title: A retroperitoneal sympathetic paraganglioma invading the duodenum and mimicking a submucosal tumor.
abstract: We report a case of an autopsy of unusual retroperitoneal sympathetic paraganglioma (SPG) that directly invaded the duodenum and showed expansive growth mimicking a submucosal tumor. The tumor was clinically suspected to be a gastrointestinal stromal tumor (GIST) of the duodenum because of its location and extension to the retroperitoneum without catecholamine-associated symptoms. However, a small biopsy specimen of the tumor showed diffuse proliferation of large basophilic cells that were negative for C-kit and CD34, ruling out GIST and indicating an epithelial malignancy. An autopsy revealed that the tumor was mainly in the retroperitoneum, measuring 7.5 x 9.5 cm, weighing 600 g and extending into the duodenum, adjacent to the pancreas but free of the adrenal glands. On cut section, the tumor involved the entire wall of the duodenum. There were no metastases in any organs. For differential diagnosis, endocrine tumors of the duodenum or pancreas and extra-adrenal SPG were considered. The tumor cells were immunohistochemically strongly positive for chromogranin A and were surrounded by cells positive for S100 protein. The Ki67-labeling index was under 1%. The four catecholamine-synthesizing enzymes were detected in the tumor cells. We report this case of SPG with emphasis on differential diagnosis and the significance of its local invasion.
authors.affiliation: Department of Pathology, Hakodate Hospital, Japan National Hospital Organization, 16-18 Kawahara, Hakodate, Hokkaido, Japan. kimura-path@hnh.hosp.go.jp
authors.forename: Noriko
authors.initials: N
authors.lastname: Kimura
authors.forename: Takuzo
authors.initials: T
authors.lastname: Ishidate
authors.forename: Takahiro
authors.initials: T
authors.lastname: Kogawa
authors.forename: Yukio
authors.initials: Y
authors.lastname: Miura
authors.forename: Masanori
authors.initials: M
authors.lastname: Ishizaka
authors.forename: Masami
authors.initials: M
authors.lastname: Ogita
mesh_terms: D000369:Aged, 80 and over; D001342:Autonomic Nervous System Diseases / Q000378:metabolism / Q000473:pathology*; D001344:Autopsy; D002395:Catecholamines / Q000378:metabolism; D049109:Cell Proliferation; D003937:Diagnosis, Differential; D004299:Dopamine beta-Hydroxylase / Q000378:metabolism; D004379:Duodenal Neoplasms / Q000378:metabolism / Q000473:pathology / Q000556:secondary*; D017809:Fatal Outcome; D006801:Humans; D007150:Immunohistochemistry; D019394:Ki-67 Antigen / Q000378:metabolism; D008297:Male; D009423:Nervous System Neoplasms / Q000378:metabolism / Q000473:pathology*; D016612:Paraffin Embedding; D010235:Paraganglioma / Q000378:metabolism / Q000473:pathology / Q000556:secondary*; D010625:Phenylethanolamine N-Methyltransferase / Q000378:metabolism; D011014:Pneumonia / Q000209:etiology / Q000473:pathology; D012186:Retroperitoneal Neoplasms / Q000378:metabolism / Q000473:pathology / Q000556:secondary; D014446:Tyrosine 3-Monooxygenase / Q000378:metabolism
chemical_list: D002395:Catecholamines; D019394:Ki-67 Antigen; D014446:Tyrosine 3-Monooxygenase; D004299:Dopamine beta-Hydroxylase; D010625:Phenylethanolamine N-Methyltransferase