Multiple endocrine neoplasia [MEN] type I

This code signifies Multiple Endocrine Neoplasia type 1 (MEN1), a rare, inherited disorder characterized by tumors in two or more endocrine glands, most commonly the parathyroid, pituitary, and pancreas. These tumors can be benign or malignant and lead to overproduction of hormones, causing a variety of symptoms.

Use this code when documentation confirms a diagnosis of MEN1, typically based on genetic testing or the presence of characteristic tumors in multiple endocrine glands. This diagnosis is often established after a patient presents with symptoms related to hyperparathyroidism, pituitary adenomas, or pancreatic neuroendocrine tumors.

• Ensure documentation specifies "type I" to differentiate from other MEN syndromes.
• Code all associated endocrine manifestations (e.g., hyperparathyroidism, pituitary adenoma, pancreatic neuroendocrine tumor) in addition to this code.
• Genetic testing results confirming a pathogenic variant in the MEN1 gene strongly support this diagnosis.