Roentgen Therapy in Neuroblastoma

Abstract

In the neuroblastomas of infancy and childhood the physician is dealing with a multipotential tumor which may be curable. The outcome will in part depend upon the localization of the primary lesion, the presence and distribution of metastases, and the selection of the type of therapy. Neuroblastoma was first described by Virchow (1) in 1864 and classified as a glioma. The clinical picture was described by Pepper (2) in 1901 and Hutchison (3) in 1907, and their names have become associated with two clinical types based upon the distribution of the metastases, the adrenal hepatic distribution of the tumor being subsequently known as Pepper's type and the adrenal skeletal, specifically with skull metastases, as the Hutchison type. Studying the suprarenal tissues of embryos, Wright (4) in 1910 first recognized the similarity of the cells to those of these tumors and concluded that the origin must be from the sympathetic nervous system. Farber (5) in 1940 and Karsner (6) in 1942 finally dispelled the concept of the Pepper and Hutchison types of neuroblastoma, showing that the distribution of the metastases bears no relationship to the cytological character or the anatomical site of the primary growth. In a recent (1942) review article, Rosendal (7) estimates the total cases of neuroblastoma reported in the literature to be approximately three hundred. Little has been written, however, on the therapy of this tumor. Farber (5), in 1940, was the first to report that “permanent cures” could occur in neuroblastoma patients. He pointed out, in a study of 40 cases, that neuroblastoma may undergo spontaneous hemorrhage and necrosis, disappearing without treatment, and that the tumor may also undergo spontaneous maturation, becoming a benign ganglioneuroma. Wyatt and Farber (8) in 1941 amplified this study, recommending radiation therapy “in every case after the diagnosis is established.” The basis for this recommendation was the fact that of 10 children (of a series of 40) with neuroblastoma who received x-ray therapy as part or all of their treatment, 5 survived two to five years, while among the 30 receiving all other forms of therapy, but no x-ray, the number of two- to five-year survivals was also 5. Although statistically not significant, these figures are suggestive that x-ray therapy is beneficial. The medical literature fails to yield any more decisive information on the value of irradiation in neuroblastoma. Scope of Review At The Children's Medical Center of Boston, 143 neuroblastomas are recorded to 1949. The patients were seen under a variety of circumstances and were handled in many different ways. Six cases lack absolute microscopic confirmation on review of material now available. Twenty lack sufficient clinical data for tabulation (some sent in for postmortem study only, some with death shortly after admission without postmortem examination, etc.).