Primary pulmonary ganglioneuroblastoma in an adult: maturation, involution and the immune response

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Abstract
The features of a primary pulmonary ganglioneuroblastoma occurring in a human adult are presented. The tumor showed evidence of maturation and involution. Maturation appeared to be occurring in a centrifugal manner, a rim of mature ganglioneuromatous tissue enclosing the primitive neuroblastoma. Necrosis of the neuroblastomatous element was widespread and associated with deposition in the walls of numerous small vessels of an amorphous eosinophilic amyloid-like material. Accumulation of this material had led to occlusion of some vessels with resultant necrosis of the related tumor. A collarette of lymphocytes surrounded the tumor and lymphocytic aggregates were prominent at the interface between neuroblastoma and ganglioneuroma. Despite widespread vascular invasion the patient remains well and apparently tumor-free 2 1/2 yr postresection. The appearances may represent a combined cellular and humoral host response and a possible relationship of this response to tumor maturation is suggested. The potential role of immunostimulation in the treatment of neuroblastoma is discussed.