Keratoameloblastoma: a tumor sui generis or a chimera?

The term keratoameloblastoma has been used to describe a histologically heterogeneous group of ameloblastoma variants which have in common the formation of keratin by the ameloblastomatous epithelium. The English language literature contains reports of only 12 cases of keratoameloblastoma, of which 4 cases exhibited a papilliferous component. We report a unique tumor that we believe falls within the broad histopathologic spectrum of keratoameloblastoma. We review the key clinical and histopathologic features of the previously reported cases of keratoameloblastoma and present an additional case that presented as an expansile, radiolucent lesion with internal opacification between the roots of teeth in the left anterior maxillary alveolar ridge of a 45-year-old white male. There is wide variation in the histopathologic appearance of cases reported under the appellation keratoameloblastoma. Our case exhibited a histopathologic feature shared by only 2 of the previously reported cases, notably islands and anastomosing cords of epithelium forming lamellated, pacinian-like stacks of parakeratin that extruded into the collagenous tumor stroma without eliciting a foreign body response. Due to the small number of reported cases, we are unable to accurately assess whether the biologic behavior of keratoameloblastoma differs from other histologic types of ameloblastoma.     

Enhanced MYCN expression and lsochromosome 17q in pineoblastoma cell lines

We have established two cell lines, PER-452 and PER-453, from an 8-month-old girl with an extensive pineoblastoma. Characterization of these lines revealed that the proto-oncogenes MYC and MYCN were not amplified, but both cell lines showed MYCN expression comparable to a cell line with 200-fold MYCN amplification. Both cell lines contained an i( 17q). These results support the concept that pineoblastomas belong to a larger group of primitive neuroectodermal tumors of the central nervous system. These two cell lines provide a unique opportunity to investigate the molecular genetic mechanisms underlying these neoplasms further. Genes Chrom Cancer 9:129-135 (1994).© 1994 Wiley-Liss, Inc.     

Involvement of A1 adenosine receptors in the acquisition of fertilizing capacity

Ejaculated mammalian spermatozoa acquire competence to fertilize oocytes by a two-step process: capacitation followed by acrosome reaction. The biochemical and biophysical modifications occurring in vivo in the female reproductive tract can be reproduced in vitro, and previous studies have suggested a capacitative role for adenosine A(1) receptor (A(1)R). Mice with a targeted disruption of the Adora 1 gene (A(1)R-/- mice) provide a useful model for better understanding the role of the A(1)R in fertility. Murine spermatozoa express A(1)R in the head, neck, midpiece region, and tail. The number of capacitated spermatozoa incubated in human tubal fluid was significantly reduced in A(1)R-/- compared with A(1)R+/+ and A(1)R+/- spermatozoa. The difference between A(1) R+/+ and A(1)R-/- mouse spermatozoa was mainly in the time necessary to reach the maximum percentage of capacitation. A(1)R+/+ murine sperm obtained the full state of capacitation within 90 minutes whereas A(1)R-/- sperm required 240 minutes. Caffeine, a known antagonist of A(1) and A(2A) adenosine receptors, lowered the number of capacitated sperm and affected the time of capacitation in a dose-dependent manner, mimicking the effects of the lack of A(1) receptors. Although number, motility, and viability of A(1)R-/- murine sperm was not significantly different from A(1)R+/+ mouse spermatozoa, a significant reduction of the number of pups produced by A(1)R-/- male mice suggests that A(1) receptors must be fully operative to accomplish the optimal degree of capacitation and thereby fertilization.     

Clinical and manometric evaluation of continence after the Bacon two-stage pull-through procedure

Twenty-eight patients who underwent the Bacon pull-through operation were evaluated for continence by clinical and radiologic means. Six patients were available for anal manometry. Eighty-three percent of those patients followed for function did not have the sensation to defecate spontaneously. They had normal functioning of the external sphincter, failure of reflex inhibition of the internal sphincter, and decreased rectal compliance secondary to fibrosis of the presacral space. These factors created a distal high-pressure zone which, when combined with sensory loss due to transection of the levators, rendered the patient continent but without the ability to defecate spontaneously unless an enema was administered. Supported in part by a grant from the Dorothy Rider Pool Health Care Trust.     

Steroidogenic gene expression following D-aspartate treatment in frog testis

Previous studies have provided evidence that D-Asp plays a role in steroid-mediated reproductive biology in amphibians, reptiles, birds and mammals. To examine the molecular involvement of D-Asp on steroidogenic pathway regulation, we analysed the expression of StAR, P450 aromatase and 5αRed2 mRNAs in Pelophylax esculentus testis, either in relation to the reproductive cycle or D-Asp treatment. Basal StAR mRNA levels, as well as D-Asp and testosterone concentrations, were higher in reproductive than in post-reproductive frogs. D-Asp treatment increased StAR mRNA expression and immunolocalisation in both the reproductive and post-reproductive periods. In control testis, aromatase mRNA levels were higher in the post-reproductive period, but following D-Asp administration, they increased only in the reproductive period. The level of 5αRed2 mRNA was higher in reproductive frogs than in post-reproductive frogs, and it increased after D-Asp treatment only in the post-reproductive phase. Our results suggest that, in P. esculentus testis, D-Asp increases StAR mRNA in both periods, and P450 aromatase and 5αRed2 mRNAs at different points during the reproductive cycle.