Anaplastic/large cell medulloblastoma

Anaplastic/large cell medulloblastoma is an aggressive subtype of medulloblastoma, a malignant embryonal tumor arising in the cerebellum (posterior fossa) of the brain. It is classified as a WHO Grade IV central nervous system tumor and is characterized by marked nuclear pleomorphism (anaplasia), high mitotic activity, abundant apoptosis, and the presence of large cells with prominent nucleoli and abundant cytoplasm. This histological variant carries a worse prognosis compared to classic medulloblastoma. It primarily affects children, though it can occasionally occur in adolescents and young adults. The ICD-10 code is C71.6, corresponding to neoplasms of the cerebellum. Patients typically present with symptoms of increased intracranial pressure due to obstruction of cerebrospinal fluid flow, including headaches (often worse in the morning), nausea, vomiting, and lethargy. Cerebellar dysfunction manifests as ataxia (unsteady gait), poor coordination, and balance difficulties. Additional symptoms may include cranial nerve palsies, visual disturbances such as diplopia (double vision), and papilledema. Because of its aggressive biology, anaplastic/large cell medulloblastoma has a higher tendency for leptomeningeal dissemination (spread along the spinal cord and brain surfaces) at diagnosis compared to other medulloblastoma subtypes. Treatment follows a multimodal approach and typically includes maximal safe surgical resection of the tumor, followed by craniospinal irradiation and intensive adjuvant chemotherapy. Due to its high-risk classification, patients generally receive intensified treatment protocols, which may include higher doses of craniospinal radiation and aggressive multi-agent chemotherapy regimens (commonly including cisplatin, vincristine, cyclophosphamide, and lomustine). Despite intensive therapy, outcomes remain less favorable than for classic medulloblastoma, with higher rates of recurrence. Ongoing clinical trials are investigating novel targeted therapies and immunotherapies. Long-term survivors require careful monitoring for treatment-related late effects, including neurocognitive impairment, endocrine dysfunction, and secondary malignancies.

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