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Previous studies have shown that catecholamine secretion patterns have been imperfect predictors of clinical behavior of neuroblastomas. Recently, studies of nuclear DNA content in neuroblastoma have shown that an aneuploid DNA content predicts favorable clinical behavior. To determine if a correlation exists between these tumor biologic indicators, the authors analyzed both in a series of 39 patients with neuroblastoma. Flow cytometric DNA analysis performed on paraffin blocks determined that 23 patients had tumors with aneuploid DNA content (aneuploid tumors) and 16 patients showed no demonstrable anomalies of tumor DNA content (nonaneuploid tumors). Comparison of catecholamine levels in urine and tumor homogenates with DNA content data indicate that nonaneuploid neuroblastomas include a significant number (P less than 0.02) of biochemically primitive tumors which secrete high levels of 3,4 dihydroxyphenylalanine (DOPA), dopamine and homovanillic acid (HVA). This suggests a dopamine-norepinephrine pathway block, which supports previous reports of deficiency of dopamine beta-hydroxylase activity in some neuroblastomas. The study shows that in contrast to aneuploid tumors, nonaneuploid neuroblastomas secrete higher levels of early pathway catecholamine metabolites and are more likely to present in higher (unfavorable) clinical stages of disease.

A newborn male presented with respiratory distress and laryngeal stridor at the time of birth. Laryngoscopy revealed a circumscribed mass in the right vocal cord which was diagnosed as an undifferentiated malignant neoplasm on frozen section. Further light microscopic studies, special stains and electron microscopy disclosed features consistent with a special subtype of sarcoma adopted by the Intergroup Rhabdomyosarcoma Committee. Several authors have also demonstrated similarities between this type of tumor and soft tissue Ewing sarcomas. Nonepithelial malignancies of the larynx are rare in children and are only anecdotally reported in newborns. Histopathologically, the tumors predominantly include rhabdomyosarcomas among other rarer less well documented sarcomas and lymphomas. Although modern chemotherapy and radiotherapy have improved the otherwise grim prognosis of soft tissue Ewing sarcoma, this young patient was only treated with total laryngectomy at ten days of age and is alive and well two years later.

This study was undertaken to better define the relationship between catecholamine (CAT) metabolism within the malignant neuroblast and other factors known to influence clinical outcome in neuroblastoma (NB). Several CATs and their metabolites were measured in 32 tumors and 47 urines from 60 newly diagnosed children with neurogenic tumors. Absolute and relative CAT concentrations were correlated with age, clinical stage, histologic differentiation and survival duration. The major findings are: (1) urinary CAT excretion patterns often differ markedly from tumor CAT patterns in the same child, which may be explained by the continuous nature of tumor metabolism, hepatic and renal catabolism, and sampling errors; (2) definite patterns of biochemical maturation are apparent in tumors and urines, but standard light microscopy is insufficient to correlate this with degree of histologic differentiation; (3) more differentiated patterns of CAT metabolism, both in tumor and urine, are significantly associated with age less than 1 year at diagnosis, and with more favorable clinical Stage (I, II, IVS). Tumor NE was increased relative to DM and DA, and urine NMN and VMA were increased relative to DM and HVA, in these patients. (4) this more differentiated CAT pattern in infants and in less advanced stages was reflected in their better survival. Greater maturity of tumor enzyme systems is the probable reason for the better prognosis of NB in infants less than 1 year. Individual enzymes should be measured in fresh tumor tissues, and related to relative and absolute CAT concentrations in tumor and urine, to try to categorize patients on the basis of clearcut biological differences when planning new treatment strategies for NB.

Periodic acid-Schiff-positive diastase-resistant globules are described in an undifferentiated (embryonal) sarcoma of the liver in a 14-year-old boy. These globules were shown to contain alpha-1-antitrypsin (A-1-AT) protein by immunofluorescence techniques. Further immunohistochemical studied revealed albumin and gamma globulins, but no alpha-feto protein (AFP). Electron microscopic studies showed large, electron-dense bodies consistent with phagolysosomal structures, and distinct from that described in alpha-1-antitrypsin deficiency (A-1-ATD). These findings suggested that the globules in this tumor may represent trapping of serum proteins by the malignant cell. These observations are discussed in connection with recent studies linking the deficiency disease and epithelial hepatic tumors.

Adrenal cortical carcinoma is an uncommon neoplasm in children. Only a handful of congenital adrenal cortical carcinoma cases have been described. A newborn who had metastatic adrenal cortical carcinoma (skin metastases and cerebral lesions) is described. This patient underwent surgical resection of the right adrenal primary, but no further treatment was given. Hemihypertrophy developed in this patient by 2 months of age, and at 4 months of age spontaneous regression of all skin nodules and central nervous system (CNS) lesions was observed. Follow-up at 1 year shows the patient to be alive, well, and disease-free. Evaluation of the tumor included DNA ploidy analysis that showed the tumor to be polyploid, a pattern recently associated with nonmetastasizing adrenal cortical neoplasm. The observation of apparent metastatic disease that regressed spontaneously highlights the prognostic value of DNA ploidy analysis and raises the possibility of an adrenal tumor with properties similar to those of Stage IV-S neuroblastoma.