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.

Interstitial deletion of chromosome 11q in a pineoblastoma.

A case of pineoblastoma with an interesting cytogenetic abnormality is reported. Chromosomal analysis of cultured cells from the tumor of a 10-week-old white male revealed an interstitial deletion of the long arm of chromosome 11, del(11)(q13.1q13.5). Tumors of the pineal region are relatively rare, and this is the first report of a pineoblastoma with del(11q).

Fatal disseminated adenovirus infection featuring liver necrosis and prolonged viremia in an immunosuppressed child with malignant histiocytosis.

Truly disseminated adenovirus disease is rare and appears to be limited to patients with severe combined T and B cell deficiency. This report describes an 18-month-old child with malignant histiocytosis treated with chemotherapy who developed a fatal disseminated type 2 adenovirus infection. Histologic changes in the tissues of multiple organs as well as the presence of typical crystalline structures in sections of the liver seen with electron microscopy provided evidence of viral replication. Adenovirus was recovered from the involved organs and from daily serum samples obtained over a 2-week period prior to death. The possibility that the child may have had virus associated histiocytic disease prior to chemotherapy is discussed.

Ectasia of the rete testis with ipsilateral renal agenesis.

Scrotal masses are a common problem in children. Cystic lesions usually are found to be hydroceles, while solid masses suggest the possibility of neoplasia. A rare cause of a cystic scrotal mass is ectasia of the rete testis. A review of the literature reveals only 1 case similar to the patient herein described. Both patients had agenesis of the ipsilateral kidney, which explained on the basis of the embryologic overlapping of the genitourinary system. Our patient bears clinical similarity to the ACI rat and correlation is made with this animal model system. Excision of the multiloculated rete testis cyst, rather than orchiectomy, appears to be curative.

Hormonal bioassay of gonadotropin-producing hepatoblastoma.

Hepatoblastoma was associated with signs of isosexual precocity in an 11-month-old boy. Circulating gonadotropic hormone levels were elevated. Gonadotropic activity was demonstrated by bioassay in extracts of primary and metastatic tumor, and in media surrounding long-term tissue culture of the primary tumor. Morphology of the endocrine glands and bioassay of the pituitary gland obtained at autopsy indicated the presence of gonadotropins and other tropic hormones despite the physiologically abnormal hormone status. These ae the first direct studies of pituitary hormone content in this syndrome.

Group B beta hemolytic streptococcal sepsis and the idiopathic respiratory distress syndrome: a comparison.

The clinical features of 27 patients with early onset group B beta hemolytic streptococcus sepsis were reviewed. Fifteen presented with a clinical pattern indistinguishable from the idiopathic respiratory distress syndrome and were compared with 15 patients with IRDS. Rupture of membranes for greater than 12 hours prior to delivery occurred more often in patients with GBS (33%) than in the patients with IRDS (16%). Hypotension was more commonly seen in the patients with GBS (56%) than in the patients with IRDS (36%). There was no difference in the incidence of apnea or the respirator peak inspiratory pressure requirements between the two groups of patients, but there was a tendency for a decline in the total white blood cell count in the first 24 hours of life in those patients with GBS sepsis.

Disseminated immune deposits in lupus erythematosus.

Immunohistologic studies were performed on autopsy tissues of 2 patients with systemic lupus erythematosus. All tissues examined--the kidney, lung, spleen, liver, intestine, peritoneum, and choroid plexus--contained immune deposits. Antinuclear antibody concentration in immunoglobulin G eluted from lung and spleen tissue was higher than in serum immunoglobulin G. These findings support the assumption that in systemic lupus erythematosus the renal as well as the extrarenal lesions can be attributed to vascular deposition of immune complexes.

Prevention of hyaline membrane disease with plasminogen. A cooperative study.

In a double-blind, randomized study, 500 premature infants were treated with plasminogen or placebo intravenously within 60 minutes of birth. There was a substantial decrease in severe clinical respiratory distress, death caused by hyaline membrane disease, and total mortality in the plasminogen-treated infants as compared to the controls.

Anatomically corrected malposition of the great arteries (S, D, L).

Two autopsy-proved cases are presented of a rare form of congenital heart disease, anatomically corrected malposition (S, D, L). Anatomically corrected malposition means that despite the abnormal relationship between the great arteries, the aorta arises nonetheless above the anatomically left ventricle and the pulmonary artery originates above the anatomically right ventricle. (S, D. L) briefly indicates the segmental set or combination: situs solitus of viscera and atria (S), ventricular D-loop (D), AND L-MALPOSITION OF THE GREAT ARTERIES (L). These are the first cases of anatomically corrected malposition (ACM) in whom the presence of a subaortic muscular conus only has been documented pathologically. This established that ACM can occur with a subaortic conus, as well as with a bilateral conus. From the diagnostic standpoint, the distributions or epicardial courses of the coronary arteries are recommended as a helpful approach to ventricular identification. This diagnostic method has widespread angiocardiographic and surgical applications. Symbolic terminology, exemplified by anatomically corrected malposition (S, D. L) is brief and accurate. This approach may conveniently be applied to many other forms of comples congenital heart disease, and it considerably facilitates computer data processing.