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Intraventricular rhabdoid tumor
Faiz Uddin Ahmad1, Ashish Suri, Ashok Kumar Mahapatra
1Department of Neurosurgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.
Insights
Malignant rhabdoid tumors (MRT) are aggressive brain cancers in children. Despite surgical removal and treatment, this case highlights the poor prognosis of CNS rhabdoid tumors.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Pathology
Background:
- Malignant rhabdoid tumor (MRT) is a rare and aggressive cancer.
- While most common in the kidney, MRT can occur in the central nervous system (CNS), typically in the cerebellum.
- CNS rhabdoid tumors present a significant challenge due to their aggressive nature and poor treatment response in young children.
Observation:
- A 4-year-old child presented with symptoms of increased intracranial pressure, including papilledema.
- Imaging identified a heterogeneous mass in the left lateral ventricle, causing hydrocephalus.
- The child underwent surgical tumor resection and a ventriculo-peritoneal shunt placement.
Findings:
- Histopathology confirmed the diagnosis of rhabdoid tumor.
- Despite aggressive treatment including radiotherapy and chemotherapy, the patient experienced disease progression.
- The child succumbed to the disease 10 months post-surgery.
Implications:
- This case underscores the highly malignant nature of CNS rhabdoid tumors.
- Early diagnosis and aggressive management are critical for improving outcomes.
- Further research into novel therapeutic strategies is warranted for this devastating pediatric malignancy.
Abstract:
Malignant rhabdoid tumor (MRT) most commonly occurs in kidney. In the central nervous system, cerebellum is the most common site of occurrence. CNS rhabdoid tumors typically occur in small children, do not respond favorably to treatment and are usually fatal within 1-year. Here is reported a 4-year-old child who presented with features of raised intracranial pressure. Apart from papillodema, there were no neurological signs. Imaging revealed a left lateral ventricular heterogeneous mass abutting the foramen of monro, with mild irregular contrast enhancement and hydrocephalus. The child underwent right ventriculo-peritoneal shunt followed by craniotomy and gross total tumor resection. He was discharged 10-days after surgery without any neurological deficits. Histopathology revealed features compatible with rhabdoid tumor. Despite radiotherapy and chemotherapy, the child died of progressive disease 10-months after surgery. The highly malignant nature of this tumor makes early diagnosis essential for aggressive management and prognostication.
