Burkitt's lymphoma of the parapharyngeal space.

Burkitt's lymphoma is quite rare outside of Africa. It is even more uncommon for this nonendemic form of the disease to present in the head and neck region. To our knowledge, nonendemic Burkitt's lymphoma has not been previously reported arising from the parapharyngeal space. We review the case of a 10-year-old boy who presented with an asymptomatic mass at the angle of the mandible several weeks after blunt trauma to that area. We discuss the evaluation of this mass, including the results of computed tomography and magnetic resonance imaging, along with the findings of histopathologic and cytogenetic studies. We also discuss the differential diagnosis of parapharyngeal lesions, paying particular attention to the workup and management of Burkitt's lymphoma. Since Burkitt's lymphoma has the highest growth rate of any tumor in man, rapid diagnosis and immediate treatment are important and likely to improve outcome.

Constitutional extra chromosomal element in a family with Wilms' tumor.

We report the presence of an extra chromosomal element in a family with Wilms' tumor (WT). This family has three children, two of whom were affected. One son, the proband, had bilateral and one daughter had unilateral WT. The first child, the father, and the mother did not have WT. The son with bilateral WT had a ring chromosome (R) both in the lymphocytes as well as in the kidney tissue. The size of the ring varied considerably from cell to cell. The daughter with unilateral WT had an abnormal clone containing a small chromosomal ring (r) in phytohemagglutinin (PHA)-stimulated and Epstein-Barr virus (EBV)-transformed lymphocytes. The mother had a karyotype similar to that of the daughter with WT. We hypothesize that the proband's ring chromosome could be the amplified form of the r inherited from the mother. Chromosome 11 was cytogenetically normal in all the cells examined of the affected children and the unaffected mother. In situ hybridization with a centromere-specific DNA cocktail indicated dispersed centromeric DNA both in r and R.

Quantitative study of the early effects of tracheotomy and endotracheal intubation on the rabbit tracheobronchial tree.

Histopathologic evaluation of the tracheobronchial tree was performed in 12 rabbits to study the effects of 1 hour of tracheotomy or endotracheal intubation. In five animals who underwent oral endotracheal intubation, epithelial erosions and submucosal inflammation in the proximal (bronchial) airway covered 34.9% and 71.3% of the luminal circumference, respectively. This was highly significant (p less than .001) against two control animals, in which 4.8% erosion and 3.9% inflammation were observed. Tracheotomy in three animals caused 22.8% bronchial epithelial erosion and 46.0% inflammation, which were not significantly greater than values of 15.0% and 20.6% observed following "sham" tracheotomy. The severity of the proximal (indirect) consequences of endotracheal intubation may have been attributable to the lack of humidification, but this does not explain the lesser damage caused by tracheotomy. It is postulated that increased inspiratory flow rate and pressures occurring in endotracheally intubated animals may be contributory factors.

Chronic pancreatitis with diffuse fibrosis in early childhood.

A 2-year old girl is described in which biliary obstruction was the initial presentation of chronic pancreatitis. A pancreatic biopsy done at laparotomy showed extensive fibrosis not explained by her short clinical course. Chronic pancreatitis with fibrosis in childhood is reviewed.

Massive oropharyngeal papillomatosis causing obstructive sleep apnea in a child.

Obstructive sleep apnea in children is most often secondary to severe adenotonsillar hypertrophy. A 5 1/2-year-old boy presented with loud snoring, increasing dysphagia, nocturnal choking, and apnea. Extensive papillomatosis of the uvula, soft palate, and nasopharynx was found to be causing the obstruction. Although multiple papillomas of the larynx and tracheobronchial tree are well known, the occurrence of extensive papillomas of the oral cavity is rare. The unusual clinical presentation and pathologic significance are discussed.

Heterotopic growth of dysplastic cerebellum in frontal encephalocele in an infant of a diabetic mother.

An infant of a diabetic mother lived 13 days after birth. She had a small dysplastic 12.6 gm. brain, partly forming a frontal encephalocele. The uncleaved forebrain contained a mass of poorly organized heterotopic cerebellar cortex. The cerebellum itself had normal lamination, but was small and continuous with the dysplastic tissue. The ventricular system was absent except for a few midline ependymal rosettes, and the cerebral cortex was not developed. The cerebellar dysplasia resembled a proliferative and invasive lesion by its rostral extension.

Diagnostic value of histochemistry in embryonal rhabdomyosarcoma.

Embryonal rhabdomyosarcomas from the nasopharynx of two children were examined by histochemical methods commonly applied to muscle biopsies. These stains included nicotinamide adenine dinucleotide-tetrazolium reductase (NADH-TR), succinate dehydrogenase (SDH), PAS, PAS-diastase, myophosphorylase, calcium-mediated adenosine triphosphatase (ATPase) preincubated at high and low pH, and oil red O. Myofibrils were easily identified with ATPase and blood vessel walls were also stained. NADH-TR clearly showed longitudinal and cross-striations that were not seen with H&E or PTAH stains. The modified Gomori trichrome stain additionally contributed to the recognition of myofibrils. Some techniques of muscle histochemistry applied to fresh frozen sections of tumor tissue may provide evidence of muscular differentiation in otherwise poorly differentiated sarcomas for a more accurate diagnosis of rhabdomyosarcoma.

Immune complex disease. VII. Experimental mesangiopathic glomerulonephritis produced by chronic immunization with thyroglobulin.

Immunohistologic and electron microscipic studies were performed on the kidneys of rabbits given daily intravenous injections of porcine thyroglobulin in amounts adjusted to the immune response of the individual rabbits. Glomerular lesions were restricted to the mesangium, were characterized by varying degrees of proliferation of mesangial cells and increase of mesangial matrix, and were accompanied by accumulations of rabbit immunoglobulins, C3, and porcine thyroglobulin. Electron-dense deposits were localized to the mesangium and the adjacent subendothelial space. Less than 10 per cent of the animals with mesangila lesions developed obvious impairment of glomerular function. Thyroglobulin-containing immune complexes were found to be rapidly removed from the mesangium, so that overloading of the mesangium and consequent accumulation of complexes in the adjacent capillary loops could not occur. Thus, the results provide further evidence that when immune complex deposition is restricted to the mesangium, relatively little interference with glomerular function results. This situation is paralleled in man by the lesions of subclinical lupus nephritis, chance proteinuria and hematuria, and the early lesions of Berger's disease.

Immune complex disease. VI. Some determinants of the varieties of glomerular lesions in the chronic bovine serum albumin-rabbit system.

Glomerular lesions were evaluated by light, electron, and immunofluorescence microscopy in rabbits given daily injections, for prolonged periods of time, of either 12.5 mg. of bovine serum albumin (BSA) (group I) or increasing doses of BSA, paralleling the immune response (group II). In animals clearing 12.5 mg. or more of BSA per day (excluding those dying of anaphylaxis or unknown causes), marked differences were noted in the incidence of membranous glomerulonephritis (GLN) (50 per cent in group I versus 15.4 per cent in group II), of crescentic GLN (none in group I versus 26.9 per cent in group II), and of mesangiopathic GLN (11.1 per cent in group I versus 30.8 per cent in group II). On the basis of these variations and immunofluorescence microscopy findinds, it is concluded that the occurrence of membranous GLN is favored by the presence in the circulation of small concentrations of soluble immune complexes, whereas crescentic GLN results from the presence of high concentrations of such complexes. When BSA injections were discontinued in group I animals having membranous GLN, the immune deposits were sequestered by membranous transformation so that they were not readily solubilized by injections of large amounts of BSA; one such animal developed crescentic GLN. The observation that BSA was difficult to detect in the lesions of mesangiopathic GLN suggests that this lesion results from the deposition of complexes formed at equivalence or in antibody excess.