Cerebellar liponeurocytoma, a rare, newly identified CNS neoplasm of adults, is characterized by advanced neuronal/neurocytic and focal lipomatous differentiation, low proliferative potential and a favorable clinical prognosis. Despite the different age distribution and benign biological behavior, the cerebellar liponeurocytoma shares several features with the cerebellar medulloblastoma, which may include an origin from the periventricular matrix of the fourth ventricle or the external granular layer of the cerebellum. To establish the genetic profile of cerebellar liponeurocytomas, we have formed an international consortium and collected tumor samples from 20 patients. DNA sequencing revealed TP53 missense mutations in 4 (20%) of 20 cerebellar liponeurocytomas, a frequency higher than in medulloblastomas. There was no case with PTCH, APC, or beta-catenin mutations, each of which may be present in subsets of medulloblastomas. Isochromosome 17q, a genetic hallmark of classic medulloblastomas, was not observed in any of the cases investigated by FISH analysis. cDNA array analyses were carried out on 4 cerebellar liponeurocytomas, 4 central neurocytomas, and 4 classic medulloblastomas. Cluster analysis of the cDNA expression data of 1176 genes grouped cerebellar liponeurocytomas close to central neurocytomas, but distinct from medulloblastomas. These results suggest cerebellar liponeurocytoma as a distinct tumor entity that is genetically different from medulloblastoma. Furthermore, the cDNA expression array data suggest a relationship to central neurocytomas, but the presence of TP53 mutations, which are absent in central neurocytomas, suggests that their genetic pathways are different.
The authors report a case of paraplegia caused by a lumbar intraspinal paracoccidioidomycosis (PCM) granuloma. Clinical neurological diagnosis of a compressive spinal cord lesion was confirmed by spinal magnetic resonance imaging (MRI). Patient was submitted to surgery with total excision of the lesion. Histopathological analysis confirmed the diagnosis of PCM. Patient is on sulfamethoxazole/trimethoprim combined with fluconazole and is experiencing positive neurological recovery.
Subependymomas are benign tumors that occur predominantly in the ventricular system. We describe a case of a 57-year-old man with a large cerebellopontine angle (CPA) tumor which expanded into the jugular foramen. Complete surgical excision of the tumor was achieved through a retrosigmoid approach and the histopathological diagnosis was subependymoma. Subependymomas located exclusively in the CPA without extension into the fourth ventricle are extremely rare. The mainly pathological features and the difficulty in correctly diagnosing these cases preoperatively, even with MRI, are discussed.
-The term "cerebellar liponeurocytoma", recently adopted by the World Health Organization Working Group (WHO), replaced many other different terms used up to now to give name to this rare tumor. To our knowledge, less than 20 cases have been related up to now under different names like as "lipomatous medulloblastoma, lipidized medulloblastoma, neurolipocytoma, medullocytoma and lipomatous glioneurocytoma". The new nomenclature eliminates the word "medulloblastoma", reinforces its benign caracter, and includes it in the category of glioneuronal tumors. We describe an adictional case of this distinct clinico-pathological entity removed from the right cerebellar hemisphere of a 53-year-old woman. With the present case report, we hope to contribute to the knowledge on the diagnostic and prognostic implications derived from the finding of mature adipose-like tissue within a medulloblastomatous tumour.KEY WORDS: liponeurocytoma, medulloblastoma, adipose tissue, mixed tumour. Liponeurocitoma cerebelar: uma nova entidade clínico-patológicaRESUMO -O termo "liponeurocitoma cerebelar" recentemente adotado pela Organização Mundial de Saúde (Classificação de Tumores Cerebrais -versão 2000), surgiu em substituição a vários outros utilizados até então, para denominar esta rara neoplasia. De nosso conhecimento há na literatura menos de 20 casos relatados sob termos diferentes tais como "meduloblastoma lipomatoso, meduloblastoma lipidizado, neurolipocitoma, medulocitoma e glioneurocitoma lipomatoso". A nova nomenclatura elimina a palavra "meduloblastoma", enfatiza seu caráter benigno e o coloca na categoria dos tumores glioneuronais. Descrevemos mais um caso desta rara entidade clínico-patológica, ressecada do hemisfério cerebelar direito em uma mulher de 53 anos. Com este caso esperamos contribuir para o melhor conhecimento sobre o diagnóstico, prognóstico e possibilidades terapêuticas advindas da presença de tecido adiposo em tumor meduloblastomatoso de adultos. PALAVRAS-CHAVE: liponeurocitoma, meduloblastoma, tecido adiposo, tumor misto.
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