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===General=== | ===General=== | ||
*Considered to be a [[paraganglioma]].<ref name=Ref_EP327>{{Ref EP|327}}</ref> | *Considered to be a [[paraganglioma]].<ref name=Ref_EP327>{{Ref EP|327}}</ref> | ||
*Literally means "dusky" (pheo) "colour" (chromo) - dull appearance on gross. | |||
*Tumour arises from medulla | |||
Clinical: | |||
*Classic finding: hypertension. | |||
*Paroxysms (i.e. episodes) of tachycardia, headache, anxiety, hypertension. | |||
Laboratory findings (urine): | |||
*Vanillylmandelic acid (VMA). | |||
*Metanephrines. | |||
Epidemiology: | |||
*May be associated with [[multiple endocrine neoplasia]] 2a and 2b. | |||
=== | ===Microscopic=== | ||
* | Features:<ref>{{Ref PBoD8|1161}}</ref> | ||
*Usu. polygonal cells, may be spindled. | |||
*Arranged in cell nests - "Zellballen" (literally ''cell balls'') - '''key feature'''. | |||
*Stippled chromatin ([[AKA]] salt and pepper chromatin) - coarsely granular chromatin. | |||
*Granular cytoplasm, often basophilic - '''important'''. | |||
*Often haemorrhagic - highly vascular. | |||
*+/-Nuclear pleomorphism. | |||
=== | Notes: | ||
* | *The nested architecture (Zellballen) is useful for differentiating from [[ACC]]. | ||
* | |||
===IHC=== | |||
*Chromogranin +ve. | |||
*Synaptophysin +ve. | |||
*S100 +ve (sustentacular cells). | |||
=== | ===[[EM]]=== | ||
*Membrane-bound secretory granules. | |||
==Ganglioneuroma== | ==Ganglioneuroma== |
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