2020 ICD-10-CM Diagnosis Code E31.22

Multiple endocrine neoplasia [MEN] type IIA

    2016 2017 2018 2019 2020 Billable/Specific Code
  • E31.22 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2020 edition of ICD-10-CM E31.22 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of E31.22 - other international versions of ICD-10 E31.22 may differ.
Applicable To
  • Sipple's syndrome
The following code(s) above E31.22 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to E31.22:
  • E00-E89
    2020 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    Note
    • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
    Type 1 Excludes
    • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
    Endocrine, nutritional and metabolic diseases
  • E20-E35
    2020 ICD-10-CM Range E20-E35

    Disorders of other endocrine glands

    Type 1 Excludes
    Disorders of other endocrine glands
  • E31
    ICD-10-CM Diagnosis Code E31

    Polyglandular dysfunction

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • ataxia telangiectasia [Louis-Bar] (G11.3)
    • dystrophia myotonica [Steinert] (G71.11)
    • pseudohypoparathyroidism (E20.1)
    Polyglandular dysfunction
  • E31.2
    ICD-10-CM Diagnosis Code E31.2

    Multiple endocrine neoplasia [MEN] syndromes

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Applicable To
    • Multiple endocrine adenomatosis
    Code Also
    • any associated malignancies and other conditions associated with the syndromes
    Multiple endocrine neoplasia [MEN] syndromes
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.
ICD-10-CM E31.22 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 643 Endocrine disorders with mcc
  • 644 Endocrine disorders with cc
  • 645 Endocrine disorders without cc/mcc

Convert E31.22 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change

Diagnosis Index entries containing back-references to E31.22:
  • Neoplasia
    • endocrine, multiple (MEN) E31.20
      ICD-10-CM Diagnosis Code E31.20

      Multiple endocrine neoplasia [MEN] syndrome, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code
      Applicable To
      • Multiple endocrine adenomatosis NOS
      • Multiple endocrine neoplasia [MEN] syndrome NOS
      • type IIA E31.22
  • Sipple's syndrome E31.22
  • Syndrome - see also Disease
    • Sipple's E31.22

ICD-10-CM Codes Adjacent To E31.22
E30.0 Delayed puberty
E30.1 Precocious puberty
E30.8 Other disorders of puberty
E30.9 Disorder of puberty, unspecified
E31 Polyglandular dysfunction
E31.0 Autoimmune polyglandular failure
E31.1 Polyglandular hyperfunction
E31.2 Multiple endocrine neoplasia [MEN] syndromes
E31.20 Multiple endocrine neoplasia [MEN] syndrome, unspecified
E31.21 Multiple endocrine neoplasia [MEN] type I
E31.22 Multiple endocrine neoplasia [MEN] type IIA
E31.23 Multiple endocrine neoplasia [MEN] type IIB
E31.8 Other polyglandular dysfunction
E31.9 Polyglandular dysfunction, unspecified
E32 Diseases of thymus
E32.0 Persistent hyperplasia of thymus
E32.1 Abscess of thymus
E32.8 Other diseases of thymus
E32.9 Disease of thymus, unspecified
E34 Other endocrine disorders
E34.0 Carcinoid syndrome

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.