Extra-adrenal Composite Paraganglioma with Ganglioneuroma Component Presenting as a Pancreatic Mass

Authors: Inzani, Frediano¹; Rindi, Guido²; Tamborrino, Ernesto³; Cobelli, Rocco⁴; Bordi, Cesare²

Source: Endocrine Pathology, Volume 20, Number 3, September 2009 , pp. 191-195(5)

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Abstract:

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.

Keywords: composite paraganglioma; extra-adrenal paraganglia; chromaffin cells; immunohistochemistry; pancreas

Document Type: Research article

DOI: http://dx.doi.org/10.1007/s12022-009-9085-z

Affiliations: 1: Department of Pathology, University of Parma, Via Gramsci 14, 43100, Parma, Italy, Email: finzani@ao.pr.it 2: Department of Pathology, University of Parma, Via Gramsci 14, 43100, Parma, Italy, 3: Department of Emergency, University of Parma, Via Gramsci 14, 43100, Parma, Italy, 4: Department of Radiology, University of Parma, Via Gramsci 14, 43100, Parma, Italy,

Publication date: 2009-09-01