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Int J Clin Exp Pathol 2013;6(9):1948-1952

Case Report
Gangliocytic paraganglioma of the appendix with features suggestive of malignancy, a
rare case report and review of the literature

Maisoun Qassim Abdelbaqi, Maryam Tahmasbi, Masoumeh Ghayouri

Department of Anatomic Pathology, H. Lee Moffitt Cancer Center and Research Institution, Tampa, Florida; Department of Pathology and Cell
Biology, University of South Florida, Tampa, Florida, USA

Received May 31, 2013; Accepted June 15, 2013; Epub August 15, 2013; Published September 1, 2013

Abstract: We report a case of appendicial paraganglioma in a 40 year old female who presented with acute appendicitis and underwent
laparoscopic appendectomy. To the best of our knowledge this is the first reported case of appendicial gangliocytic paraganglioma with
features suggestive of malignancy in the modern literature. Van Eeden S. et al. reported the first case of appendicial paraganglioma in a 47
year old man who also presented with acute appendicitis. The appendectomy specimen showed a distended appendix with thickened wall,
and a 1.3 cm mucosal based yellow lesion. Microscopically this lesion was centered in the submucosa and consisted of three different cell
types: (a) epithelioid cells with pale eosinophilic finely granular cytoplasm containing bland oval nucleus with stippled chromatin, that form
solid nests lying in a trabecular pattern and in formations reminiscent of ‘Zellballen’ as seen in paragangliomas (b) second type cells have
large vesicular nuclei with prominent nucleoli and abundant cytoplasm that are scattered singly, (c) third type cells with bland elongated nuclei
form broad fascicle and envelop the epithelioid and ganglion cells. Immunohistochemical analysis showed the epithelioid cell nests
immunoreactive for synaptophysin and the ganglion-like cells and spindle Schwann cells to be immunoreactive for S100 protein, whereas all
three cells populations were negative for CAM5.2 and Pancytokeratin. We do believe that an accurate diagnosis of Gangliocytic paraganglioma
(GP) of the appendix was rendered, detailed microscopic examination of doubled hematoxylin and eosinophil stained sections as well as the
immunohistochemical phenotype of the three components have been undertaken to confirm the diagnosis of GP. (IJCEP1305039).

Keywords: Appendix, gangliocytic paraganglioma, carcinoid, immunohistochemistry

Address correspondence to: Dr. Masoumeh Ghayouri, Anatomic Pathology Division, Moffitt Cancer Center Moffitt Cancer Center and Research
Institution, Tampa, Florida. Tel: 813-745-3284; Fax: 813-745-1708; E-mail: Masoumeh.Ghayouri@moffitt.org