Primary central nervous system lymphoma

This code represents a malignant lymphoma originating in the brain, spinal cord, meninges, or eyes, without evidence of systemic disease. It is a specific type of non-Hodgkin lymphoma that primarily affects the central nervous system.

Assign this code when documentation confirms a diagnosis of primary central nervous system lymphoma, explicitly excluding Burkitt, lymphoblastic, peripheral T-cell, or other specified types. This diagnosis is typically established through biopsy and imaging studies.

• Carefully review pathology reports to confirm the specific subtype of primary CNS lymphoma.
• Ensure there is no evidence of systemic lymphoma, as this would change the primary site.
• Refer to the Excludes1 notes to avoid incorrect co-coding with other specific primary CNS lymphoma subtypes.