Ganglioneuroblastoma (GNB) represents an intermediate entity in the spectrum of neuroblastic tumors derived from neural crest cells, bridging the malignant neuroblastoma and benign ganglioneuroma. Primary pulmonary involvement by GNB is extremely rare, with very few cases reported in the literature. We report a case of a three-year-old female child who presented with cough and rapid breathing for two to three days. Imaging revealed a well-defined pleural-based mass in the right lower lobe of the lung, initially suspected to be an inflammatory myofibroblastic tumor or pulmonary pleuroblastoma. Histopathological examination demonstrated a biphasic pattern comprising mature ganglion cells and immature neuroblasts within Schwannian stroma, consistent with ganglioneuroblastoma. Immunohistochemistry showed positivity for S-100 and neuron-specific enolase (NSE), confirming neural and Schwannian differentiation. This case underscores the diagnostic challenge of identifying GNB in atypical thoracic locations, where clinical and radiologic findings are nonspecific. Recognition of characteristic histological features and appropriate immunohistochemical profiling are essential for accurate diagnosis and classification. Our report adds to the limited literature on primary pulmonary GNB and emphasizes the need for multidisciplinary evaluation in such rare presentations.
Gill et al. (Fri,) studied this question.