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Multiple endocrine neoplasia type 2A
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Multiple endocrine neoplasia type 2A

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Contributors: Marilyn Augustine MD
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Synopsis

Multiple endocrine neoplasia type 2A (Sipple syndrome, MEN2A) is an autosomal dominant disorder caused by mutation of the RET gene. Variants within MEN2A include classic MEN2A, MEN2A with cutaneous lichen amyloidosis, MEN2A with Hirschsprung disease, and familial medullary cancer with pheochromocytoma or parathyroid hyperplasia. Patients are at risk of developing tumors in the thyroid, parathyroid, and adrenal glands as well as nerve defects such as neuromas. Other findings include cervical lymphadenopathy, depressed mood, constipation, diarrhea, fatigue, headache, flushing, hypertension, memory impairment, pruritus, and palpitations.

Thyroidectomy is a prophylactic measure. Treatment includes surgical excision of tumors.

For more information, see OMIM.

Related topics: MEN1, MEN2b

Codes

ICD10CM:
E31.22 – Multiple endocrine neoplasia [MEN] type IIA

SNOMEDCT:
61808009 – Multiple endocrine neoplasia, type 2

Differential Diagnosis & Pitfalls

Best Tests

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References

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Last Updated: 03/29/2017
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Multiple endocrine neoplasia type 2A
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Multiple endocrine neoplasia type 2A
Copyright © 2019 VisualDx®. All rights reserved.