Benign pineal cysts in children with bilateral retinoblastoma: A new variant of trilateral retinoblastoma?

Popović, Maja; Balmer, Aubin; Maeder, Philippe; Bragança, Teresa; Munier, Francis L.

doi:10.1002/pbc.20464

Cited by 38 publications

(16 citation statements)

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“…Congenital brain malformations occur mainly in patients with 13q- deletion syndrome [10]. Benign pineal cysts should not be misinterpreted as pineal PNET, even in children with retinoblastoma [10, 66]. Thin-section T2-weighted and contrast-enhanced T1-weighted slices are helpful for differential diagnoses.…”

Section: Image Analysis Checklist For Mr Reporting (Table 2)mentioning

confidence: 99%

Guidelines for imaging retinoblastoma: imaging principles and MRI standardization

Göricke²,

et al. 2011

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Retinoblastoma is the most common intraocular tumor in children. The diagnosis is usually established by the ophthalmologist on the basis of fundoscopy and US. Together with US, high-resolution MRI has emerged as an important imaging modality for pretreatment assessment, i.e. for diagnostic confirmation, detection of local tumor extent, detection of associated developmental malformation of the brain and detection of associated intracranial primitive neuroectodermal tumor (trilateral retinoblastoma). Minimum requirements for pretreatment diagnostic evaluation of retinoblastoma or mimicking lesions are presented, based on consensus among members of the European Retinoblastoma Imaging Collaboration (ERIC). The most appropriate techniques for imaging in a child with leukocoria are reviewed. CT is no longer recommended. Implementation of a standardized MRI protocol for retinoblastoma in clinical practice may benefit children worldwide, especially those with hereditary retinoblastoma, since a decreased use of CT reduces the exposure to ionizing radiation.

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Section: Image Analysis Checklist For Mr Reporting (Table 2)mentioning

confidence: 99%

Guidelines for imaging retinoblastoma: imaging principles and MRI standardization

Göricke²,

et al. 2011

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“…Because of life-threatening side effects that may be related with curative aggressive treatment in TRb patients [25], it is important that cysts are not misinterpreted as tumor. Pineal cysts are diagnosed if (1) an enlarged PG is present, (2) with a hypointense central region with respect to white matter on T1-weighted-images and isointense with respect to CSF on T2-weighted images, and (3) a thin wall of 2 mm or less with discrete rim enhancement after gadolinium injection [20]. Although these criteria are formulated, pineal lesions in retinoblastoma are causing radiological dilemmas, especially if the cyst wall is irregularly thickened (>2 mm) or shows a fine nodular aspect of the wall [19, 20].…”

Section: Discussionmentioning

confidence: 99%

Trilateral retinoblastoma: neuroimaging characteristics and value of routine brain screening on admission

et al. 2012

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Trilateral retinoblastoma (TRb) is a rare disease associating intraocular retinoblastoma with intracranial primitive neuroectodermal tumor. Treatment is difficult and prognosis is poor. This multicenter study evaluates clinical findings and MR imaging characteristics of associated intracranial tumors in Rb patients. Clinical data of 17 patients (16 TRb and 1 quadrilateral Rb patients) included time intervals between Rb and TRb diagnosis and presence of baseline brain-imaging (BBI). Two reviewers reviewed all images individually and one reviewer per center evaluated their images. Consensus was reached during a joint scoring session. Studies were reviewed for tumor location, size and imaging characteristics (signal intensity (SI) on T1- and T2-weighted images, enhancement pattern and cystic appearance). Of 18 intracranial tumors, 78 % were located in the pineal gland and 22 % suprasellar. All tumors showed well-defined borders with mostly heterogenous enhancement (72 %) and isointense SI on T1- (78 %) and T2-weighted images (72 %) compared to gray matter. The majority of pineal TRbs showed a cystic component (57 %). TRb detected synchronously with the intraocular tumors on BBI (n = 7) were significantly smaller (P = 0.02), and mainly asymptomatic than TRb detected later on (n = 10). Overall, 5-year-survival of TRb patients detected on BBI was 67 % (95 % CI 29–100 %) compared to 11 % (95 % CI 0–32 %) for the group with delayed diagnosis. TRb mainly develops in the pineal gland and frequently presents with a cystic appearance that could be misinterpreted as benign pineal cysts. Routine BBI in all newly diagnosed Rb patients can detect TRb at a subclinical stage.

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“…However, approximately 5% of patients with bilateral disease develop pineal cysts; which appear to be a forme fustre of trilateral retinoblastoma (Fig. 9.3) (RodriguezGalindo et al 2003;Beck-Popovic et al 2006).…”

Section: Trilateral Retinoblastomamentioning

confidence: 98%

Retinoblastoma

2010

Pediatric Oncology

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Benign pineal cysts in children with bilateral retinoblastoma: A new variant of trilateral retinoblastoma?

Abstract: This report describes benign cystic lesions of the pineal gland in patients with hereditary Rb, suggesting a benign variant of TRb. Underlying possible pathogenetic mechanisms are discussed.

Cited by 38 publications

References 37 publications

Guidelines for imaging retinoblastoma: imaging principles and MRI standardization

Guidelines for imaging retinoblastoma: imaging principles and MRI standardization

Trilateral retinoblastoma: neuroimaging characteristics and value of routine brain screening on admission

Retinoblastoma

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