lens invasion in retinoblastoma
TRANSCRIPT
Pediatr Blood Cancer 2012;58:477–478
LETTER TO THE EDITORLens Invasion in Retinoblastoma
To the Editor: Lens invasion in retinoblastoma has not been
reported in human eye. We report a case of retinoblastoma with
primary lens invasion by tumor cells.
A 2-year-old female was brought by her father with complaint
of right eye leukocoria for 3 months. The lens in the right eye was
displaced with presence of white retrolental reflex. Fundus exam-
ination showed a white mass occupying entire vitreous cavity,
encroaching on displaced lens. Left eye examination was normal.
B-scan confirmed the mass with calcification. MRI scan showed
no evidence of extraocular extension. Examination under general
anaesthesia (EUA) was performed which confirmed above
findings. The intraocular pressures (tonopen non-contact tonome-
ter) were 22 and 17 mmHg in right and left eyes, respectively.
A clinical diagnosis of right eye retinoblastoma was made. The
child underwent right eye enucleation. Gross examination of
enucleated eye showed a whitish mass arising from retina filling
the vitreous cavity. Microscopically, a large basophilic mass was
identified, composed of small tumor cells with hyperchromatic
nuclei. Majority of cells were undifferentiated with areas of cal-
cification and necrosis. There were no tumor cells in anterior
chamber, trabecular meshwork, or iris stroma. However, the
lens was displaced with the mass infiltrating the posterior and
temporal equatorial surface of lens (Fig. 1A). Tumor cells were
also identified in the lens matrix (Fig. 1B). There was full thick-
ness invasion of choroid and anterior sclera. Optic nerve showed
prelaminar invasion but laminar portion, postlaminar portion, and
surgical end were free from tumor cells. Based on the above
findings, the child was given six cycles of adjuvant chemotherapy.
Invasion of lens capsule by tumor is rarely reported in humans
[1,2]. The lack of histologic findings consistent with traumatic
lens rupture supports the interpretation that there was a primary
lens invasion in present case.
The crystalline lens has long been regarded as tumor-immune
tissue [3]. Even highly invasive tumors occupying the entire
vitreous demonstrate well-defined borders at the lens capsule
interface. Several hypotheses have been proposed for this. These
include anerobic metabolism of lens, impermeability of lens cap-
sule, and genetic factors [3]. It has been speculated that chemicals
like anti-angiogenic factors, growth inhibitors, and anti-lens cap-
sule digestion factors repel the tumor cells from capsule. Besides,
the lens capsule is the thickest basement membrane of body and is
thought to be impermeable to cells [4].
This report can be of value in understanding that lens invasion,
though rare, can be possible in retinoblastoma. However, the
clinical and prognostic significance of such finding is doubtful
since lens is an avascular tissue.
Aditi Gupta, MS, DNB
Gopal Lingam, MS, FRCS Ed
Shri Bhagwan Mahavir Vitreoretinal Services
Sankara Nethralaya
Chennai, Tamil Nadu, India
S. Krishnakumar, MD
Department of Ocular Pathology
Chennai, Tamil Nadu, India
*Correspondence to: Vikas Khetan, DO, DNB, Shri Bhagwan
Mahavir Vitreoretinal Services, Sankara Nethralaya, 18 College
Road, Chennai 600 006, Tamil Nadu, India.
E-mail: [email protected]
Received 7 July 2011; Accepted 19 July 2011
Fig. 1. A: Photomicrograph showing full section of the right globe
(pupil optic nerve axis). Tumor arising from the retina spreading
anteriorly and infiltrating and surrounding the lens (H&E stain,
�2). B: Photomicrograph under higher magnification showing the
infiltration of the lens matrix by tumor cells (H&E stain, �40). [Color
figure can be seen in the online version of this article, available at
http://wileyonlinelibrary.com/journal/pbc]
� 2011 Wiley Periodicals, Inc.DOI 10.1002/pbc.23310Published online 26 August 2011 in Wiley Online Library(wileyonlinelibrary.com).
Vikas Khetan, DO, DNB*
Shri Bhagwan Mahavir Vitreoretinal Services
Sankara Nethralaya
Chennai, Tamil Nadu, India
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478 Gupta et al.
Pediatr Blood Cancer DOI 10.1002/pbc