II. Associated Conditions

  1. Multiple Endocrine Neoplasia Type 2
    1. Thyroid Adenoma or Thyroid Cancer
    2. Adrenal adenoma
    3. Parathyroid carcinoma
  2. Multiple Endocrine Neoplasia Type 2-A
    1. Medullary Thyroid Carcinoma
    2. Pheochromocytoma
    3. Parathyroid tumor

III. Prevention: Screening in family members

  1. Identify specific gene mutation in known positive family member with MEN II
  2. Screen family members for the specific gene mutation

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Ontology: Multiple Endocrine Neoplasia Type 2a (C0025268)

Definition (MSHCZE) MEN 2 – syndrom mnohočetné endokrinní neoplazie, jehož základní charakteristikou je medulární karcinom štítné žlázy. U MEN 2A (Sippleův syndrom) je přítomna primární hyperparatyreóza (klinicky obv. nepříliš výrazná) a méně často feochromocytom. Syndrom je autozomálně dominantně dědičný, postižena je oblast 10. chromozomu (RET protoonkogen) s částečnými odlišnostmi u varianty A a B. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI) Multiple endocrine neoplasia caused by mutation of the RET gene. Patients develop medullary thyroid carcinomas, and may also develop pheochromocytomas and parathyroid gland hyperplasia.
Definition (MSH) 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.
Concepts Neoplastic Process (T191)
MSH D018813
ICD9 258.02
ICD10 E31.22
SnomedCT 61808009
English MEA II, MEN II, Sipple Syndrome, MEA 2a, MEA IIa, MEN 2, MEN 2a, MEN IIa, MEN2a, Multiple Endocrine Neoplasia Type 2a, Multiple Endocrine Neoplasms Type 2a, Neoplasia, Multiple Endocrine Type 2a, Neoplasms, Multiple Endocrine Type 2a, MEN syndrome type 2A, Multiple endoc neoplas type 2A, MEA Type 2a, MEN Type 2a, MULTIPLE ENDOCRINE NEOPLASIA, TYPE IIA, MEN2A, PHEOCHROMOCYTOMA AND AMYLOID-PRODUCING MEDULLARY THYROID CARCINOMA, SIPPLE SYNDROME, PTC SYNDROME, MULTIPLE ENDOCRINE NEOPL TYPE 2A, MEN 002 A, NEOPLASIA MULTIPLE ENDOCRINE TYPE 002 A, NEOPLASMS MULTIPLE ENDOCRINE TYPE 002 A, MEN 002, NEOPL MULTIPLE ENDOCRINE TYPE 2A, type II multiple endocrine neoplasia, multiple endocrine neoplasia, type 2 (diagnosis), multiple endocrine neoplasia, type 2, type II multiple endocrine neoplasia (diagnosis), type IIa multiple endocrine neoplasia (diagnosis), type IIa multiple endocrine neoplasia, Multiple Endocrine Neoplasia Type 2A, MEN 2A, Multiple Endocrine Neoplasia, Type II, Mult endo neop type IIA, Multiple endocrine neoplasia [MEN] type IIA, Multiple Endocrine Neoplasia Type 2a [Disease/Finding], MEN 2A Syndrome, MEN-2A Syndromes, MEN-2A Syndrome, sipple syndrome, multiple endocrine neoplasia type 2A (MEN 2A), men type 2, men type ii, sipple's syndrome, Pheochromocytoma And Amyloid Producing Medullary Thyroid Carcinoma, Multiple Endocrine Neoplasia, Type IIa, Pheochromocytoma And Amyloid-Producing Medullary Thyroid Carcinoma, Multiple endocrine neoplasia Type 2, MEA Type II, Multiple endocrine adenomatosis Type II, MEN Type II, Multiple endocrine neoplasia Type IIA, Multiple endocrine adenomatosis Type IIa, Multiple endocrine neoplasia Type 2A, Multiple endocrine neoplasia Type II, Sipple's syndrome, Familial chromaffinomatosis, MEA, type 2, MEN, type 2, Multiple endocrine adenomatosis, type 2, Multiple endocrine neoplasia, type 2, PTC syndrome, Sipple syndrome, MEN 2A - Multiple endocrine neoplasia syndrome type 2A, MEN 2A syndrome, Multiple endocrine neoplasia syndrome type 2A, Multiple endocrine neoplasia, type 2 (disorder), Multiple Endocrine Adenomatosis Type 2A, Multiple Endocrine Adenomatosis Type 2a, Multiple Endocrine Adenomatosis Type II, Multiple Endocrine Adenomatosis, Type II, Multiple Endocrine Neoplasia Type II, Multiple Endocrine Neoplasia Type 2
Swedish Multipla hormonella tumörer typ 2a
Finnish MEN 2A -oireyhtymä
French Apudomatose de type IIa, Adénomatose endocrine multiple type II, Tumeur endocrine multiple de type IIA, Tumeur endocrine multiple de type 2, Tumeur endocrine multiple de type 2A, Adénomatose polyendocrinienne de type II, MEN (adénomatose polyendocrinienne) de type II, AEM de type II, Polyadénomatose endocrinienne type IIa, Syndrome de Sipple, Néoplasie endocrinienne multiple de type 2a, Polyadénomatose endocrinienne de type 2A, Adénomatose endocrinienne multiple de type 2a, NEM 2a, NEM IIa, NEM2a
Russian MEN 2A, ENDOKRINNAIA NEOPLAZIIA MNOZHESTVENNAIA TIPA 2A, SIPPLA SINDROM, MEN 2, NOVOOBRAZOVANIIA ENDOKRINNYE MNOZHESTVENNYE TIPA 2A, ENDOKRINNYKH ZHELEZ NOVOOBRAZOVANIIA TIPA 2A MNOZHESTVENNYE, NEOPLAZIIA ENDOKRINNAIA MNOZHESTVENNAIA TIPA 2A, МЭН 2A, МЭН 2, НЕОПЛАЗИЯ ЭНДОКРИННАЯ МНОЖЕСТВЕННАЯ ТИПА 2A, НОВООБРАЗОВАНИЯ ЭНДОКРИННЫЕ МНОЖЕСТВЕННЫЕ ТИПА 2A, СИППЛА СИНДРОМ, ЭНДОКРИННАЯ НЕОПЛАЗИЯ МНОЖЕСТВЕННАЯ ТИПА 2A, ЭНДОКРИННЫХ ЖЕЛЕЗ НОВООБРАЗОВАНИЯ ТИПА 2A МНОЖЕСТВЕННЫЕ
German MEN 002, MEN 002 A, TUMOREN MULTIPLE ENDOKRINE TYP 002 A, NEOPLASIE MULTIPLE ENDOKRINE TYP 002 A, multiple endokrine Neoplasie Typ II, multiple endokrine Adenomatose Typ IIa, MEN Typ II, MEA Typ II, Multiple endokrine Neoplasie Typ 2A, Multiple endokrine Neoplasie Typ IIA, multiple endokrine Adenomatose Typ II, Multiple endokrine Neoplasie Typ 2, MEN 2a, MEN 2, Multiple endokrine Neoplasie Typ 2a, Neoplasie, multiple endokrine, Typ 2a, Sipple-Syndrom, Tumoren, multiple endokrine, Typ 2a
Polish MEN 2, MEN 2a, Gruczolakowatość wewnątrzwydzielnicza typu 2a
Japanese MEN2A型, 多発性内分泌腫瘍2型, タハツセイナイブンピセンシュショウ2Aガタ, 多発性内分泌腺腫症2型, タハツセイナイブンピツセンシュショウ2ガタ, 多発性内分泌腺腫症2A型, タハツセイナイブンピツセンシュショウ2Aガタ, タハツセイナイブンピセンシュショウ2ガタ, タハツセイナイブンピツシュショウ2ガタ, 多発性内分泌腫症2型, タハツセイナイブンピシュショウ2ガタ, Sipple症候群, シップル症候群, 多内分泌腫瘍2A, 多内分泌腫瘍2, 多発性内分泌腫瘍2A型, 多発性内分泌腺腫症2A, 多発性内分泌腺腫症2, 腫瘍-多発性内分泌2A型
Norwegian Sipples syndrom, Medullært tyreoidakarsinom med feokromocytom og paratyreoideasvulster, Medullært thyreoidakarsinom med feokromocytom og parathyreoideasvulster, MEN 2, Multippel endokrin neoplasi type 2a
Czech Mnohočetná endokrinní neoplazie typ 2, Mnohočetná endokrinní adenomatóza Typ IIa, MEN Typ II, Mnohočetná endokrinní adenomatóza Typ II, MEA Typ II, Mnohočetná endokrinní neoplazie typ IIA, Mnohočetná endokrinní neoplazie typ 2A, Mnohočetná endokrinní neoplazie Typ II, Sippleův syndrom, nádory mnohočetné endokrinní typ 2a, mnohočetná endokrinní neoplazie typ 2A, MEN 2A, MEN 2, mnohočetná endokrinní neoplazie typu 2A, syndrom mnohočetné endokrinní neoplazie MEN-2A
Dutch MEN type II, multipele endocriene neoplasie type 2A, multipele endocriene neoplasie type II, multipele endocriene adenomatosis type IIa, multipele endocriene adenomatose type II, MEA type II, multipele endocriene neoplasie type 2, multipele endocriene neoplasie type IIA, MEN 2, MEN 2a, Multipele-endocriene-neoplasiesyndroom type 2a, Neoplasie type 2a, multipele endocriene, Neoplasmata type 2a, multipele endocriene, Sipple-syndroom, Syndroom type 2a, Multipele-endocriene-neoplasie-
Hungarian MEA típus II, Többszörös endocrin neoplasia IIA. típus, Többszörös 2A. típusú endocrin neoplasia, Ember típus II, Multiplex endocrin adenomatosis II. típus, Többszörös 2. típusú endocrin neoplasia, Többszörös endocrin adenomatosis típus iia, Többszörös endocrin neoplasia típus II
Italian Neoplasia endocrina multipla di tipo 2A, Adenomatosi endocrina multipla tipo IIa, Adenomatosi endocrina multipla tipo II, Neoplasia endocrina multipla tipo II, Neoplasia endocrina multipla di tipo 2, Neoplasia endocrina multipla di tipo IIA, Neoplasia endocrina multipla tipo 2
Portuguese Neoplasia endócrina múltipla tipo IIA, Adenomatose endócrina múltipla tipo II, Neoplasia endócrina múltipla tipo 2, Adenomatose endócrina múltipla tipo IIa, Neoplasia endócrina múltipla tipo II, Neoplasia endócrina múltipla tipo 2A, MEN 2, MEN 2a, Neoplasia Endócrina Múltipla Tipo 2a, Síndrome de Sipple, Neoplasias Endócrinas Múltiplas Tipo 2a
Spanish Neoplasia endocrina múltiple tipo 2A, Neoplasia endocrina múltiple de tipo II, Neoplasia endocrina múltiple tipo 2, Adenomatosis endocrina múltiple tipo IIa, Neoplasia endocrina múltiple de tipo IIA, AEM de tipo II, Adenomatosis endocrina múltiple de tipo II, NEM de tipo II, NEM, tipo 2, adenomatosis endocrina múltiple, tipo 2, cromafinomatosis familiar, neoplasia endocrina múltiple, tipo 2 (trastorno), neoplasia endocrina múltiple, tipo 2, síndrome de PTC, síndrome de Sipple, MEN 2, MEN 2a, Neoplasia Endocrina Múltiple Tipo 2a, Neoplasia Múltiple Endocrina Tipo 2a, Síndrome de Sipple, Neoplasias Múltiples Endocrinas Tipo 2a