Gangliocytic paraganglioma of the appendix

Aims: A case of gangliocytic paraganglioma is reported in the appendix which, to the best of our knowledge, is the first case at this particular site to be described in modern literature. Methods and results: A 47-year-old man with signs and symptoms of acute appendicitis underwent appendectomy. In the resected specimen a tumour with a diameter of 9 mm was found, which microscopically consisted of three different cell types: (a) epithelioid cells lying in a trabecular pattern and in formations reminiscent of 'Zellballen' as seen in paragangliomas (b) spindle cells and (c) ganglion- like cells. A diagnosis of 'gangliocytic paraganglioma' was made and confirmed by immunohistochemical and ultrastructural examination. Conclusion: Gangliocytic paragangliomas are rare tumours of uncertain histogenesis. More than 40 years ago a tumour in the appendix with features similar to our case was described by Masson as 'neurocarcinoide'. Concerning its origin, Masson, as well as other authors describing gangliocytic paragangliomas decades later, referred to the endodermal-neuroectodermal complexes found by Van Campenhout. It is felt that the current finding of a gangliocytic paraganglioma in the appendix supports the hypothesis that gangliocytic paragangliomas arise from these embryonal structures.