Description:
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Atul Kumar, Subijay Sinha
Dr. Rajendra Prasad Centre for Ophthalmic Sciences
All India Institute of Medical Sciences, New Delhi, India
akum66mm@yahoo.co.in
Can J Ophthalmol 2007;42:3356
doi: 10.3129/can j ophthalmol.i07-026
Ciliary body leiomyoma with atypical features
C
iliary body leiomyoma (CBL) is a rare benign
smooth muscle tumour; it appears as an amelanotic
mass.
1
Its clinical and light microscopic diagnosis is dif-
ficult.
1,2
Positive immunoreactivity for alpha smooth
muscle actin (
SMA) or electron microscopic (EM)
study is required to make a correct diagnosis.
2
We report
a case of CBL in a 17-year-old female.
A healthy 17-year-old female patient was referred with
an iris and ciliary body mass (Fig. 1). Iridocyclectomy with
an en block tumour resection was performed.
Histopathologic examination disclosed a ciliary body cir-
cumscribed mass that was composed of nonpigmented
spindle-shaped cells with blunt-ended nuclei (Fig. 2A) and
foci of nuclear pleomorphism with few mitoses. Iris tissue
was tumour-free. The presence of focal atypical features in
the tumour misled us to a preliminary impression, before
immunohistochemistry studies, of malignant melanoma.
By means of immunohistochemistry, the tumour cells
were shown to be strongly immunoreactive for
SMA
(Fig. 2B) but not for S100, HMB45, pancytokeratin,
AE1/3 and MNF116, neuron-specific enolase (NSE),
and glial fibrillary acidic protein (GFAP). A histopatho-
logic diagnosis of leiomyoma with focal atypical features
was made. Two-year follow-up of this patient was unre-
markable, with 20/20 vision in the operated eye.
Light microscopic diagnosis of leiomyoma is difficult
because its features may be quite similar to neurofibro-
mas, amelanotic spindle cell melanomas, and neurilemo-
mas.
2
Use of immunohistochemistry for S100,
SMA,
desmin, and human melanoma antigen (HMB45) helps
to distinguish tumours of Schwann cell origin from mes-
enchymal tumours, like leiomyomas.
3
Positive
immunoreactivity for
SMA or EM study is required to
make a diagnosis of a smooth muscle tumour.
2
In our
case, lack of immunoreactivity of the tumour cells for
S100 and HMB45 excluded the diagnoses of spindle cell
melanoma and Schwann cell tumours. Absence of
Correspondence
336
CAN J OPHTHALMOLVOL. 42, NO. 2, 2007
Fig. 1The vascularized ciliary body mass pre-
sented at the peripheral iris of the right eye.
Fig. 2A: Tightly packed bundles of oval- to
spindle-shaped cells with little collagenous
stroma and blunt-ended nuclei on hema-
toxylineosin staining (original magnification