Treatment Approach for Myxopapillary Ependymoma with Panhypopituitarism
The treatment of myxopapillary ependymoma with concurrent panhypopituitarism requires gross total resection of the tumor whenever feasible, followed by hormone replacement therapy for the endocrine dysfunction, with postoperative radiotherapy (≥50 Gy) indicated only if complete resection cannot be achieved. 1, 2
Primary Management of Myxopapillary Ependymoma
Surgical Management
- Gross total resection (GTR) is the primary treatment goal with level B evidence 1
- Careful surgical technique is critical to avoid capsular violation, as this strongly correlates with recurrence 1, 2
- Postoperative MRI should be performed to evaluate the extent of resection 1
- Disease staging with both craniospinal MRI and CSF cytology should be performed 2-3 weeks after surgery 1
Adjuvant Therapy
- Postoperative radiotherapy with doses ≥50 Gy is recommended only in cases of incomplete resection (Level B evidence) 1
- For patients with GTR, observation without radiotherapy is appropriate 1, 3
- The 10-year progression-free survival improves from <40% to 70% with radiotherapy after incomplete resection 1
- Chemotherapy has limited role in primary treatment of myxopapillary ependymoma 4
Management of Panhypopituitarism
Comprehensive hormone replacement therapy is essential for managing panhypopituitarism 2
- Glucocorticoid replacement (hydrocortisone or prednisone)
- Thyroid hormone replacement (levothyroxine)
- Sex hormone replacement (testosterone for men; estrogen/progesterone for women)
- Growth hormone replacement if indicated
- Desmopressin (DDAVP) if diabetes insipidus is present
Regular monitoring of hormone levels and adjustment of replacement doses is necessary 2
Special Considerations
Radiotherapy Planning
- When radiotherapy is required, careful planning is essential to minimize additional damage to the pituitary region 2
- Endocrine function should be closely monitored during and after radiotherapy 2
Pediatric Patients
- Pediatric patients have significantly higher recurrence rates (40.5%) compared to adults (23.4%) 3
- Children may benefit more from adjuvant radiotherapy than adults, suggesting biological differences in tumors between age groups 3
Monitoring for Recurrence
- Long-term follow-up with enhanced MRI is essential due to the risk of asymptomatic and late relapses 1, 2
- Metastatic dissemination within the CNS occurs in up to 57.9% of patients 5
- Most common sites for metastases are thoracic and sacral spine (38.9% and 33.3% respectively) 5
Management of Recurrence or Metastasis
- In case of relapse, consider re-operation, re-irradiation, or chemotherapy 1
- For asymptomatic metastases, close clinical and MRI follow-up may be sufficient as many remain stable without progression 5
- Intervention is recommended if metastases become symptomatic 5
Prognosis
- Overall survival at 10 years exceeds 90% 1, 2
- Presacral MPE shows worse outcomes compared to MPE of the filum terminale/cauda equina region 1
- Despite the generally favorable prognosis, CSF dissemination can occur if tumor capsule is violated 6
Pitfalls and Caveats
- Failure to achieve GTR significantly increases recurrence risk (32.6% vs 15.5% with GTR) 3
- Violation of tumor capsule during surgery dramatically increases risk of CSF dissemination and recurrence 6
- Delaying or omitting radiotherapy after incomplete resection may lead to progression and poorer outcomes 1, 6
- Inadequate hormone replacement for panhypopituitarism can lead to life-threatening adrenal crisis, hypothyroidism, and reduced quality of life 2