title: Primary pulmonary ganglioneuroblastoma: a clinicopathologic and immunohistochemical study of two cases.
abstract: We present two patients with primary ganglioneuroblastoma involving the bronchial wall. The first, a 38-year-old woman, presented with signs and symptoms suggestive of multiple endocrine neoplasia, including gastric ulceration and hypercalcemia. Chest radiographic studies revealed a 3-cm nodule in the hilus of the right lung and two less-pronounced lesions in the periphery of the right lung. The second, a 20-year-old asymptomatic woman, was evaluated for a solitary mass in the upper lobe of the left lung that was peribronchial and that impinged on the lumen of a bronchus. Grossly, both neoplasms extended from bronchi, were well-circumscribed, firm, tan or white, and homogeneous, and measured 5 x 5 cm and 3 x 3 cm, respectively. Histologically, both tumors were characterized by neuroblastoma with areas of neuropil and multifocal areas of ganglion cells. Immunohistochemical studies performed in one case showed focal staining for neurofilament protein and S-100 protein and diffuse staining for neuron-specific enolase. Follow-up information showed that one patient died a few days after admission to the hospital; the second patient has remained well and without evidence of recurrence or metastases 1 year after initial diagnosis. These two cases confirm that ganglioneuroblastoma can occur as a primary pulmonary tumor in adults, presumably arising from sympathetic ganglia of the bronchus.
authors.affiliation: Department of Pulmonary and Mediastinal Pathology, Armed Forces Institute of Pathology, Washington, DC, USA.
authors.forename: L
authors.initials: L
authors.lastname: Hochholzer
authors.forename: C A
authors.initials: CA
authors.lastname: Moran
authors.forename: M N
authors.initials: MN
authors.lastname: Koss
mesh_terms: D000328:Adult; D001980:Bronchi / Q000294:innervation / Q000473:pathology; D005260:Female; D018305:Ganglioneuroblastoma / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology*; D006801:Humans; D008175:Lung Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology*; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications / Q000473:pathology; D011859:Radiography; D015043:Zollinger-Ellison Syndrome / Q000473:pathology