Intraocular metastatic Wilms’ tumour in a child

Wilms’ tumour (nephroblastoma) is a congenitalrenal neoplasm. It has been described in association

with aniridia and has been linked in these cases to chromo-some 11p13.1 Orbital metastasis is rare.2 A single caseof intraocular metastasis to the choroid in an adultpatient has been previously reported.3

We present a pediatric case of metastatic intraocularWilms’ tumour with histopathologic demonstration ofthe primary tumour and the metastatic lesion.A 5-year-old female was first diagnosed as having left-side

Wilms’ tumour, for which nephrectomy was performed. Themass was 14 cm in diameter. Histopathology confirmed thediagnosis, with favourable mixed tubular and blastemal pat-terns, rare mitoses, and no anaplastic features or necrosis.Focal extension to perinephric fat and one positive lymphnode were noted. The patient’s metastatic work-up was nega-tive and the tumour was classified as stage 3. She receivedadjunctive chemotherapy and radiotherapy. She presented 16months later with left-eye leukocoria, mid-dilated pupil, irisneovascularization, and no view of the posterior cavity. Mag-netic resonance imaging detected a 12 mm uveal mass in theupper temporal quadrant of the left globe (Fig. 1). She waslost to follow-up and presented 6 months later with a painfulbuphthalmic blind eye. Histopathology of the enucleatedglobe revealed funnel-shaped total retinal detachment and

a retrolental undifferentiated round cell tumour arisingfrom the ciliary body area with an appearance similar toher original tumour (Fig. 2), surrounding fibrous stroma,and calcifications. The cells were positive toWilms’ tumourimmunohistochemical marker (WT1, cat. no. NCL-WT-1, dilution 1:20; Novocastra, Vision BioSystems, Newcas-tle Upon Tyne, U.K.), confirming a metastatic lesion.

Fig. 1—Magnetic resonance imaging showing left eye mass (indicated

by line) and total retinal detachment.

Correspondence

646 CAN J OPHTHALMOL—VOL. 45, NO. 6, 2010

Orbital metastatic Wilms’ tumour is generally rare.2

Albert et al.4 reviewed 60 cases of Wilms’ tumour withoutevidence of orbital involvement. The first case of orbital

involvement was reported in 1947, with the primarytumour diagnosed at the time of autopsy.5 Only 2 orbitalmetastatic Wilms’ tumours with histopathologic detailswere further reported.2,6 On the other hand, a single caseof intraocular metastatic Wilms’ tumour has beendescribed in an adult.3 The patient was 37 years old andpresented with isolated right eye choroidal metastasis2 years after the diagnosis of Wilms’ tumour. The patienthad concurrent biopsy-proven lung and liver metastases,which were considered risk factors for metastatic spread. Incontrast, our patient was a child and had a negative meta-static work-up before her ophthalmic presentation. There-fore, we consider our case exceptionally rare, and one thatoccurred despite initial proper surgical management andadjunctive therapy.

REFERENCES

1. Jolterand V, Boisjoly HM, Harnois C, et al. 11 p13 deletion,Wilms’ tumour, and aniridia: unusual genetic, non-ocular andocular features of three cases. Br J Ophthalmol 1990;74:568–70.

2. Apple DJ.Wilms’ tumor metastatic to the orbit. Arch Ophthal-mol 1968;80:480–3.

3. Finger PT, Warren FA, Gelman YP, McCormick SA. AdultWilms’ tumor metastatic to the choroid of the eye. Ophthal-mology 2002;109:2134–6.

4. Albert DM, Rubenstein RA, Scheie HG. Tumor metastasisto the eye: II. Clinical study in infants and children. Am JOphthalmol 1967;63:727–32.

5. Goulding HB. Orbital metastases from Wilms’ tumor. TransOphthalmol Soc UK 1947;67:491–2.

6. Fratkin JD, Purcell JJ, Krachmer JH, Taylor JC.Wilms’ tumormetastatic to orbit. JAMA 1977;238:1841–2.

Saleh Al-Mesfer, Hind AlKatanKing Khaled Eye Specialist Hospital,Riyadh, Kingdom of Saudia Arabia

Correspondence to Hind AlKatan, MD: hkatan@kkesh.med.sa

Can J Ophthalmol 2010;45:646–7

doi:10.3129/i10-033

Fig. 2—(A) Histopathologic appearance of the primaryWilms’ tumour

(hematoxylin and eosin; original magnification6200). (B) Similar histo-

pathologic appearance of the intraocular metastatic tumor showing

tubules and focal calcification (hematoxylin and eosin; original mag-

nification6200).

Correspondence

CAN J OPHTHALMOL—VOL. 45, NO. 6, 2010 647