Spindle Cell Carcinoids of the Lung with Paraganglioid Features: a Reappraisal of their Histogenetic Origin from Paraganglia Using Immunohistochemical and Electronmicroscopic Techniques

Five cases of spindle cell carcinoids of the lung were analyzed by immunohistochemical and ultrastructural technique. They were found to be biphasic tumors composed of the major component of neuroendocrine cells (chief cells) and a minor component of dendritic cells (supporting cells). The chief cells displayed positivity for neuroendocrine phenotypic antigenic markers: neuron specific enolase (NSE), chromogranin A, and synaptophysin. They contained varying numbers of dense-core granules by electron microscopy. In addition, the chief cells expressed cytoplasmic positivity for cytokeratins. The supporting cells were dendritic in appearance and displayed strong positivity for S-100 protein in all cases. Glial fibrillary acidic protein was positive in two cases. On electron microscopy, the supporting cells were agranular and found along the external lamina surrounding the nests of tumor cells. In two cases, rare ganglion cell-like cells were present. The histomorphologic, immunohistochemical, and ultrastructural features were contrastingly different from the classical pulmonary carcinoid and rather resembled gangliocytic paragangliomas arising from small intestine and spine. It is proposed that pulmonary carcinoids with biphasic features are better designated as gangliocytic paragangliomas of the lung rather than paraganglioid carcinoids.