title: Primary pulmonary paraganglioma: report of a functioning case with immunohistochemical and ultrastructural study.
abstract: We describe a case of primary pulmonary paraganglioma, a tumor that has not been reported in sufficient detail in previous literature. The patient was a 55-year-old woman with hypertension accompanied by an elevated serum norepinephrine level (2651 pg/mL; normal 100-450 pg/mL). Computed tomography revealed a well-circumscribed solid mass, 3.5 cm in diameter, located in the lower lobe of the left lung. In the lobectomy specimen, the tumor had invaded the B8 bronchus and hilar lymph nodes with microscopic metastasis to the mediastinal nodes. The tumor showed histologic, immunohistochemical, and ultrastructural features of paraganglioma: argyrophilic cells arranged in a nesting (Zellballen) or anastomosing trabecular pattern within an arcuate vascular network. Neoplastic chief cells positive for neuroendocrine markers (CD56, synaptophysin, chromogranin A) were surrounded by sustentacular cells positive for S-100 protein. Neurofilament protein was positively stained, but cytokeratins were totally negative. On electron microscopy, chief cells possessed abundant dense core granules with an eccentric halo ("norepinephrine-type" granules). The patient's blood pressure began to decline soon after the resection, and her serum norepinephrine promptly returned to almost normal. On the basis of our experience, our case is a bona fide primary pulmonary paraganglioma, a tumor heretofore subject to considerable skepticism.
authors.affiliation: Department of Pathology, Graduate School of Medicine, University of Tokyo, Tokyo, Japan. jshiba-tky@umin.ac.jp
authors.forename: Junji
authors.initials: J
authors.lastname: Shibahara
authors.forename: Akiteru
authors.initials: A
authors.lastname: Goto
authors.forename: Toshiro
authors.initials: T
authors.lastname: Niki
authors.forename: Makoto
authors.initials: M
authors.lastname: Tanaka
authors.forename: Jun
authors.initials: J
authors.lastname: Nakajima
authors.forename: Masashi
authors.initials: M
authors.lastname: Fukayama
mesh_terms: D019002:CD56 Antigen / Q000032:analysis; D053379:Chromogranin A; D002864:Chromogranins / Q000032:analysis; D003594:Cytoplasmic Granules / Q000648:ultrastructure; D005260:Female; D006801:Humans; D006973:Hypertension / Q000209:etiology; D007633:Keratins / Q000032:analysis; D008175:Lung Neoplasms / Q000737:chemistry* / Q000601:surgery / Q000648:ultrastructure*; D008207:Lymphatic Metastasis / Q000648:ultrastructure; D008875:Middle Aged; D016900:Neurofilament Proteins / Q000032:analysis; D009638:Norepinephrine / Q000097:blood; D010235:Paraganglioma / Q000737:chemistry* / Q000601:surgery / Q000648:ultrastructure*; D009418:S100 Proteins / Q000032:analysis; D016708:Synaptophysin / Q000032:analysis
chemical_list: D019002:CD56 Antigen; D053379:Chromogranin A; D002864:Chromogranins; D016900:Neurofilament Proteins; D009418:S100 Proteins; D016708:Synaptophysin; D007633:Keratins; D009638:Norepinephrine