Introduction

For almost 140 years physicians have been aware of the enigmatic tumor in young children called "neuroblastoma." In 1864 Virchow was the first to describe a child with an abdominal tumor which he designated as "glioma." For more than a century the glioma-tous aspect of neuroblastoma has been neglected,but it is now vehemently debated whether the stromal part is neoplastic (Mora et al. 2001) or reactive (Am-bros et al. 1996) in nature. Marchand disclosed in 1891 the common features of tumors from the sympathetic nervous system and the adrenal medulla. In 1901 Pepper described the prenatal metastases of an adrenal sarcoma to the liver with ascites, scrotal edema, anemia, and recurrent fever (Pepper type; Pepper 1901) which is presently known as stage-4S neuroblastoma, the prototype of a spontaneously regressing tumor. Hutchinson observed in 1907 the characteristic bilateral periocular hematomas with proptosis of the eyes as symptoms of orbital and skeletal metastases of an adrenal tumor (Hutchinson type; Hutchinson 1907), the prototype of a progressing,still widely treatment-resistant neu-roblastoma.

The term neuroblastoma was introduced in 1910 by Wright when he demonstrated that the tumor originated from embryonal neuroblasts of the sympathetic peripheral nervous system (Wright 1910). The ability of neuroblastoma for spontaneous maturation into ganglioneuroma was first described in 1927 by Cushing and Wolbach. The detection of catecholamine metabolites as tumor markers was first reported by Mason et al. (1957) in a patient with hypertension, a quite rare feature in this disease. Two years later, vanillylmandelic acid (VMA) was identified as one of the main metabolites and tumor markers (Greenberg 1957; Stickler et al. 1959). In 1971 Evans et al. proposed the first internationally accepted staging system (Evans et al. 1971). Schwab and coworkers (1983) detected MYCN amplification as an important molecular feature of cell lines and primary tumors which proved to be a reliable marker for indicating rapid tumor progression (Seeger et al. 1985) and is now considered an essential parameter for risk estimation.

This chapter describes the clinical presentation of children with neuroblastoma and a comprehensive international perspective of the current criteria that are used for diagnosis,staging, and risk classification. The data regarding clinical presentation are derived from the German experience during the past 24 years. Greater than 95% of all German neuroblastoma patients known to the central children's tumor registry were enrolled in national trials during this time period, and only those with the wrong diagnosis were excluded from analysis; thus, these clinical data can be considered representative of neuroblastoma in a Western country.

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