Multiple endocrine neoplasia [MEN] type IIB
ICD-10 E31.23 is a billable code used to indicate a diagnosis of multiple endocrine neoplasia [men] type iib.
Multiple endocrine neoplasia type IIB (MEN IIB) is a rare genetic disorder characterized by the development of tumors in multiple endocrine glands. This condition is associated with medullary thyroid carcinoma, pheochromocytoma, and mucosal neuromas, often leading to significant clinical manifestations. Patients may experience growth abnormalities due to hormonal imbalances, particularly during puberty, as the condition can disrupt normal endocrine function. Additionally, MEN IIB is linked to polyglandular dysfunction, where multiple glands are affected, leading to a complex interplay of symptoms. Thymus disorders may also arise, contributing to immune dysfunction. The genetic basis of MEN IIB is primarily due to mutations in the RET proto-oncogene, which plays a crucial role in cell signaling and growth. Early diagnosis and management are essential to mitigate the risks of malignancies and other complications associated with this syndrome.
Thorough documentation of hormonal levels, imaging studies, and genetic testing results.
Patients presenting with symptoms of hypercalcemia, hypertension, or thyroid nodules.
Ensure all endocrine glands are evaluated and documented, including any surgical interventions.
Detailed pathology reports and treatment plans for malignancies such as medullary thyroid carcinoma.
Management of thyroid cancer or pheochromocytoma in MEN IIB patients.
Document the staging of cancers and any genetic counseling provided.
Used when a biopsy is performed to confirm medullary thyroid carcinoma.
Pathology report must be included in the patient's medical record.
Endocrinologists and oncologists should collaborate on pathology findings.
Primary symptoms include growth abnormalities, hypertension due to pheochromocytoma, and symptoms related to medullary thyroid carcinoma such as neck swelling or thyroid nodules.
