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.