Lethal midline granuloma

This code represents a rare, aggressive, and often fatal form of T-cell lymphoma characterized by progressive destruction of the midfacial tissues, including the nose, palate, and paranasal sinuses. It is a localized, extranodal natural killer/T-cell lymphoma, nasal type, that can lead to significant disfigurement and functional impairment.

Use this code for documented cases of lethal midline granuloma, also known as extranodal NK/T-cell lymphoma, nasal type, when the pathology report confirms this specific diagnosis. Documentation should clearly describe the destructive lesions in the midfacial region and the histological findings consistent with this lymphoma.

• Ensure pathology reports explicitly confirm "lethal midline granuloma" or "extranodal NK/T-cell lymphoma, nasal type."
• Do not confuse with non-neoplastic granulomatous conditions; this code is specifically for a malignant lymphoma.
• Consider additional codes for associated complications, such as bone destruction or secondary infections, if documented.