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Multiple mucosal neuromas

MedGen UID:
233722
•Concept ID:
C1334828
•
Neoplastic Process
Synonyms: multiple mucosal neuroma; Multiple Mucosal Neuromas; multiple mucosal neuromas
 
HPO: HP:0031023
Monarch Initiative: MONDO:0003307

Definition

Multiple painful, dome-shaped, translucent pink to skin-colored papules on oral mucosa. Histologically, the lesions may demonstrate dermal proliferation of well-demarcated nerve bundles associated with abundant mucin and surrounded by a distinct perineural sheath. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMultiple mucosal neuromas

Professional guidelines

PubMed

Hereditary Medullary Thyroid Cancer Genotype-Phenotype Correlation.
Frank-Raue K, Raue F
Recent Results Cancer Res 2015;204:139-56. doi: 10.1007/978-3-319-22542-5_6. PMID: 26494387
Genotype-phenotype relationship in multiple endocrine neoplasia type 2. Implications for clinical management.
Raue F, Frank-Raue K
Hormones (Athens) 2009 Jan-Mar;8(1):23-8. doi: 10.14310/horm.2002.1218. PMID: 19269918
Genetic testing in multiple endocrine neoplasia and related syndromes.
Calender A
Forum (Genova) 1998 Apr-Jun;8(2):146-59. PMID: 9666051

Recent clinical studies

Etiology

Dermal Hyperneury and Multiple Sclerotic Fibromas in Multiple Endocrine Neoplasia Type 2A Syndrome.
Alegría-Landa V, Jo-Velasco M, Robledo M, Requena L
JAMA Dermatol 2017 Dec 1;153(12):1298-1301. doi: 10.1001/jamadermatol.2017.3959. PMID: 29049491Free PMC Article
Reactive hyperplasias,precancerous and malignant lesions of the oral mucosa.
Krahl D, Altenburg A, Zouboulis CC
J Dtsch Dermatol Ges 2008 Mar;6(3):217-32. doi: 10.1111/j.1610-0387.2007.06625.x. PMID: 18315622
Multiple endocrine neoplasia type 2B--genetic basis and clinical expression.
Lee NC, Norton JA
Surg Oncol 2000 Nov;9(3):111-8. doi: 10.1016/s0960-7404(00)00038-4. PMID: 11356339
The Multiple Endocrine Neoplasia syndromes.
Brunt LM, Wells SA Jr
Ann Chir Gynaecol 1983;72(3):153-9. PMID: 6137991
Medullary carcinoma of the thyroid in the multiple mucosal neuromas syndrome.
Netzloff ML, Garnica AD, Rodgers BM, Frias JL
Ann Clin Lab Sci 1979 Sep-Oct;9(5):368-73. PMID: 533232

Diagnosis

Reactive hyperplasias,precancerous and malignant lesions of the oral mucosa.
Krahl D, Altenburg A, Zouboulis CC
J Dtsch Dermatol Ges 2008 Mar;6(3):217-32. doi: 10.1111/j.1610-0387.2007.06625.x. PMID: 18315622
Laryngeal solitary multiple mucosal neuromas without multiple endocrine neoplasia (MEN) type 2B.
Shimazaki T, Yoshida Y, Izumaru S, Nakashima T
Auris Nasus Larynx 2003 May;30(2):191-5. doi: 10.1016/s0385-8146(02)00115-3. PMID: 12753993
Hereditary medullary thyroid carcinoma.
Vasen HF, Vermey A
Cancer Detect Prev 1995;19(2):143-50. PMID: 7750101
The Multiple Endocrine Neoplasia syndromes.
Brunt LM, Wells SA Jr
Ann Chir Gynaecol 1983;72(3):153-9. PMID: 6137991
Multiple mucosal neuromas, pheochromocytoma, medullary carcinoma of the thyroid and marfanoid body build with muscle wasting: reexamination of a syndrome of neural crest malmigration.
Gorlin RJ, Vickers RA
Birth Defects Orig Artic Ser 1971 May;7(6):69-72. PMID: 4950920

Prognosis

Multiple endocrine neoplasia type 2B--genetic basis and clinical expression.
Lee NC, Norton JA
Surg Oncol 2000 Nov;9(3):111-8. doi: 10.1016/s0960-7404(00)00038-4. PMID: 11356339
A case of multiple endocrine neoplasia type 2B.
Ohta M, Tokuda Y, Suzuki Y, Kuge S, Okumura A, Kubota M, Tajima T, Osamura RY, Mitomi T
Jpn J Clin Oncol 1997 Aug;27(4):268-73. doi: 10.1093/jjco/27.4.268. PMID: 9379517
Multiple endocrine neoplasia: Part II. Sipple's syndrome.
Modlin IM, Lamers CB
S Afr Med J 1979 Sep 22;56(13):521-5. PMID: 550388
Medullary carcinoma of the thyroid in the multiple mucosal neuromas syndrome.
Netzloff ML, Garnica AD, Rodgers BM, Frias JL
Ann Clin Lab Sci 1979 Sep-Oct;9(5):368-73. PMID: 533232
Pathological and clinical findings in a series of 67 cases of medullary carcinoma of the thyroid.
Williams ED, Brown CL, Doniach I
J Clin Pathol 1966 Mar;19(2):103-13. doi: 10.1136/jcp.19.2.103. PMID: 5909693Free PMC Article

Clinical prediction guides

Laryngeal neuroma in multiple endocrine neoplasia type 2B.
Kudo N, Matsubara A, Abe T, Inoue T, Takahata J
Auris Nasus Larynx 2014 Aug;41(4):389-91. Epub 2014 Jan 2 doi: 10.1016/j.anl.2013.12.007. PMID: 24389350
Laryngeal solitary multiple mucosal neuromas without multiple endocrine neoplasia (MEN) type 2B.
Shimazaki T, Yoshida Y, Izumaru S, Nakashima T
Auris Nasus Larynx 2003 May;30(2):191-5. doi: 10.1016/s0385-8146(02)00115-3. PMID: 12753993
Multiple endocrine neoplasia (MEN) type II b: report of a case observed at autopsy with immunohistochemical study of mucosal neuromas.
Lesourd A, Mikol J, Bishopric G, Dubost C, Brocheriou C
Clin Neuropathol 1988 Sep-Oct;7(5):238-43. PMID: 2905214

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