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Multifocal presentation of gangliocytic paraganglioma in the
mediastinum and esophagus.
Hum Pathol. 2004
Oct;35(10):1288-91.
Gangliocytic
paraganglioma (GP) is a rare, typically benign tumor that shows
neuroectodermal (neurosustentacular or Schwannian and neuronal) and
neuroendocrine differentiation. Once thought to arise exclusively in
the periampullary region as a solitary lesion, recent reports have
documented both origin of GP in a variety of extra-duodenal sites as
well as synchronous multifocal presentation of the tumor. Herein, we
describe the first reported case of simultaneous occurrence of GP in
the superior mediastinum and esophagus. A mass in the mid-distal
esophagus and a separate mass in the superior mediastinum at the
thoracic inlet were found in a 58-year-old woman by computed
tomography scan. Subsequent biopsy of the superior mediastinal mass
showed nests of epithelioid tumor cells coexisting with
ganglioneuromatous elements, whereas biopsy of the esophageal mass
showed nests of epithelioid cells with interspersed ganglion cells.
The epithelioid tumor cells showed diffuse immunohistochemical
expression of keratin (CAM 5.2), chromogranin, and synaptophysin
supporting true neuroendocrine differentiation; ganglion cells
expressed S-100 protein and neurofilament protein; and the spindled
elements expressed S-100 protein, neurofilament protein, and glial
fibrillary acidic protein indicating Schwannian differentiation. The
finding of another GP occurring outside the periampullary region
bolsters the argument for a stem cell origin of this unusual tumor. |
