Multiple endocrine neoplasia [MEN] type IIB

This code represents Multiple Endocrine Neoplasia type IIB (MEN2B), a rare, inherited disorder characterized by the development of tumors in multiple endocrine glands. Key features include medullary thyroid carcinoma, pheochromocytoma, and characteristic physical findings such as mucosal neuromas and a marfanoid habitus.

Use this code when documentation explicitly states a diagnosis of Multiple Endocrine Neoplasia type IIB. This typically applies to patients with a confirmed genetic mutation (e.g., RET proto-oncogene) or a clinical presentation consistent with the syndrome, including the presence of associated tumors or physical stigmata.

• Always confirm the specific MEN type (IIB) as there are other MEN syndromes with distinct codes.
• Code all manifestations of MEN2B, such as medullary thyroid carcinoma and pheochromocytoma, in addition to the MEN syndrome code.
• Ensure documentation clearly distinguishes between MEN2A and MEN2B, as their clinical presentations and coding differ.