摘要
Background and Importance: Lipomatous medulloblastoma (cerebellar liponeurocytoma) is a rare cerebellar tumor, with only twenty-nine cases reported, considered a distinct variant of medulloblastoma. The few cases described support an indolent nature for this tumor. We aim at defining the optimum treatment strategy and long-term behavior for this tumor entity. Clinical presentation: A 74 years old male presented on September 2010 complaining of mild dizziness and headache slowly progressing over a few months. This gentleman was operated on at our department some 18 years ago for a right cerebellar hemispheral lesion, defined as a liponeurocytoma. This patient received no adjuvant treatment. Current magnetic resonance imaging (MRI) studies disclosed a right hemispheral cerebellar mass, locally recurrent in the original surgical tumor bed. Gross total resection of the tumor was accomplished through a suboccipital craniotomy, with complete resection of the lesion. The histopathological diagnosis was defined as cerebellar liponeurocytoma. No adjuvant therapy was given as initially, after the first operation. Currently, the patient is alive, fully alert with minimal neurological deficits, Barthel index 90, Kernofsky performance status of 90 and with no evidence of disease on neuroimaging. Conclusion: This patient portrays this tumor’s natural history after surgical intervention with no adjuvant treatment, being the longest reported follow-up and recurrence. This distinct variant of medulloblastoma appears to have a uniquely favorable prognosis, even without adjuvant therapy. A complete surgical resection with close follow-up seems both sufficient and prudent.
Background and Importance: Lipomatous medulloblastoma (cerebellar liponeurocytoma) is a rare cerebellar tumor, with only twenty-nine cases reported, considered a distinct variant of medulloblastoma. The few cases described support an indolent nature for this tumor. We aim at defining the optimum treatment strategy and long-term behavior for this tumor entity. Clinical presentation: A 74 years old male presented on September 2010 complaining of mild dizziness and headache slowly progressing over a few months. This gentleman was operated on at our department some 18 years ago for a right cerebellar hemispheral lesion, defined as a liponeurocytoma. This patient received no adjuvant treatment. Current magnetic resonance imaging (MRI) studies disclosed a right hemispheral cerebellar mass, locally recurrent in the original surgical tumor bed. Gross total resection of the tumor was accomplished through a suboccipital craniotomy, with complete resection of the lesion. The histopathological diagnosis was defined as cerebellar liponeurocytoma. No adjuvant therapy was given as initially, after the first operation. Currently, the patient is alive, fully alert with minimal neurological deficits, Barthel index 90, Kernofsky performance status of 90 and with no evidence of disease on neuroimaging. Conclusion: This patient portrays this tumor’s natural history after surgical intervention with no adjuvant treatment, being the longest reported follow-up and recurrence. This distinct variant of medulloblastoma appears to have a uniquely favorable prognosis, even without adjuvant therapy. A complete surgical resection with close follow-up seems both sufficient and prudent.
