNDROME were see NEOPLASMS, MULTIPLE ENDOCRINE 1983-94
Online Note: use MULTIPLE ENDOCRINE NEOPLASIA to search MEA II, MEN II, & SIPPLE SYNDROME 1983-94
History Note: 95; MEA II, MEN II, & SIPPLE SYNDROME were see NEOPLASMS, MULTIPLE ENDOCRINE 1983-94
Entry Version: MULTIPLE ENDOCRINE NEOPL TYPE 2A
DeCS ID: 32133
Unique ID: D018813
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1995/01/01
Date of Entry: 1994/05/23
Revision Date: 2012/07/03
Multiple Endocrine Neoplasia Type 2a - Preferred
Concept UI M0028166
Scope note 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.
Preferred term Multiple Endocrine Neoplasia Type 2a
Entry term(s) MEA 2a
MEA II
MEA IIa
MEN 2
MEN 2A Syndrome
MEN 2a
MEN II
MEN IIa
MEN-2A Syndrome
MEN-2A Syndromes
MEN2a
Multiple Endocrine Neoplasia Type 2
Multiple Endocrine Neoplasia, Type IIa
Multiple Endocrine Neoplasms Type 2a
Neoplasia, Multiple Endocrine Type 2a
Neoplasms, Multiple Endocrine Type 2a
Pheochromocytoma And Amyloid Producing Medullary Thyroid Carcinoma
Pheochromocytoma And Amyloid-Producing Medullary Thyroid Carcinoma
Sipple Syndrome



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