Diagnostic usefulness of sustentacular cells in paragangliomas: immunocytochemical and ultrastructural investigation.

Neuroendocrine tumors may derive either from neuroectoderm or endoderm. Both may present with a similar histologic pattern known as "Zellballen" regardless of their histogenetic origin, making it difficult to separate the two different histogenetic entities in certain cases. To evaluate the usefulness of sustentacular cells in the recognition of tumors of paraganglionic origin, the authors analyzed immunohistochemical and ultrastructural characteristics of 25 paragangliomas and 19 pulmonary and 10 small intestinal carcinoids. Sustentacular cells with characteristic dendritic features, strong immunoreactivity for S-100 protein, and agranular cytoplasm by electron microscopy were found consistently in the paragangliomas and not found in typical carcinoid tumors, except for four examples of spindle cell carcinoid of the lung. The presence of sustentacular cells in tumors with "Zellballen" pattern therefore denotes paraganglionic origin.