Lateral ventricular liponeurocytoma: Review of literature and case illustration.

BACKGROUND: Liponeurocytoma is an uncommon tumor of the central nervous system. It is very rare for this tumor to originate within the lateral ventricle. In the context of the rarity of this tumor entity, this review article aims to summarize the clinical, radiological, and pathological features of lateral ventricular liponeurocytoma to facilitate its diagnosis and management. METHODS: Here, we conduct a systematic literature review using the Pubmed, Scopus, and Cochrane Library database for all cases of lateral ventricular liponeurocytoma. A case illustration complements this review. RESULTS: The described cases from 1997 onward include 14 cases that have been published in full papers in the English literature. Six additional cases are reported in short English abstracts in full non-English papers, and one case was described in a central neurocytoma report. There is a definite male predominance of 70% (14 male) and a mean age of 37 years (range 24-62). Heterogenous enhancement and signals in magnetic resonant images (MRI) are the radiological characteristics. In all reported cases, the presence of lipocytes and fat vacuoles is considered the paramount histopathological feature. Total surgical resection was achieved in 80% (12 out of 15) of the cases. Only two cases (including ours) received radiation therapy. Recurrence was seen in two patients during follow-up that was treated by radiation therapy in one and surgery in the other. The proliferation index is mostly below 5% in all cases, with the Ki-67 range between<1% to 10%. CONCLUSIONS: Lateral ventricular liponeurocytoma has been treated effectively by surgical resection in a limited number of cases. The decision for radiation therapy is based on a high proliferation index and tumor recurrence.

High-Grade Transformation (Dedifferentiation) of Acinic Cell Carcinoma of the Parotid Gland: Report of an Unusual Variant.

Acinic cell carcinoma with high-grade transformation of the salivary gland is an unusual variant with less than fifty cases being reported in the literature. It is characterized by a low- and high-grade component juxtaposed with one another and tends to take on a more aggressive clinical course than its low-grade counterpart, suggesting a poor clinical outcome. We, hereby, report a case of acinic cell carcinoma in a 48-year-old woman with a 6-month history of a right parotid facial swelling rapidly increasing in size. The tumor was initially resected; however, residual focal tissue subsequently revealed areas typical of low-grade acinic cell carcinoma as well as high-grade transformation/dedifferentiation via histopathology.

Neuropathologic evidence that the Lewy body variant of Alzheimer disease represents coexistence of Alzheimer disease and idiopathic Parkinson disease.

We undertook this study to investigate the neuropathologic relationships among Alzheimer disease (AD), idiopathic Parkinson disease (PD), and the Lewy body variant of AD (AD/LBV). We retrieved 30 autopsy cases in which Lewy bodies (LB) had been identified in the substantia nigra (SN) in routine hematoxylin-eosin-stained sections. Twenty-two of the cases had a primary clinical diagnosis of dementia and neuropathologic changes of AD; 12 of these demented patients also had clinical parkinsonism. Eight cases had clinical and neuropathologic evidence of PD with minimal or no AD neuropathology, though 6 had clinical dementia. Controls consisted of 6 cases of AD without SN LB by hematoxylin-eosin, and 5 neurologically normal aged controls. Paraffin sections of SN, superior temporal gyrus, and cingulate gyrus from each case were immunostained with rabbit anti-ubiquitin antiserum, randomized, and analyzed individually by light microscopy, and the density of LB-like profiles in each section were graded. None of 5 nondemented aged controls showed any neocortical LB, even though 2 had significant numbers of incidental SN LB by ubiquitin immunostaining. Of 6 AD cases without SN LB by hematoxylin-eosin, 3 had rare SN LB on ubiquitin stain, 1 of which showed rare neocortical Lewy-like profiles. Seven of 8 PD cases showed neocortical LB, including the 6 with dementia. Twenty-one of 22 AD cases with SN LB showed ubiquitin-immunoreactive Lewy-like bodies in the neocortex that were statistically significantly greater in number than in either pure PD or pure AD cases. The frequent occurrence of LB in the neocortex in PD alone suggests that AD/LBV likely represents mixed AD/PD. However, AD neuropathology may favor or promote the formation of neocortical LB in patients who go on to develop mixed AD/PD pathology.

Hormone receptor immunoreactivity in hemangioblastomas and clear cell renal cell carcinomas.

Several primary central nervous system (CNS) neoplasms, including meningiomas, spinal cord ependymomas, and acoustic nerve schwannomas, express hormone receptors. In the present study, we investigated hormone receptor immunoreactivity in hemangioblastomas on the basis of recent reports of these tumors complicating pregnancy. We also evaluated cases of renal cell carcinoma (RCC) metastatic to the CNS, hypothesizing that estrogen receptor (ER), progesterone receptor (PR), or androgen receptor (AR) immunoreactivity might help to distinguish between these histologically similar neoplasms. Immunohistochemical analysis for ERs, PRs, and ARs was performed on paraffin-embedded sections of 27 hemangioblastomas, 12 primary clear cell RCCs, and 5 clear cell RCCs metastatic to the CNS. All of the hemangioblastomas demonstrated PR immunoreactivity, whereas 10 of 12 primary RCCs were negative. In addition, four of the five metastatic RCC were PR positive. All but one primary RCC were ER negative. AR immunoreactivity was seen in three hemangioblastomas, five primary RCCs, and one metastatic RCC. Although hormone receptor immunoreactivity was unable to distinguish between hemangioblastoma and clear cell RCC metastatic to the CNS, the identification of PR immunoreactivity in hemangioblastomas is a new finding that might have adjuvant therapy treatment implications.

Dementia associated with cortical dysplasia.

Detailed neuropathologic examination was performed on a 47.5-year-old man with an unusual adult-onset dementing illness. His initial symptoms were those of depression, memory loss, and personality change. He developed progressive cognitive decline with prominent psychiatric symptoms. Seizures began approximately 11 months prior to death and he died 5.5 years after onset of symptoms. Pathologic examination of the brain at autopsy revealed organizing necrosis of the hippocampi, felt to be the result of his seizures. More significant was the finding of widespread microscopic nodular cortical dysplasia. The dysplastic nodules were composed of clusters of abnormal cells with enlarged, pleomorphic, vesicular nuclei, many of which contained nucleoli and had ballooned cytoplasm. There were no mitoses. Cortical dysplasia is most commonly associated with childhood-onset seizures. It has not, to our knowledge, been reported as a cause of dementia. Whether or not the dysplasia was the basis of the patient's dementia is difficult to say with certainty, but we discuss possible pathoetiologic mechanisms of dementia due to cortical dysplasia.