Primary pulmonary primitive neuroectodermal tumor metastasis to the pancreas: a rare case with seven-year follow-up

摘要

AbstractThere are only nine primitive neuroectodermal tumor (PNET) cases that have arisen in lung parenchyma without pleural or chest wall involvement in the literature. Here, we present a long–term survival case of pulmonary PNET. A pulmonary mass was detected in a 19-year-old man on a chest radiograph and computed tomography image. At the three-year follow-up, the mass had enlarged in diameter by two-fold. The lesion was resected via lower left lobectomy. Histologically, the tumor was composed of uniform cells with round nuclei and scanty cytoplasm arranged in lobules with rosettes and pseudorosettes formation. Immunohistochemically, the tumor was positive for CD99, vimentin, neuron specific enolase and chromogranin A, and negative for cytokeratins, CD3, desmin, and leukocyte common antigen. Pancreatic metastasis occurred sixteen months after the first surgery, which was managed by pancreatectomy. The patient has survived seven years after the mass was initially detected, and four years after the first lobectomy.