Malignant Neoplasm of the Brain B030

Current RMA Instruments

Changes from previous Instruments

ICD Coding:

• ICD-10-AM Codes: C71

Brief description

This SOP covers malignant neoplasms arising from the cells of the brain itself (primary brain cancers). These tumours originate within brain tissue and are distinct from tumours arising in adjacent structures, such as meninges or pituitary gland, or from cancers that have spread to the brain from elsewhere in the body. 

For the purposes of this SOP, malignant neoplasm of the brain includes malignant neuroepithelial tumours and primary germ cell tumours of the brain. Neuroepithelial tumours arise from cells that form the supporting and functional tissue of the brain and include a broad group of tumours. This SOP excludes tumours of non-brain origin. 

There are many different types of primary brain tumours, and the terminology can be complex. The distinction between benign and malignant tumours in the brain is less clear-cut than in other organs, as even slower-growing tumours can cause significant neurological impairment due to their location. 

Confirming the diagnosis

Diagnosis requires histological confirmation. Assessment and management typically involve a neurosurgeon, neurologist or oncologist. 

Diagnoses covered by SOP

Common

• Astrocytoma (any grade or type)
• Glioblastoma (multiforme)

Uncommon

• Astroblastoma
• Choroid plexus carcinoma
• Primary choriocarcinoma of the brain
• Primary embryonal carcinoma of the brain
• Ependymoblastoma
• Ependymoma
• Ganglioglyoma
• Primary germinoma of the brain
• Gliomatosis cerebri
• Medulloblastoma
• Medulloepithelioma
• Neuroblastoma
• Oligoastrocytoma
• Oligodendroglioma
• Pineoblastoma
• Pineocytoma
• Polar spongioblastoma
• Primary teratoma of the brain 

Conditions excluded from SOP

• Acoustic neuroma *
• Choroid plexus papilloma #
• Craniopharyngioma #
• Dysembryoplastic neuroepithelial tumour #
• Gangliocytoma # (usually benign)
• Haemangioblastoma # soft tissue sarcoma
• Haemangiopericytoma # soft tissue sarcoma
• Hodgkin's lymphoma of the brain *
• Meningioma *
• Neurilemmoma *  - acoustic neuroma
• Neurofibroma #
• Non-Hodgkin's lymphoma of the brain *
• Pituitary adenoma *
• Schwannoma^* - acoustic neuroma
• Secondary/metastatic cancer involving the brain (code to primary cancer site)
• Soft tissue sarcoma of the brain*

* another SOP applies

^# non-SOP condition

Clinical onset

The clinical presentation of malignant brain tumours is highly variable and depends on the tumour's location, size, and rate of growth. Early symptoms may include headaches, seizures, focal neurological deficits (such as weakness, speech or visual changes), or cognitive or behavioural changes. 

Once the diagnosis has been confirmed, clinical onset may be backdated to the time when the first symptoms consistent with the tumour were present. 

Clinical worsening

The natural history of most malignant brain tumours is progressive disease, although the rate of progression varies depending on tumour type and grade. Clinical worsening is primarily associated with the inability to obtain appropriate clinical management, as timely treatment may slow progression or alleviate symptoms. 

Management typically depends on tumour type and stage and may include surgery, radiotherapy, and/or chemotherapy. Specialist input is required to evaluate whether any observed deterioration represents progression beyond the expected course of the disease.