Multiple endocrine neoplasia type 2
| MEN type 2A (Sipple syndrome) | |
|---|---|
 |
|
| Bilateral pheochromocytomas associated with Multiple endocrine neoplasia type 2 | |
| Classification and external resources | |
| Specialty | oncology |
| ICD-10 | D44.8 |
| ICD-9-CM | 258.02 |
| OMIM | 171400 |
| DiseasesDB | 7984 |
| MedlinePlus | 000399 |
| eMedicine | med/1520 |
| MeSH | D018813 |
| GeneReviews | |
Multiple endocrine neoplasia type 2 (MEN2) (also known as "Pheochromocytoma and amyloid producing medullary thyroid carcinoma", "PTC syndrome," and "Sipple syndrome") is a group of medical disorders associated with tumors of the endocrine system. The tumors may be benign or malignant (cancer). They generally occur in endocrine organs (e.g. thyroid, parathyroid, and adrenals), but may also occur in endocrine tissues of organs not classically thought of as endocrine.
MEN2 is a sub-type of MEN (multiple endocrine neoplasia) and itself has sub-types, as discussed below.
Before gene testing was available, the type and location of tumors determined which type of MEN2 a person had. Gene testing now allows a diagnosis before tumors or symptoms develop.
A table in the multiple endocrine neoplasia article compares the various MEN syndromes. MEN2 and MEN1 are distinct conditions, despite their similar names. MEN2 includes MEN2A, MEN2B and familial medullary thyroid cancer (FMTC).
The common feature among the three sub-types of MEN2 is a high propensity to develop medullary thyroid carcinoma.
MEN2 can present with a sign or symptom related to a tumor or, in the case of multiple endocrine neoplasia type 2b, with characteristic musculoskeletal and/or lip and/or gastrointestinal findings.Medullary thyroid carcinoma (MTC) represent the most frequent initial diagnosis. Occasionally pheochromocytoma or primary hyperparathyroidism may be the initial diagnosis.
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