The Spectrum of Neurogenic Tumors of the Sympathetic Nervous System: Maturation and Adrenergic Function

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Abstract
The spontaneous maturation of fully malignant neuroblastomas to benign ganglioneuromas has been observed sporadically since the now famous case of Gushing and Wolbach reported in 1927. More recently, tumors of the neuroblastoma family have been observed to produce significant amounts of catechol amines resulting in hypertensive or diarrheal syndromes. The present study reviews all the cases belonging to this group of tumors seen at The Johns Hopkins Hospital and selected only on the basis of availability of histologic material and follow-up information. Of the 66 cases, 30 showed clear evidence of maturation. Of these 30, 11 had completed the maturation to the structure of benign ganglioneuroma. The maturation phenomenon is judged to be the single most important factor influencing the clinical behavior of the tumor. Maturation correlates directly with surgical resectability and eventual salvage. Examples of dramatic responses to X ray and/or nitrogen mustard therapy in the immature and clinically inoperable group are cited. Maturation to ganglioneuroma is observed at the sites of metastasis. The observations correlate very well with recently reported in vitro studies. Ten of the 66 cases showed clinical evidence of significant catechol amine production. This phenomenon is observed in the entire spectrum of tumors and does not correlate apparently with morphologic maturity.