Extra-adrenal composite paraganglioma with ganglioneuroma component presenting as a pancreatic mass.
Paragangliomas may have composite forms in which they combine features of a typical paraganglioma with those of a neural component consisting of either neuroblastoma, ganglioneuroblastoma, or ganglioneuroma. These variants are rare and generally located in the adrenal. Herein, we describe a retroperitoneal, extra-adrenal composite paraganglioma-ganglioneuroma of a 57-year-old woman. Radiological evaluation revealed a nodular mass with apparent pancreatic location, with findings suggestive of an endocrine tumor, yielding the diagnosis of a pancreatic endocrine tumor. At histology the neoplasm, strictly adhering to the external surface of the pancreatic gland but well demarcated, displayed a main central region with typical paraganglioma features and cells arranged in cords and in a nesting "zellballen" pattern, positive for neuroendocrine markers, and a distinct peripheral area consisting of dense bundles of wavy spindled Schwann cells, with scattered ganglionic cells.
Inzani
Frediano
F
Department of Pathology, University of Parma, Via Gramsci 14, 43100, Parma, Italy. finzani@ao.pr.it
Rindi
Guido
G
Tamborrino
Ernesto
E
Cobelli
Rocco
R
Bordi
Cesare
C
D014408:Biomarkers, Tumor / Q000032:analysis; D001941:Breast Diseases / Q000150:complications / Q000601:surgery; D005260:Female; D005729:Ganglioneuroma / Q000150:complications / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007150:Immunohistochemistry; D007889:Leiomyoma / Q000150:complications / Q000601:surgery; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000150:complications / Q000473:pathology* / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000150:complications / Q000473:pathology* / Q000601:surgery; D010236:Paraganglioma, Extra-Adrenal / Q000150:complications / Q000473:pathology* / Q000601:surgery; D014057:Tomography, X-Ray Computed; D014594:Uterine Neoplasms / Q000150:complications / Q000601:surgery
D014408:Biomarkers, Tumor