II. Associated Conditions

  1. Familial Primary Hyperparathyroidism (occurs in >80% of MEN I cases)
  2. Pituitary Adenoma
  3. Pancreatic islet tumors
  4. Zollinger-Ellison Syndrome
    1. Hypergastrinemia
    2. Peptic Ulcer Disease

III. Prevention: Family Members of MEN I patients

  1. Consider annual lab screening starting at age 8 years
    1. Serum Calcium annually
    2. Consider PTH Level annually or if Serum Calcium is abnormal
    3. Malone (2004) Otolaryngol Clin North Am 37(4): 715-36 [PubMed]

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Ontology: Multiple Endocrine Neoplasia Type 1 (C0025267)

Definition (MSHCZE) Wermerův syndrom – syndrom mnohočetné endokrinní neoplazie (MEN 1) charakterizovaný primární hyperparatyreózou, tumory endokrinního pankreatu a hypofýzy. Dědičnost je autozomálně dominantní, postižený gen (tumor-supresorový) byl lokalizován na 11. chromozomu. Klinický obraz je dán příslušným postižením, obv. hyperfunkcí, hyperparatyreóza je nejčastější, ale klinicky může být méně nápadná. Nádory v oblasti trávicího traktu mohou být někdy maligní a mnohočetné. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI_NCI-GLOSS) A rare, inherited disorder that affects the endocrine glands and can cause tumors in the parathyroid and pituitary glands and the pancreas. These tumors are usually benign (not cancer). They cause the glands to secrete high levels of hormones, which can lead to other medical problems, such as kidney stones, fertility problems, and severe ulcers. In some cases, tumors inside the pancreas can become malignant (cancer).
Definition (NCI) Multiple endocrine neoplasia caused by inactivation of the tumor suppressor gene MEN-1. Patients may develop hyperparathyroidism and parathyroid gland adenomas, pituitary gland adenomas, pancreatic islet cell neoplasms, and carcinoid tumors.
Definition (MSH) A form of multiple endocrine neoplasia that is characterized by the combined occurrence of tumors in the PARATHYROID GLANDS, the PITUITARY GLAND, and the PANCREATIC ISLETS. The resulting clinical signs include HYPERPARATHYROIDISM; HYPERCALCEMIA; HYPERPROLACTINEMIA; CUSHING DISEASE; GASTRINOMA; and ZOLLINGER-ELLISON SYNDROME. This disease is due to loss-of-function of the MEN1 gene, a tumor suppressor gene (GENES, TUMOR SUPPRESSOR) on CHROMOSOME 11 (Locus: 11q13).
Concepts Neoplastic Process (T191)
MSH D018761
ICD9 258.01
ICD10 E31.21
SnomedCT 190566000, 30664006
English MEA I, MEN I, Wermer Syndrome, MEA 1, MEN 1, MEN1, Multiple Endocrine Neoplasia Type 1, Multiple Endocrine Neoplasia Type I, Multiple Endocrine Neoplasms Type 1, Neoplasia, Multiple Endocrine Type 1, Neoplasms, Multiple Endocrine Type 1, Neoplasms, Multiple Endocrine Type I, MEN syndrome type 1, Multiple endoc neoplas type 1, MEA Type 1, MEN Type 1, Wermer's Syndrome, MULTIPLE ENDOCRINE NEOPLASIA, TYPE I, ENDOCRINE ADENOMATOSIS, MULTIPLE, WERMER SYNDROME, NEOPL MULTIPLE ENDOCRINE TYPE 1, MEN 001, NEOPLASIA MULTIPLE ENDOCRINE TYPE 001, NEOPLASMS MULTIPLE ENDOCRINE TYPE 001, MULTIPLE ENDOCRINE NEOPL TYPE 1, type I multiple endocrine neoplasia (diagnosis), type I multiple endocrine neoplasia, Mult endo neoplas type I, Wermer's syndrome, Multiple endocrine neoplasia [MEN] type I, Multiple Endocrine Neoplasia Type 1 [Disease/Finding], wermer syndrome, men 1 syndrome, wermer's syndrome, men type 1, multiple endocrine neoplasia type 1 (MEN1), men type i, MEN Type I, Multiple endocrine neoplasia Type 1, Multiple endocrine adenomatosis Type I, MEA Type I, Multiple endocrine neoplasia Type I, multiple endocrine neoplasia type 1 syndrome, MEN1 syndrome, MEA, type 1, MEN, type 1, Multiple endocrine adenomatosis, type 1, Multiple endocrine neoplasia, type 1, Wermer syndrome, MEN 1 - Multiple endocrine neoplasia syndrome type 1, MEN 1 syndrome, Multiple endocrine neoplasia syndrome type 1, Multiple endocrine neoplasia, type 1 (disorder), Multiple Endocrine Adenomatosis Type 1, Multiple Endocrine Adenomatosis Type I, Multiple Endocrine Adenomatosis, Type I, Multiple Endocrine Neoplasia, Type I
Swedish Multipla hormonella tumörer typ 1
Finnish Multippeli endokriininen neoplasia tyyppi 1
French Apudomatose de type I, Adénomatose polyendocrinienne de type I, AEM de type I, Adénomatose endocrine multiple type I, Tumeur endocrine multiple de type 1, Syndrome de Wermer, Néoplasie endocrinienne multiple de type 1, Polyadénomatose endocrinienne de type 1, Adénomatose endocrinienne multiple de type 1, NEM 1, NEM I, NEM1
Russian NOVOOBRAZOVANIIA ENDOKRINNYE MNOZHESTVENNYE TIPA 1, VERMERA SINDROM, ENDOKRINNAIA NEOPLAZIIA MNOZHESTVENNAIA TIPA 1, NEOPLAZIIA ENDOKRINNAIA MNOZHESTVENNAIA TIPA 1, ENDOKRINNYKH ZHELEZ NOVOOBRAZOVANIIA TIPA 1 MNOZHESTVENNYE, MEN 1, ВЕРМЕРА СИНДРОМ, МЭН 1, НЕОПЛАЗИЯ ЭНДОКРИННАЯ МНОЖЕСТВЕННАЯ ТИПА 1, НОВООБРАЗОВАНИЯ ЭНДОКРИННЫЕ МНОЖЕСТВЕННЫЕ ТИПА 1, ЭНДОКРИННАЯ НЕОПЛАЗИЯ МНОЖЕСТВЕННАЯ ТИПА 1, ЭНДОКРИННЫХ ЖЕЛЕЗ НОВООБРАЗОВАНИЯ ТИПА 1 МНОЖЕСТВЕННЫЕ
German NEOPLASIE MULTIPLE ENDOKRINE TYP 001, TUMOREN MULTIPLE ENDOKRINE TYP 001, MEN 001, multiple endokrine Neoplasie Typ I, MEA Typ I, multiple endokrine Adenomatose Typ I, MEN Typ I, MEN 1, Multiple endokrine Neoplasie Typ 1, Neoplasie, multiple endokrine, Typ 1, Tumoren, multiple endokrine, Typ 1, Wermer-Syndrom
Italian Sindrome di Wermer, MEA 1, MEN 1, MEN I, MEA I, Neoplasia endocrina multipla di tipo 1, Neoplasia endocrina multipla tipo I, Adenomatosi endocrina multipla tipo I, Neoplasia endocrina multipla tipo 1
Polish Gruczolakowatość wewnątrzwydzielnicza typu 1, Nowotwory mnogie hormonalne typ I, Zespół Wermera, MEN 1
Japanese 多発性内分泌腫瘍1型, MEN1型, タハツセイナイブンピセンシュショウ1ガタ, 多発性内分泌腺腫症1型, タハツセイナイブンピツシュショウ1ガタ, タハツセイナイブンピシュショウ1ガタ, 多発性内分泌腫症1型, タハツセイナイブンピツセンシュショウ1ガタ, Wermer症候群, ウェルマー症候群, 多内分泌腫瘍1, 多内分泌腫瘍1型, 多発性内分泌腺腫症1, 腫瘍-多発性内分泌1型
Norwegian Multippel endokrin neoplasi type 1, MEN 1, Wermers syndrom
Czech Mnohočetná endokrinní adenomatóza Typ I, Mnohočetná endokrinní neoplazie typ 1, Mnohočetná endokrinní neoplazie typu I, MEN Typ I, MEA Typ I, MEN‑1, syndrom mnohočetné endokrinní adenomatózy – typ I, syndrom mnohočetné endokrinní neoplazie - MEN 1, Wermerův syndrom, mnohočetná endokrinní neoplazie typu 1, syndrom MEN 1, MEN 1, nádory mnohočetné endokrinní typ 1
Dutch MEA type I, MEN type I, multipele endocriene adenomatose type I, multipele endocriene neoplasie type 1, multipele endocriene neoplasie type I, MEN 1, Multipele-endocriene-neoplasiesyndroom type 1, Neoplasie type 1, multipele endocriene, Neoplasmata type 1, mutipele endocriene, Syndroom type 1, multipele-endocriene-neoplasie-, Wermer-syndroom
Hungarian Multiplex endocrin adenomatosis I. típus, Ember típus I, Többszörös 1. típusú endocrin neoplasia, MEA típus I, Többszörös endocrin neoplasia típus I
Portuguese Adenomatose endócrina múltipla tipo I, Neoplasia endócrina múltipla tipo I, Neoplasia endócrina múltipla tipo 1, MEN 1, Neoplasia Endócrina Múltipla Tipo 1, Síndrome de Wermer, Neoplasias Endócrinas Múltiplas Tipo 1
Spanish Neoplasia endocrina múltiple de tipo I, AEM de tipo I, Adenomatosis endocrina múltiple de tipo I, Neoplasia endocrina múltiple tipo 1, NEM de tipo I, NEM, tipo 1, adenomatosis endocrina múltiple, tipo 1, neoplasia endocrina múltiple, tipo 1 (trastorno), neoplasia endocrina múltiple, tipo 1, síndrome de Wermer, MEN 1, Neoplasia Endocrina Múltiple Tipo 1, Neoplasia Múltiple Endocrina Tipo 1, Síndrome de Wermer, Neoplasias Múltiples Endocrinas Tipo 1