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Successful treatment of disseminated central nervous system malignant rhabdoid tumor
Purpose:
Malignant rhabdoid tumor (MRT) of the central nervous system (CNS) is pathologically identical to MRT of the kidney. CNS MRTs have the clinicopathological behavior of a high-grade intracranial sarcoma, and the children have a very poor prognosis. We report on three cases of primary CNS MRT with a review and summary of the pediatric literature with respect to demographic features and multidisciplinary management.
Patients And Methods:
The 18 cases reviewed had a male to female ratio of 1.0 and an extremely young median age of 32 months. Our three cases of CNS MRT were treated with surgery, chemotherapy, radiotherapy, and triple intrathecal (TIT) chemotherapy similar to the Intergroup Rhabdomyosarcoma Study III guidelines for parameningeal primary tumors with intracranial extension.
Results:
The three patients described in this report are surviving with no evidence of disease at 5 years, 2 years, and 9 months from diagnosis. Before these three cases, only four of 16 reported patients were known to have survived. One unique case in our report involved disease in the cerebral cortex, sinuses, and orbit with metastases to the subarachnoid space. This metastatic MRT responded to treatment with TIT, multiagent chemotherapy and cranial-spinal radiation after partial resection of only the cortical portion of the MRT.
Conclusions:
Disseminated CNS MRTs can be treated using multidisciplinary management with an approach similar to that used to treat rhabdomyosarcoma.
Insights
Pediatric central nervous system malignant rhabdoid tumors (CNS MRTs) are aggressive. Multidisciplinary management, including triple intrathecal chemotherapy, offers improved survival outcomes for these rare CNS tumors.
Area of Science:
- Pediatric Oncology
- Neuro-Oncology
- Cancer Biology
Background:
- Malignant rhabdoid tumor (MRT) of the central nervous system (CNS) shares pathological characteristics with renal MRT.
- CNS MRTs exhibit aggressive behavior, resembling high-grade intracranial sarcomas with a historically poor prognosis in children.
Observation:
- This study reports on three pediatric cases of primary CNS MRT.
- A review of 18 pediatric cases highlights a median age of 32 months and a 1:1 male to female ratio.
- One unique case presented with extensive disease in the cerebral cortex, sinuses, orbit, and subarachnoid space.
Findings:
- The three reported patients achieved long-term survival (5 years, 2 years, 9 months) with no evidence of disease.
- Prior to this study, only 4 of 16 reported patients had survived.
- The unique metastatic case responded favorably to triple intrathecal (TIT) chemotherapy, multiagent chemotherapy, and cranial-spinal radiation after partial resection.
Implications:
- Multidisciplinary management, incorporating strategies similar to those for rhabdomyosarcoma, can improve outcomes for CNS MRT.
- Aggressive treatment protocols, including TIT chemotherapy, are effective for disseminated CNS MRT.
- These findings offer hope and guide treatment strategies for this rare and aggressive pediatric malignancy.