Cystic variant of calcifying epithelial odontogenic tumor.

Calcifying epithelial odontogenic tumor (CEOT) is a benign, locally aggressive odontogenic neoplasm characterized by sheets and nests of epithelial cells with deeply eosinophilic or occasionally clear cytoplasm, calcifications, and eosinophilic amorphous material that stains positive for amyloid. Although many cases of CEOT are associated with impacted teeth and occasionally appear radiographically as dentigerous cysts, a true cystic variant has not been previously reported. We report a 15-year-old white male with a large cystic maxillary lesion that filled most of the left maxillary sinus. It deformed the medial wall, the inferior orbital floor, and caused narrowing of the left inferior meatus. Histologically, the cystic lining showed characteristics of CEOT. An intraluminal component that featured histologic characteristics of CEOT was identified during surgery. The lesion was enucleated and the postsurgical course of the patient was uneventful. Because follow-up has been for less than 1 year, a meaningful long-term prognosis cannot be determined at present. However, the patient has not reported any symptoms or signs of recurrence during the follow-up period.     

Surgical management of massive cerebellar infarction.

Massive cerebellar infarction accounts for less than 2% of strokes. Unlike massive hemispherical infarctions, in pure cerebellar infarctions, the prognosis is better. This case report discusses, a 61 year old lady who presented with atrial fibrillation and a massive cerebellar infarction. Timely surgical intervention reversed the deterioration in neurological status. The indications for surgical management, based on review of the literature, is presented.     

9p21 and 13q14 dosages in ependymomas. A clinicopathologic study of 101 cases.

Ependymomas are glial neoplasms whose clinical behavior is difficult to predict based on histology alone. Recently, a comparative genomic hybridization study identified frequent chromosome 9p and 13q losses in anaplastic ependymomas, suggesting that p16 and RB alterations may be involved in tumor progression. In order to test this hypothesis further, 101 myxopapillary, conventional, and anaplastic ependymomas (51 spinal and 50 intracranial tumors) were tested for RB and p16 deletions using fluorescence in situ hybridization. Clinical follow-up, ranging from 2 to 198 months (median 46 months), was obtained in 90 cases (91%). RB and p16 deletions were seen in 22 of 92 (24%) and 22 of 89 (25%) informative cases, respectively. Polysomies were more frequent in the grade I and II spinal tumors, consistent with prior reports of increased aneuploidy in such cases. No significant genetic associations were seen with tumor grade, recurrence, or death, suggesting that 9p and 13q deletions do not play a prominent role in the malignant progression of ependymomas, as has been implicated in other glioma subtypes.     

Competing functions encoded in the allergy-associated F(c)epsilonRIbeta gene

Allergic reactions are triggered via crosslinking of the high-affinity receptor for immunoglobulin E, F(c)epsilonRI. In humans, F(c)epsilonRI is expressed as a tetramer (alphabetagamma(2)) and a trimer (alphagamma(2)). The beta subunit is an amplifier of F(c)epsilonRI surface expression and signaling. Here, we show that as a consequence of alternative splicing, the F(c)epsilonRIbeta gene encodes two proteins with opposing and competing functions. One isoform is the full-length classical beta, the other a novel truncated form, beta(T). In contrast to beta, beta(T) prevents F(c)epsilonRI surface expression by inhibiting alpha chain maturation. Moreover, beta(T) competes with beta to control F(c)epsilonRI surface expression in vitro. We propose that the relative abundance of the products of the beta gene may control the level of F(c)epsilonRI surface expression and thereby influence susceptibility to allergic diseases.