662Cerebellar liponeurocytoma (LPN) is a rare central nervous system tumour recently recognized as a distinct clinicopathological entity. It was included in the previous 2000 edition of the World Health Organization (WHO) classification as a grade I neoplasm under the heading of glioneuronal tumours 1 . With increasing recognition of this entity and longer clinical follow-up periods in its investigation, it has become clear that this tumour has a rate of recurrence that is higher than previously thought and not compatible with a grade I designation. In light of the substantial rate of tumour recurrences, the current 2007 WHO classification assigns the cerebellar liponeurocytoma to WHO grade II tumour 2 . In this paper, we report a new case of a recurrent cerebellar LPN with supratentorial extension despite persistently benign histological features. To the best of our knowledge, recurrent cerebellar LPN with supratentorial extension has never been reported before, as the nine cases of recurrent cerebellar LPN published to date were confined to the posterior fossa. CLINICAL HISTORYA 42-year-old previously healthy man, presented with a fivemonth history of headaches, vertigo, visual disturbance and progressive unsteadiness of gait. On admission, neurological examination showed signs of cerebellar dysfunction, including ataxia, wide-based gait, right dysmetria on finger-to-nose testing and a positive Romberg sign. The fundus examination revealed unilateral papilloedema. Computed tomogram scan ( Figure 1) demonstrated a hypodense lesion within the vermis and right cerebellar hemisphere compressing the 4th ventricle and enhancing after contrast injection. Magnetic resonance imaging (MRI) scan showed a heterogeneous mass measuring 5 × 4 cm, characterized by discrete hyperintensity on T1-weighted images and high signal intensity on T2-weighted images. Several central parts of the lesion were very hyperintense on both T1 and T2-weighted sequences suggesting fat content. An intra-operative frozen section was reported as being consistent with a medulloblastoma. A gross total resection of the tumour was achieved. Histological examination of the surgical specimen revealed cerebellar tissue infiltrated by a highly cellular tumour formed by sheets of isomorphic small round cells (Figure 2). A significant proportion of the tumour demonstrated lipomatous differentiation characterized by large areas of cells containing a single cytoplasmic vacuole displacing the nucleus to the periphery mimicking mature fat cells (Figure 2A). There were only occasional mitotic figures and a low rate of proliferation Figure 2B) and neuron-specific enolase. Glial fibrillary acidic protein immunopositive tumour cells confirmed the presence of an additional glial component. All of these features were considered to be consistent with the diagnosis of cerebellar liponeurocytoma. A post-operative MRI performed three days after the surgery, demonstrated no residual tumour. No adjuvant radiation therapy was administered post-operatively. At the last rout...
Esophageal involvement in Behcet's disease is very rare, and normally is observed as aphtosis and esophagitis, but serious complications such as erosions, perforations, and stenosis may occur. We carried out this prospective study to evaluate the prevalence of esophageal involvement in Behcet's disease and to establish if routine endoscopy and/or manometry are necessary. Twenty-three patients who fulfilled the diagnostic criteria of the international study group for Behcet's disease were enrolled. None were taking drugs or had disease that might produce esophageal abnormalities or alter any existing changes due to the Behcet's disease itself. Twenty- three patients underwent esophagogastroduodenoscopy by a single observer. Esophageal biopsies were performed in thirteen patients and esophageal manometry in twenty. At the beginning of the study, the disease activity was defined by the presence of more than one symptom related to Behcet's disease, and upon the classification of Behcet's disease. Of the twenty three patients enrolled two were excluded from final analysis because of the presence of hiatus hernia. Thus, 13 men and 8 women, ranging in age from 20 to 63 years with a mean age of 36.2 years were included. Ten patients (47.6%) had active disease and four (19%) complained of upper gastrointestinal symptoms at the time of the study. Fourteen patients had endoscopic, manometric and/or microscopic abnormalities. Esophageal manometry was performed in twenty patients and was abnormal in 7 cases (35%). Esophageal biopsies were done in 13 patients and revealed histopathological abnormalities in 5 cases. Microscopic findings showed vasculities in one case, and non-specific inflammatory infiltration mainly consisting of neutrophils in 4 cases. Our results suggest that the prevalence of esophageal involvement in Behcet's disease is rather high and occur even in asymptomatic patients, but that this usually does not result in specific abnormalities.
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