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Routine Neuroimaging in Retinoblastoma for the Detection of Intracranial Tumors
Arch Ophthalmol. 2001;119:450-452.
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The occurrence of a midline intracranial primitive neuroectodermal tumor
(PNET) with bilateral retinoblastoma has been called "trilateral retinoblastoma."1 This rare complication of heritable retinoblastoma
is associated with an extremely poor prognosis, accounting for up to 50% of
tumor-associated mortality among patients with retinoblastoma.2
Primitive neuroectodermal tumors usually occur 2 to 4 years after diagnosis
of bilateral retinoblastoma. However, PNETs have been diagnosed in patients
with unilateral retinoblastoma 6 months before diagnosis of bilateral retinoblastoma
and in 2 siblings of patients with bilateral retinoblastoma with no intraocular
disease.3 In most cases, patients with PNET
are seen for symptoms of increased intracranial pressure after the tumor is
large, and the disease is usually fatal.3
Patients diagnosed with small, asymptomatic PNETs, however, have improved
survival rates when treated with chemotherapy and radiation.4
This has led to routine neuroimaging among patients with heritable retinoblastoma
to detect preclinical intracranial neoplasms in many centers. However, no
. . . [Full Text of this Article] Report of Cases
Case 1 Case 2 Case 3
Comment
Corresponding author: Joan M. O'Brien, MD, Box 0730, University of
California, San Francisco, San Francisco, CA 94143-0730 (e-mail: aleja@itsa.ucsf.edu).
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ABSTRACT
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