mm
PART TWO
Diagnostic Cytology
B
Fig. 27.59 (A) Pheochromocytoma
with ganglioneuromatous component
(Papanicouloau x HP). (B) Corresponding histo-
logic section. (C) Pheochromocytoma with
positive staining for neuron-specific enolase (x MP).
Key features of pheochromocytoma
• T u m o rs o f c h ro m a ffin cells o f re n a l m e d u lla ;
• U s u a lly s y m p to m a tic due to h yp e rsec re tion o f catechol-
a m in e s, re s u ltin g in p a ro xysm a l h y p e rte n s io n , sw eating ,
a nd p a lp ita tio n s ;
• M a y be sp orad ic in occurrence o r p a rt o f m u ltip le e n d o -
crine n e o p la sia synd ro m e ;
• S m a ll o r m od e rate -sized p o ly g o n a l cells w ith u n ifo rm
ro u n d /o v a l o r elong ated n u c le i w ith a b u n d a n t fin e ly
g ra n u la r cytop lasm ;
• Large m y o id -lik e cells w ith eccentric large n u c le i and
a b u n d a n t g ra n u la r c ytop lasm ;
• T u m o r cells arranged in sheets, lo o se clusters, a nd nests;
• M a y e x h ib it areas o f s ig n ific a n t a typ ia w ith o u t increased
m ito tic a c tivity; and
• P o sitive fo r c h ro m o g ra n in and syn a p to p h ysin , a nd v a ri-
ab ly p o s itiv e fo r C K .
Neuroblastoma, Ganglioneuroblastoma,
and Ganglioneuroma
N eurob lastom a, g ang lioneuroblastom a, and g ang lioneurom a
are tu m o rs
derived fro m th e n o n c h ro m a ffin
cells
o f the
sym pathetic nervous system o f the m ed u lla. Because th ey are
capable o f the synthesis and m etab olism o f catecholam ines, ele-
vations in u rin a ry and plasm a levels o f catecholam ines and th e ir
m etabolites are useful tu m o r markers. These tu m o rs constitute
a spectrum representing progressive stages in the m a tu ra tio n
o f neuroblasts to m ature g anglion cells. T he y m ay occur in the
adrenal, pelvis, and retro p e rito ne um . N eurob lastom a is a h ig h ly
m a lig n a n t tu m o r o f child h oo d ; however, less th an 10% o f cases
occur in patients o ld e r th a n 10 years. In adults and adolescents,
neurob lastom a has a lon g er course th an in children; however,
the u ltim a te outcom e is poor, regardless o f stage. It is com posed
o f cells th a t resem ble p rim itiv e neuroblasts. A p art fro m tu m o rs
o f the central nervous system, it is th e m ost c om m o n solid
tu m o r in infa nc y and c h ild h o o d and occurs m ost fre q ue ntly
856
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