Malignant neoplasm of carotid body

This code signifies a primary cancerous tumor originating in the carotid body, a chemoreceptor located at the bifurcation of the common carotid artery. These rare neuroendocrine tumors, also known as paragangliomas, can be unilateral or bilateral and may present as a palpable neck mass.

Apply this code when documentation explicitly states a malignant neoplasm of the carotid body. This diagnosis is typically confirmed through biopsy and imaging studies (e.g., MRI, CT angiography). Use for primary malignant tumors, not metastatic disease to the carotid body.

• Differentiate from benign carotid body tumors (paragangliomas), which are coded differently.
• Ensure documentation specifies "malignant" to avoid miscoding with benign or uncertain behavior.
• Consider additional codes for any associated syndromes (e.g., MEN syndromes) if documented.