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Descriptor English: Multiple Endocrine Neoplasia Type 2b
Descriptor Spanish: Neoplasia Endocrina Múltiple Tipo 2b
Descriptor neoplasia endocrina múltiple tipo 2b
Entry term(s) NEM 2b
NEM 3
NEM tipo 2b
Scope note: Tipo de neoplasia endocrina múltiple que se presenta como forma congénita aislada o como una enfermedad autosómica dominante definida. Se caracteriza por una incidencia del 100 por ciento de carcinoma medular del tiroides y feocromocitomas frecuentes; los pacientes presentan hiperparatiroidismo con poca frecuencia. Se distingue del MEN 2a por la apariencia física característica resultante de numerosas lesiones neurales como neuromas mucosos en los párpados, labios y lengua. Las anomalías neurales incluyen también neurogangliomatosis diseminada del tubo digestivo que produce alteraciones de la motilidad. El tratamiento suele requerir la tiroidectomía total tras la evaluación de la presencia de feocromocitomas.
Descriptor Portuguese: Neoplasia Endócrina Múltipla Tipo 2b
Descriptor French: Néoplasie endocrinienne multiple de type 2b
Entry term(s): MEA 2b
MEA IIb
MEN 2b
MEN 3
MEN III
MEN IIb
MEN2b
Mucosal Neuroma Syndrome
Mucosal Neuroma Syndromes
Multiple Endocrine Neoplasia, Type 2b
Multiple Endocrine Neoplasia, Type IIb
Multiple Endocrine Neoplasms Type 2b
Neoplasia, Multiple Endocrine Type 2b
Neoplasms, Multiple Endocrine Type 2b
Neuroma Syndrome, Mucosal
Neuromata, Mucosal, With Endocrine Tumors
Syndrome, Wagenmann-Froboese
Wagenmann Froboese Syndrome
Wagenmann-Froboese Syndrome
Tree number(s): C04.588.322.400.510
C04.651.600.510
C04.700.630.510
C16.320.700.630.510
C19.344.400.510
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D018814
Scope note: Similar to MEN2A, it is also caused by mutations of the MEN2 gene, also known as the RET proto-oncogene. Its clinical symptoms include medullary carcinoma (CARCINOMA, MEDULLARY) of THYROID GLAND and PHEOCHROMOCYTOMA of ADRENAL MEDULLA (50%). Unlike MEN2a, MEN2b does not involve PARATHYROID NEOPLASMS. It can be distinguished from MEN2A by its neural abnormalities such as mucosal NEUROMAS on EYELIDS; LIP; and TONGUE, and ganglioneuromatosis of GASTROINTESTINAL TRACT leading to MEGACOLON. 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 (1975-1994)
Public MeSH Note: 1995; MEA III & MEN III see NEOPLASMS, MULTIPLE ENDOCRINE 1983-1994
History Note: 1995; MEA III & MEN III see NEOPLASMS, MULTIPLE ENDOCRINE 1983-1994
Entry Version: MULTIPLE ENDOCRINE NEOPL TYPE 2B
DeCS ID: 32132
Unique ID: D018814
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 2b - Preferred
Concept UI M0028167
Scope note Similar to MEN2A, it is also caused by mutations of the MEN2 gene, also known as the RET proto-oncogene. Its clinical symptoms include medullary carcinoma (CARCINOMA, MEDULLARY) of THYROID GLAND and PHEOCHROMOCYTOMA of ADRENAL MEDULLA (50%). Unlike MEN2a, MEN2b does not involve PARATHYROID NEOPLASMS. It can be distinguished from MEN2A by its neural abnormalities such as mucosal NEUROMAS on EYELIDS; LIP; and TONGUE, and ganglioneuromatosis of GASTROINTESTINAL TRACT leading to MEGACOLON. It is an autosomal dominant inherited disease.
Preferred term Multiple Endocrine Neoplasia Type 2b
Entry term(s) MEA 2b
MEA IIb
MEN 2b
MEN 3
MEN III
MEN IIb
MEN2b
Mucosal Neuroma Syndrome
Mucosal Neuroma Syndromes
Multiple Endocrine Neoplasia, Type 2b
Multiple Endocrine Neoplasia, Type IIb
Multiple Endocrine Neoplasms Type 2b
Neoplasia, Multiple Endocrine Type 2b
Neoplasms, Multiple Endocrine Type 2b
Neuroma Syndrome, Mucosal
Neuromata, Mucosal, With Endocrine Tumors
Syndrome, Wagenmann-Froboese
Wagenmann Froboese Syndrome
Wagenmann-Froboese Syndrome



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