PMID- 10674273 OWN - NLM STAT- MEDLINE DCOM- 20000301 LR - 20191103 IS - 0344-0338 (Print) IS - 0344-0338 (Linking) VI - 196 IP - 1 DP - 2000 TI - Sustentacular cells in sporadic paraganglioma-like medullary thyroid carcinoma: report of a case with diagnostic and histogenetic considerations. PG - 55-9 AB - Sustentacular cells (SCs) are glial supporting cells of the fetal, adult normal, and neoplastic extra and adrenal human chromaffin cell lineage. SCs have also been identified in some cases of medullary thyroid carcinoma (MTC), raising both diagnostic and histogenetic problems. We report a rare case of a sporadic paraganglioma-like variant of MTC showing numerous S-100 protein and glial fibrillar acid protein (GFAP) positive SCs encircling neoplastic cells in a nesting (Zellballen) pattern similar to that observed in paragangliomas or pheochromocytomas. Although stromal amyloid deposits were only focally detected, diagnosis was immunohistochemically confirmed by immunoreactivity of the neoplastic cells for cytokeratin, CEA, calcitonin, chromogranin A, neuron-specific-enolase, and synapthopysin. As for the histogenesis of SCs, if we assume that MTC is a neural crest-derived tumor, it is likely that these cells reflect the ability of the common precursor cell to differentiate towards a sustentacular-type glial cell lineage in addition to the typical neuroendocrine one. This viewpoint is supported by the evidence that rare cases of MTC may contain neoplastic or supporting cells showing a multidirectional differentiation (usually neuroendocrine and melanocytic) that recapitulates the different cell lineages arising from the developing neural crests. FAU - Magro, G AU - Magro G AD - Institute of Anatomical Pathology, University of Catania, Italy. FAU - Grasso, S AU - Grasso S LA - eng PT - Case Reports PT - Journal Article PL - Germany TA - Pathol Res Pract JT - Pathology, research and practice JID - 7806109 RN - 0 (Glial Fibrillary Acidic Protein) RN - 0 (S100 Proteins) SB - IM MH - Carcinoma, Medullary/chemistry/*pathology MH - Diagnosis, Differential MH - Female MH - Glial Fibrillary Acidic Protein/analysis MH - Humans MH - Immunoenzyme Techniques MH - Middle Aged MH - Neuroglia/chemistry/*pathology MH - Paraganglioma/chemistry/*pathology MH - S100 Proteins/analysis MH - Thyroid Neoplasms/chemistry/*pathology EDAT- 2000/02/16 09:00 MHDA- 2000/03/04 09:00 CRDT- 2000/02/16 09:00 PHST- 2000/02/16 09:00 [pubmed] PHST- 2000/03/04 09:00 [medline] PHST- 2000/02/16 09:00 [entrez] AID - 10.1016/s0344-0338(00)80022-9 [doi] PST - ppublish SO - Pathol Res Pract. 2000;196(1):55-9. doi: 10.1016/s0344-0338(00)80022-9.