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such as RCC and CD10, although these stains alone are not
speciﬁc. More importantly, renal cell carcinoma is excluded
using MAML2 FISH, radiographic imaging demonstrating a
lack of a renal mass, and/or the presence of mucin-containing
cells. Perivascular epithelioid cell tumor is positive for HMB45,
MART1, melanA, SMA, and desmin. Hyalinizing clear cell
carcinoma (HCCC) contains clear cells and mucin-producing
cells. HCCC is diﬀerentiated from PMEC using FISH, as
HCCC is positive for EWSR1-ATF1 fusion.
Using the current World Health Organization classiﬁcation,
PMEC is subclassiﬁed into low-grade and high-grade. Most
cases are low-grade PMEC and have an excellent prognosis,
while high-grade PMEC behaves similarly to other non-small
cell carcinomas of the lung (2, 3, 5). Treatment for PMEC is
surgical resection (2, 4, 19), with a low rate of recurrence for
patients with a low-grade tumor (20, 21).
PMEC is a rare primary malignancy in the lung with a
spectrum of morphologies that might include a predominance
of clear cells. In diagnostically challenging cases, FISH or realtime PCR might identify MAML2 rearrangement, which is a
MEC-speciﬁc genetic abnormality previously identiﬁed in a
subset of PMEC.

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