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

The features of a primary pulmonary ganglioneuroblastoma occurring in an adult are presented. The tumour showed evidence of both 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 related tumour. A collarette of lymphocytes surrounded the tumour, and lymphocytic aggregates were prominent at the interface between neuroblastoma and ganglioneuroma. Despite widespread vascular invasion, the patient remains well and apparently tumour-free, 2 1/2 years post-resection. The appearances may represent a combined cellular and humoral host response, and a possible relationship of this response to tumour maturation is suggested. The potential role of immunostimulation in the treatment of neuroblastoma is discussed.