HighlightsEwing sarcoma/primitive neuroectodermal tumor of the kidney (ES/PNET) is a member of Ewing’s sarcoma family, occurring in young adults.The clinical course and prognosis of ES/PNET are different from renal cell carcinoma (RCC).For definite diagnosis, in addition to cytogenetic analysis; other techniques may be needed; such as fluorescent in situ hybridization (FISH), reverse transcriptase-polymerase chain reaction (RT-PCR) of the t (11; 22) translocation [1].
Background: Relatively little is known with certainty about the status and role of p53 or MDM2 in predicting prognosis and survival of renal cell carcinoma. The present study aimed to determine the value of P53 and MDM2 over-expression, alone and simultaneously, to predict five-year survival of patients with kidney cancer in Iran. Materials and Methods: Patients with kidney cancer referred to Hasheminejad Kidney Center between 2007 and 2009, underwent radical nephrectomy and had pathology reports of clear cell, papillary or chromophobe renal cell carcinoma were included in our cohort study. Other histological types of renal cell carcinoma were not included. The patients with missed, incomplete or poor quality paraffin blocks were also excluded. Overall ninety one patients met the inclusion and exclusion criteria. To assess the histopathological features of the tumor, immunohistochemical (IHC) staining of formalin fixed, paraffin-embedded tumor samples were performed. The five-year survival was determined by the patients' medical files and telephone following-up. Results: In total, 1.1% of all samples were revealed to be positive for P53. Also, 20.8% of all samples were revealed to be positive for MDM2.The patients were all followed for 5 years. In this regard, 5-year mortality was 30.5% and thus 5-year survival was 85.3%. According to the Cox proportional hazard analysis, positive P53 marker was only predictor for patients' 5-year survival that the presence of positive p53 increased the risk for long-term mortality up to 2.8 times (HR=2.798, 95%CI: 1.176-6.660, P=0.020). However, the presence of MDM2 could not predict long-term mortality. In this regard, analysis by the ROC curve showed a limited role for predicting long-term survival by confirming P53 positivity (AUC=0.610, 95%CI: 0.471-.750, P=0.106). The best cutoff point for P53 to predict mortality was 0.5 yielding a low sensitivity (32.0%) but a high specificity (97.9%). In similar analysis, measurement of MDM2 positivity could not predict mortality (AUC=0.449, 95%CI: 0.316-.583, P=0.455). Conclusions: The simultaneous presence of both P53 and MDM2 markers in our population is a rare phenomenon and the presence of these markers may not predict long-term survival in patients who undergoing radical nephrectomy.
Background
Gliomatosis cerebri (GC) is a rare diffuse infiltrative growth pattern of gliomas presenting with unspecified clinical manifestations. Herpes simplex encephalitis (HSE) is a central nervous system (CNS) viral infection, usually involving temporal lobes, that can lead to acute or sub-acute cerebral dysfunction. Differentiating these entities is necessary for patients with misleading presentations and imaging findings. To the best of our knowledge, this is the first case of glioma mimicking herpes encephalitis, which has complicated by blindness.
Case Presentation
The patient was a 35 years-old male in a drug rehabilitation center for heroin addiction. He presented with a headache, a single episode of seizure, and a two-month history of bilateral decrease in visual acuity, which had acutely worsened. Magnetic resonance imaging (MRI) and computed tomography (CT) showed bilateral temporal lobe involvement, which when combined with the patient's demographic and history, led to empirical treatment for herpes simplex encephalitis being commenced. Unfortunately, no improvement in visual acuity was detected following this treatment. Ophthalmological studies showed bilateral papilledema, absence of visual evoked potential, and thickening of the retinal nerve fiber layer. Due to the atypical clinical presentation, normal laboratory data, and suspicious MRI findings, further investigation with magnetic resonance spectroscopy (MRS) was performed. Results showed a greatly increased ratio of choline to creatinine or N-acetyl aspartate (NAA), suggesting a possible neoplastic nature of the disease. Subsequently, the patient was referred for a brain tissue biopsy with a suspicion of malignancy. The pathology results revealed adult-type diffuse glioma with isocitrate dehydrogenase (IDH) mutation.
Conclusion
When presented with a young patient displaying acute or sub-acute neurological symptoms and bilateral temporal lobe involvement on MRI, it is common for clinicians to presume HSE or another viral encephalitis as the primary differential diagnosis. However, as demonstrated in this study, neoplastic lesions such as adult-type diffuse glioma must be considered, especially when the clinical and imaging findings are not completely typical for HSE.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.