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 Table of Contents  
Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 842-843

Retinoblastoma with spinal cord metastasis: When an eye tumor bites the cord!

1 Pediatric Hematology-Oncology Unit, Department of Pediatrics, Advanced Pediatrics Center, Chandigarh, India
2 Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
3 Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission15-Mar-2021
Date of Acceptance13-Jun-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Deepak Bansal
Pediatric Hematology-Oncology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh - 160 012
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_603_21

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Keywords: Carcinomatosis, CNS, drop, leptomeninges, quadriplegia

How to cite this article:
Roy PS, Singh U, Tadepalli S, Ahuja CK, Bansal D. Retinoblastoma with spinal cord metastasis: When an eye tumor bites the cord!. Indian J Ophthalmol Case Rep 2021;1:842-3

How to cite this URL:
Roy PS, Singh U, Tadepalli S, Ahuja CK, Bansal D. Retinoblastoma with spinal cord metastasis: When an eye tumor bites the cord!. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 28];1:842-3. Available from: https://www.ijoreports.in/text.asp?2021/1/4/842/327700

Central nervous system (CNS) involvement by extraocular retinoblastoma is not infrequent.[1],[2] The tumor may invade the CNS along the optic nerve fibers or cerebrospinal fluid (CSF) space around it. CNS disease may present as a suprasellar mass, leptomeningeal carcinomatosis, or be asymptomatic and detected on CSF examination. Metastatic retinoblastoma resulting in spinal cord compression is, however, unusual. We report a case of extraocular retinoblastoma with multifocal spinal cord compression secondary to drop metastases.

A 2-year-old boy with exotropia of the right eye from the age of 3-months was diagnosed with retinoblastoma. Magnetic resonance imaging (MRI) of the orbit and brain revealed an extraocular extension of the tumor along the optic nerve, without intracranial extension. CSF malignant cytology was negative. A bone scan or spinal imaging was not performed as the patient lacked suggestive symptoms. Following 4-cycles of systemic chemotherapy (vincristine, carboplatin, and etoposide), the diseased eye was enucleated along with a 1.3 cm-long optic nerve stump. On histopathology, the optic nerve transection margin was infiltrated.

Subsequently, external-beam radiotherapy was administered to the orbit. While receiving radiotherapy, the patient had status epilepticus and post-ictal encephalopathy. There were accompanying hypotonia, hyporeflexia, a paucity of movements of all four limbs, urinary retention, and constipation. Contrast-enhanced computed tomography (CECT) head revealed diffuse meningeal enhancement indicative of leptomeningeal carcinomatosis [Figure 1]a. MRI-spine, performed with a clinical suspicion of cord compression, revealed intradural, extramedullary drop metastases causing cord compression, extending from the cervico-medullary junction to the S1 vertebra level [Figure 1]b and [Figure 1]c. Palliative care was advised.
Figure 1: CECT scan of the head (a) showing diffuse leptomeningeal enhancement (solid arrows). Sagittal (b) and axial (c) contrast-enhanced T1-weighted MR images of the cervical spine demonstrating the spinal extension of the leptomeningeal lesions in the form of diffuse sheet-like enhancing leptomeningeal deposits (arrowheads) with nodularity at places (dotted arrows) indicating drop metastases. Circumferential leptomeningeal enhancement is noted on the axial image, causing cord compression

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  Discussion Top

Less than 20% of patients with retinoblastoma and CNS metastasis have spinal meningeal involvement. Radiotherapy, high-dose steroids, systemic or intrathecal chemotherapy, or laminectomy are the treatment options.[3],[4] Unfortunately, our patient developed spinal cord metastases while on therapy, conferring a poor prognosis. CNS metastasis of retinoblastoma universally translates to a poor outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Pérez V, Sampor C, Rey G, Parareda-Salles A, Kopp K, Dabezies AP, et al. Treatment of nonmetastatic unilateral retinoblastoma in children. JAMA Ophthalmol 2018;136:747-52.  Back to cited text no. 1
Honavar SG, Manjandavida FP, Reddy VAP. Orbital retinoblastoma: An update. Indian J Ophthalmol Case Rep2017;65:435-42.  Back to cited text no. 2
Kamaleshwaran KK, Shibu DK, Mohanan V, Shinto AS. Rare case of trilateral retinoblastoma with spinal canal drop metastasis detected with fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography imaging. Indian J Nucl Med 2014;29:115-6.  Back to cited text no. 3
[PUBMED]  [Full text]  
Chang C-Y, Hung G-Y, Hsu W-M, Kao SC, Hwang B, Hsieh YL, et al. Retinoblastoma with spinal recurrence presenting as spinal cord compression. J Formos Med Assoc 2006;105:497-502.  Back to cited text no. 4


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