Treatment for Infratentorial and Supratentorial Enhancing Lesions
The treatment approach depends critically on establishing a definitive histological diagnosis first, followed by multimodal therapy tailored to the specific pathology—surgical resection is the cornerstone for most lesions, with adjuvant radiation and/or chemotherapy determined by tumor type, grade, and extent of resection.
Initial Diagnostic Workup
Before initiating treatment, tissue diagnosis is essential for enhancing lesions:
- For patients without known primary malignancy: Perform systematic workup including chest CT, abdominal/pelvic CT, and consider FDG-PET if multiple brain lesions are present 1
- Stereotactic or open biopsy is indicated when no readily accessible tumor exists elsewhere or when diagnosis is uncertain 1
- MRI with gadolinium is the preferred imaging modality for characterizing lesions and planning treatment 1
- Spinal MRI and lumbar puncture should be considered for infratentorial tumors to assess for CSF dissemination 1
Management of Acute Complications
Hydrocephalus Management
For patients presenting with obstructive hydrocephalus:
- External ventricular drain via frontal trajectory for acute intracranial hypertension requiring immediate ICP stabilization 1
- Endoscopic third ventriculostomy (ETV) is preferred in centers with neuroendoscopic expertise, as tumor biopsy can be performed simultaneously with lower complication rates compared to shunting 1
- CSF shunting remains reliable for insidious hydrocephalus, particularly in limited-resource settings 1
- Ventriculoperitoneal shunt for persistent hydrocephalus in medulloblastoma and primitive neuroectodermal tumors 1
Treatment by Tumor Type
Pineal Region Tumors (Infratentorial/Supratentorial Interface)
Surgical approach selection is critically informed by: (1) relationship to deep venous network, (2) angle of straight sinus, (3) tumor height along vertical axis 1
Surgical corridor options include:
- Midline infratentorial supracerebellar: For mildly sloped/straight sinus with low-lying tumor 1
- Lateral supracerebellar: For most pineal lesions, especially those extending laterally into thalamus 1
- Occipital transtentorial: For large tumors occupying both supra- and infratentorial spaces 1
- Interhemispheric transcallosal: For tumors high along splenial-fourth ventricle axis 1
Treatment by histology:
- Pineocytoma: Gross total resection is usually the only treatment required 1
- Pineoblastoma: Maximal safe resection followed by risk-adapted craniospinal irradiation (23.4-36 Gy) plus chemotherapy 1
Medulloblastoma and Supratentorial PNETs
Surgical resection should be routine initial treatment to establish diagnosis, relieve symptoms, and maximize local control—complete resection achieved in 50% of patients and associated with improved survival 1
Adjuvant radiation therapy after surgery is standard of care:
- Conventional dose: 30-36 Gy craniospinal irradiation with boost to 55.8 Gy to primary site 1
- Reduced dose: 23.4 Gy craniospinal combined with chemotherapy for average-risk patients, though one randomized trial found increased relapse risk with dose reduction 1
Systemic chemotherapy: Postirradiation chemotherapy increasingly common, especially for children, to allow radiation dose reduction 1
Pleomorphic Xanthoastrocytoma (Supratentorial)
Optimal surgical resection should be undertaken in all patients as primary treatment 1, 2
Postoperative management:
- Grade 2 (typical PXA) with complete resection: Simple clinical follow-up with serial MRI 1, 2
- Grade 3 (anaplastic features): Postoperative external-beam radiotherapy mandatory, irrespective of resection extent 1, 2
- Incomplete resection or progression: Consider surgery, radiotherapy, and/or chemotherapy (though optimal regimens uncertain) 1, 2
Ependymoma (Infratentorial or Supratentorial)
Localized lesions:
- Surgery is standard treatment with complete resection confirmed by early postoperative MRI being a good prognostic factor 1
- Localized radiotherapy (not craniospinal) should be used if radiotherapy is administered 1
Grade 2 with complete resection: No complementary treatment necessary 1
Grade 3 or incomplete resection: Localized postoperative radiotherapy should be offered 1
Metastatic lesions: Craniospinal radiotherapy should be offered, with poor prognosis 1
Brain Metastases (1-3 Limited Lesions)
For patients with limited systemic disease or reasonable systemic treatment options:
Surgical candidates with single lesion:
- Surgical resection plus postoperative WBRT (Category 1 recommendation) 1
- SRS plus WBRT (Category 1 recommendation) 1
- SRS alone or after resection are reasonable options 1
Unresectable tumors: WBRT and/or radiosurgery 1
WBRT dosing: 30-45 Gy in 1.8-3.0 Gy fractions depending on performance status 1
Progressive extracranial disease with survival <3 months: WBRT alone, though surgery may be considered for symptom relief 1
Glioblastoma (Supratentorial)
Initial treatment: Maximal safe surgical resection followed by focal radiotherapy (60 Gy/30 fractions) with concomitant temozolomide (75 mg/m² daily during RT for 42 days, maximum 49 days) 3
Maintenance therapy: 6 cycles of temozolomide (150-200 mg/m² on Days 1-5 of every 28-day cycle) starting 4 weeks after RT completion 3
PCP prophylaxis required during temozolomide plus RT regardless of lymphocyte count 3
Recurrent disease:
- Local recurrence: Repeat resection if possible, followed by systemic chemotherapy if performance status favorable 1
- Reirradiation is Category 2B option if prior radiation produced good/durable response 1
- Alternating electric field therapy may be considered for glioblastoma (Category 2B) 1
Follow-Up Protocol
Serial MRI surveillance:
- 2-6 weeks post-RT, then every 2-4 months for 2-3 years, then less frequently 1
- Early scans allow appropriate corticosteroid titration, as scans may appear worse during first 3 months after RT completion (pseudoprogression) 1
- MR spectroscopy, MR perfusion, or PET can be considered to rule out radiation-induced necrosis or pseudoprogression 1
For esthesioneuroblastoma: Neurological assessment and neuroimaging at 3-month intervals 4
Common Pitfalls to Avoid
- Do not assume all enhancing lesions are malignant: Enhancement >3 months should raise suspicion for sarcoidosis, vascular abnormality (developmental venous anomaly, capillary telangiectasia), or other non-neoplastic pathology 1
- Confirm enhancement with pre-contrast images: Spontaneous T1 hyperintensity from lipid/iron-laden macrophages can mimic enhancement 1
- Do not perform WBRT twice: Patients previously treated with WBRT should not undergo repeat WBRT at recurrence due to neurotoxicity concerns 1
- Avoid inappropriate grouping: Esthesioneuroblastoma has significantly better prognosis (5-year survival ~90%) than other sinonasal malignancies and should not be treated identically 4