Gangliocytic paraganglioma of the appendix

Aims: A case of gangliocytic paraganglioma is reported in the appendix whic h, 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 app endicitis underwent appendectomy. In the resected specimen a tumour with a diameter of 9 mm was found, which microscopically consisted of three differ ent cell types: (a) epithelioid cells lying in a trabecular pattern and in formations reminiscent of 'Zellballen' as seen in paragangliomas (b) spindl e cells and (c) ganglion-like cells. A diagnosis of 'gangliocytic paragangl ioma' was made and confirmed by immunohistochemical and ultrastructural exa mination. Conclusions: Gangliocytic paragangliomas are rare tumours of uncertain hist ogenesis. More than 40 years ago a tumour in the appendix with features sim ilar to our case was described by Masson as 'neuro-carcinoide'. Concerning its origin, Masson, as well as other authors describing gangliocytic paraga ngliomas decades later, referred to the endodermal-neuroecto dermal complex es found by Van Campenhout. It is felt that the current finding of a gangli ocytic paraganglioma in the appendix supports the hypothesis that gangliocy tic paragangliomas arise from these embryonal structures.