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[Medulloblastoma with neuronal differentiation: a report of five cases]
S Nishio1, T Morioka, T Inamura
1Department of Neurosurgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Abstract:
This report presents a retrospective analysis of 5 patients who were treated for cerebellar medulloblastoma with neuronal differentiation. Four males and 1 female ranged in age from 6 months to 9 years at the time of diagnosis. Total removal of the tumor was achieved in 3 patients, and partial removal in 2. While these tumors were composed of small cells and had regions resembling desmoplastic medulloblastoma, they in part showed neuronal characteristics which included parallel row or linear array arrangements of tumor cells in an eosinophilic fibrillary matrix. Postoperatively, 3 patients received craniospinal radiation therapy, one received local radiation to the primary site, and the remaining one received only systemic chemotherapy. During the follow-up period of 3.8-25.2 years, 4 patients have been in continuous remission with mild to moderate neurological deficits, while the remaining one died 3.9 years after surgery. The clinical and anatomic pathological features of medulloblastomas with neuronal differentiation are reviewed while the therapeutic problems associated with these tumors are also discussed.
Insights
This study analyzed 5 patients with cerebellar medulloblastoma showing neuronal differentiation. Most patients achieved long-term remission with neurological deficits, highlighting treatment challenges for this rare tumor type.
Area of Science:
- Neuro-oncology
- Pathology
- Pediatric oncology
Context:
- Medulloblastoma is a common malignant brain tumor in children.
- Medulloblastomas with neuronal differentiation represent a rare subtype with distinct histopathological features.
- Understanding the clinical behavior and treatment outcomes of this subtype is crucial for patient management.
Purpose:
- To present a retrospective analysis of 5 patients diagnosed with cerebellar medulloblastoma exhibiting neuronal differentiation.
- To review the clinical and anatomic pathological features of these rare tumors.
- To discuss the therapeutic challenges and outcomes associated with medulloblastomas with neuronal differentiation.
Summary:
- Five patients (4 males, 1 female; age 6 months-9 years) with cerebellar medulloblastoma showing neuronal differentiation were analyzed.
- Tumors displayed characteristics of small cells and desmoplastic medulloblastoma, alongside neuronal features like linear cell arrangements in a fibrillary matrix.
- Treatment involved surgery (total or partial removal), followed by craniospinal radiation, local radiation, or chemotherapy.
- Follow-up (3.8-25.2 years) showed 4 patients in continuous remission with mild-to-moderate neurological deficits; one patient died 3.9 years post-surgery.
Impact:
- This case series provides insights into the clinical presentation and long-term outcomes of a rare medulloblastoma subtype.
- The findings underscore the variability in treatment response and the potential for long-term survival with neurological sequelae.
- Further research into optimal therapeutic strategies for medulloblastomas with neuronal differentiation is warranted.