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 desc...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.展开更多
We report an extremely rare tumor presenting with myoid features in the left cerebellum lobe in a 62-year-old man. This tumor consisted of medium to large round cells with focal lipomatous and myoid differentiation. I...We report an extremely rare tumor presenting with myoid features in the left cerebellum lobe in a 62-year-old man. This tumor consisted of medium to large round cells with focal lipomatous and myoid differentiation. Immunohistochemically, the tumor cells expressed synaptophysin, GFAP (glial fibrillary acidic protein) and focally desmin. From these findings, we concluded that this tumor was a liponeurocytoma with myoid features. To our knowledge, this is the second report of liponeurocytoma with myoid differentiation in the cerebellum.展开更多
Cerebellar liponeurocytoma (CL) described under many different names, is a rare WHO grade I or II well differentiated neurocytic tumor of the cerebellum with focal lipomatous differentiation. Mainly reported in adulth...Cerebellar liponeurocytoma (CL) described under many different names, is a rare WHO grade I or II well differentiated neurocytic tumor of the cerebellum with focal lipomatous differentiation. Mainly reported in adulthood, it is fought to be a posterior fossa benign tumor. In this paper, we talk about a 64-year-old woman, following up for Parkinson’s disease presented in our department for 7 months’ history of headache and gait disturbance. MRI showed a right cerebellopontine angle (CPA) heterogeneous unlimited tumor mimicking an epidermoid cyst. She underwent a lateral suboccipital craniectomy procedure that permitted obtaining the whole gross total resection of this tumor. The histopathological diagnosis was a cerebellar liponeurocytoma. She was discharged from hospital the 8th day after surgery and was free of symptoms since 5 years.展开更多
Lipomatous medulloblastoma is not a separate entity but apparently distinct variant of medulloblastoma. Since the first case report in 1978 by Bechtel et al. [1], few cases have been published. We report an additional...Lipomatous medulloblastoma is not a separate entity but apparently distinct variant of medulloblastoma. Since the first case report in 1978 by Bechtel et al. [1], few cases have been published. We report an additional case of a 52-year-old right-handed man presented to the emergency department with evaluation of progressive severe intracranial hypertension and ataxia. His past medical history was unremarkable. His physical examination showed cerebellar syndromes. He underwent magnetic resonance imaging (MRI) which revealed a unique mass in the posterior fossa, lying within a cerebellar hemisphere. The patient underwent a suboccipital craniotomy for with a complete resection and samples were sent for histologic examination, showing closely packed, and round to oval cells. The nuclei had an irregular shape with clumps of heterochromatin. Lipid accumulation was a prominent feature of neoplastic cells. Postoperatively the patient did not receive fractionated radiotherapy. He is alive without deficit and with no evidence of recurrence on neuroimaging.展开更多
文摘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.
文摘We report an extremely rare tumor presenting with myoid features in the left cerebellum lobe in a 62-year-old man. This tumor consisted of medium to large round cells with focal lipomatous and myoid differentiation. Immunohistochemically, the tumor cells expressed synaptophysin, GFAP (glial fibrillary acidic protein) and focally desmin. From these findings, we concluded that this tumor was a liponeurocytoma with myoid features. To our knowledge, this is the second report of liponeurocytoma with myoid differentiation in the cerebellum.
文摘Cerebellar liponeurocytoma (CL) described under many different names, is a rare WHO grade I or II well differentiated neurocytic tumor of the cerebellum with focal lipomatous differentiation. Mainly reported in adulthood, it is fought to be a posterior fossa benign tumor. In this paper, we talk about a 64-year-old woman, following up for Parkinson’s disease presented in our department for 7 months’ history of headache and gait disturbance. MRI showed a right cerebellopontine angle (CPA) heterogeneous unlimited tumor mimicking an epidermoid cyst. She underwent a lateral suboccipital craniectomy procedure that permitted obtaining the whole gross total resection of this tumor. The histopathological diagnosis was a cerebellar liponeurocytoma. She was discharged from hospital the 8th day after surgery and was free of symptoms since 5 years.
文摘Lipomatous medulloblastoma is not a separate entity but apparently distinct variant of medulloblastoma. Since the first case report in 1978 by Bechtel et al. [1], few cases have been published. We report an additional case of a 52-year-old right-handed man presented to the emergency department with evaluation of progressive severe intracranial hypertension and ataxia. His past medical history was unremarkable. His physical examination showed cerebellar syndromes. He underwent magnetic resonance imaging (MRI) which revealed a unique mass in the posterior fossa, lying within a cerebellar hemisphere. The patient underwent a suboccipital craniotomy for with a complete resection and samples were sent for histologic examination, showing closely packed, and round to oval cells. The nuclei had an irregular shape with clumps of heterochromatin. Lipid accumulation was a prominent feature of neoplastic cells. Postoperatively the patient did not receive fractionated radiotherapy. He is alive without deficit and with no evidence of recurrence on neuroimaging.
