E31.22

Applicable To

• Sipple’s syndrome

Approximate Synonyms

• Multiple endocrine neoplasia type 2a

• Multiple endocrine neoplasia type 2a cancerous

• Multiple endocrine neoplasia type 2a noncancerous

• Multiple endocrine neoplasia type 2a, benign

• Multiple endocrine neoplasia type 2a, malignant

• Multiple endocrine neoplasia, type 2

Clinical Information

• 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.

• Multiple endocrine neoplasia caused by mutation of the ret gene. Patients develop medullary thyroid carcinomas, and may also develop pheochromocytomas and parathyroid gland hyperplasia.

Details

• E31.22 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

• The 2023 edition of ICD-10-CM E31.22 became effective on October 1, 2022.

• This is the American ICD-10-CM version of E31.22 – other international versions of ICD-10 E31.22 may differ.