Kidneys, Adrenals, and Retroperitoneum
Fig. 27.46 Benign adrenal nodule (A) showing staining for melan A (A103) (B), calretinin (C), and negative for inhibin (D) (x HP),
neoplasm can occur at any tim e fro m early c h ild h o o d to old
age. ACCs are classified as eith er fu n c tio n a l o r n on fu n c tio n a l.
H o rm o n a l excess produces classic clinical signs such as hypercor-
tiso le m ia (w h ic h occurs in 50% o f cases), v iriliz a tio n (20% ), o r a
c o m b in a tio n o f these (1 0 -1 5 % ). Between 10 and 15% o f tum ors
are n o n fu n c tio n a l. Levels o f precursor steroids m ay be elevated
in c lin ic a lly "n o n fu n c tio n a l" carcinomas. A n A C C m ay present
e ith er as a fu n c tio n a l tu m o r th a t is n o t suppressed by high-dose
dexam ethasone therapy o r as an intra -a b d o m in a l mass. M ost
carcinom as present as large, advanced masses w ith an average
size at presentation o f 16 cm (range 6 -4 0 cm ), and the m a jo rity
o f patients have extra-adrenal spread at the tim e o f presentation.
Sites o f metastases include the lungs, liver, lym p h nodes, bone,
pancreas, and diaphragm . T he tu m o r has a p o or prognosis: over-
all m edian survival is reported as 14 m o n th s.137,138
Size is one o f the m ost im p o rta n t criteria fo r accessing m alig -
nancy b y radiography in p rim a ry adrenal masses. M ost carci-
nom as exceed 6 cm in diam eter. S m alle r carcinom as cannot be
re lia b ly determ ined b y im aging techniques.
Grossly, carcinom as are fre q u e n tly necrotic, hem orrhagic,
and variegated in color. C arcinom as show architectural disarray,
inc lu d in g sheet-like o r trabecular g row th and m arked increase
in vascularity. M o st tu m o rs show m oderate to m arked p leom or-
phism , m icroscopic foci o f necrosis, hem orrhage, and calcifica-
tio n .139 Increased m ito tic activity is seen. Capsular and vascular
invasion are fre q ue ntly noted. S m all size, despite m alig n an t
histo p atho lo g ic
m ost
o f favorable
outcom e.140-141
The cytologic appearance m ay range fro m w e ll differentiated to
h ig h ly anaplastic; however, c om m o n to all tum ors is the presence
o f hypercellularity, reten tion o f cytoplasm , and loss o f cohesion
o f cells. Cells m ay be fo u n d singly o r aggregated in to densely
grouped sheets. W ell-d ifferentiated tum ors have lip id -la d en cells,
b u t the nuclei are enlarged and hyperchrom atic and occasional
m itoses m ay be observed (Figs 27.50 and 27.51). C ell b lock m ay
show a w ell-d efined trabecular pattern o r "flo a tin g isla n d " s im i-
la r to th a t observed in w ell-d ifferentiated hepatocellular carci-
nom a; however, b ile pig m ent is absent (see Fig. 27.52). O ther
patterns include sheets o f cells w ith p ro m in e n t arb orizing vessels
(Fig. 27.53). Tum ors th a t are m oderately differentiated m ay show
lesser am ounts o f lip id , w ith granular cytoplasm and flocculent
spheric cytoplasm ic aggregates resem bling rhab d oid bodies A n
oncocytic variant o f AC C has been described (Fig. 27.55). P o orly
differentiated carcinomas e xh ib it extrem e cellular p leom orp hism
w ith m ultinucleated bizarre cells varying fro m spindled to p o ly-
hedral. N u cle oli are p ro m in e n t and large. G iant cells m ay be seen.
A sarcom atoid variant o f AC C has been described b y FN A .141
O ncocytic AC C is a rare b u t d istinct va ria n t o f A C C .142 These
tu m o rs are u su a lly n o n fu n c tio n in g and are com posed o f polyg-
onal cells w ith abundant granular and e osin o p hilic cytoplasm .
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