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Imaging Spectrum of Sellar and Parasellar Masses in a Paediatric

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Imaging Spectrum of Sellar and Parasellar Masses in a Paediatric Hong Kong J Radiol. 2021;24:129-40 | https://doi.org/10.12809/hkjr2117210 PICTORIAL ESSAY Imaging Spectrum of Sellar and Parasellar Masses in a Paediatric Population: a Pictorial Essay S Gupta, S Singh, R Dixit, A Prakash Department of Radiology, Maulana Azad Medical College, India INTRODUCTION Paediatric sellar/suprasellar mass Tumours in the sellar and suprasellar regions are not uncommon in the paediatric population and differ to adult sellar tumours with respect to type and frequency. Identify the location of the mass Such masses comprise about 10% of all paediatric - Sellar brain tumours.1 Magnetic resonance imaging (MRI) - Sellar-suprasellar - Suprasellar renders high intrinsic soft tissue contrast and greater - Lesions of the pituitary stalk anatomic detail. It remains the mainstay in diagnosis and - Miscellaneous characterisation of these lesions. Computed tomography may provide complimentary information, especially in identifying the presence of calcifications. The first step Characterise the mass based on imaging morphology in diagnosing these lesions is to determine their anatomic - Solid location, followed by an assessment of their intrinsic - Solid-cystic - Cystic signal and enhancement pattern. Cystic component / calcification also needs to be looked at as it aids characterisation of the lesions (Figure 1). The clinical Look for key imaging features symptoms and hormonal profile along with the age of - Intrinsic magnetic resonance signal patterns the child are also important to narrow down the list of - Contrast enhancement patterns differential diagnoses. Clinical symptoms can be non- - ± Calcifications specific due to raised intracranial pressure or specifically - ± Cysts - Other findings on brain imaging related to hormonal derangement. Altered vision may occur secondary to mass effect on the optic apparatus.1,2 NORMAL ANATOMY Differential diagnoses The sellar, suprasellar, and parasellar regions are an Figure 1. Approach to paediatric sellar/suprasellar/parasellar anatomically complex area (Figure 2). The pituitary masses. Correspondence: Dr S Gupta, Department of Radiology, Maulana Azad Medical College, India Email: [email protected] Submitted: 8 Apr 2020; Accepted: 24 Aug 2020 Contributors: All authors designed the study, acquired the data, analysed the data, drafted the manuscript, and critically revised the manuscript for important intellectual content. All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity. Conflicts of Interest: The authors declare no conflicts of interest. Funding/Support: This pictorial essay received no specific grant from any funding agency in the public, commercial or not-for-profit sectors. Ethics Approval: Ethical approval was requested and obtained from the institutional ethics committee. Patient consent was obtained in each case. © 2021 Hong Kong College of Radiologists. CC BY-NC-ND 4.0 129 Sellar and Parasellar Masses (a) (b) Figure 2. Normal anatomy. (a) Sagittal T1-weighted image, anterior pituitary isointense on T1-weighted (yellow arrow), posterior pituitary bright on T1- weighted (thin yellow arrow), pituitary stalk (red arrow), optic tract (open yellow arrow), suprasellar cistern (yellow arrowhead), and mamillary body (thin red arrow). (b) Coronal T2- weighted image: pituitary gland (yellow arrow), optic chiasma (red arrow), flow void of right internal cerebral artery (yellow arrowhead), and third ventricle (thin red arrow). fossa is a spherical depression in the sphenoid bone, Table 1. Adamantinomatous versus papillary craniopharyngioma. bounded by the sphenoid sinus antero-inferiorly, the Adamantinomatous Papillary cavernous sinuses laterally, diaphragm sellae and the Age Bimodal, children (50-60 Adults hypothalamus superiorly, and dorsum sellae and the years) brainstem posteriorly. The suprasellar cistern is an Tissue structure Mostly cystic Predominantly solid Fluid Oil Fluid expansion of the subarachnoid space and contains T1-weighted Hyperintense Hypointense the optic nerves, chiasma, tracts, Circle of Willis, and Shape Lobulated Spherical pituitary stalk / infundibulum. The hypothalamus forms Adhesion and Yes, to hypothalamus No infiltration the floor and lateral walls of the third ventricle. The tuber Enhancement Avid of the solid Mild cinereum is a ridge of tissue between the infundibulum component and mammillary bodies. The pituitary gland is composed Calcification Common (90%) Rare of two parts: the adenophysis, isointense to brain on both T1-weighted (T1W) and T2-weighted (T2W) images and the neurohypophysis, seen as a bright spot on T1W images. The anterior lobe appears hyperintense like the the suprasellar and/or sellar region. Solid components posterior lobe in newborns and becomes T1 isointense are isointense on T1W and hyperintense on T2W by 6 to 8 weeks of life.3 images and show enhancement. The cyst contents can be T1 hyperintense in about 33% cases due to high Craniopharyngioma proteinaceous / cholesterol / haemorrhagic content, Craniopharyngiomas are the most common paediatric referred to as ‘Motor-oil’ appearance7 (Figure 4a). intracranial tumours of non-glial origin, accounting for up to 10% of paediatric brain tumours4 and 50% of Germ Cell Tumours paediatric suprasellar tumours.5 Histologically, there Germ cell tumours are classified as germinomatous germ are two subtypes: the adamantinomatous type that has a cell tumours or non-germinomatous GCTs. They usually bimodal distribution and is mostly seen in children, and arise in the pineal region, followed by the suprasellar the squamous papillary type that is seen mostly in adults cistern. Smaller lesions tend to be homogenous and are (Table 1). Craniopharyngiomas are most commonly both centred in the pituitary stalk. Thickening of the pituitary intrasellar and suprasellar (53-75%), followed by purely stalk with absence of the normal T1 hyperintense suprasellar (20-41%) and, rarely, purely intrasellar.6 neurohypophysis bright spot may be seen. Larger lesions Computed tomography scan is useful for detecting tend to be solid-cystic in appearance (Figure 5), with the calcifications that may present in up to 87% of the cases solid component usually being isointense to hypointense (Figure 3). on T2W imaging. The lesions show heterogenous post-contrast enhancement (Figure 6a). The risk of Adamantinomatous craniopharyngiomas on MRI leptomeningeal dissemination emphasises the importance (Figure 4) appear as lobulated solid-cystic masses in of imaging the entire craniospinal axis8 (Figure 6b). 130 Hong Kong J Radiol. 2021;24:129-40 S Gupta, S Singh, R Dixit, et al Figure 3. Craniopharyngioma. Axial computed tomography images showing hyperdense mass (yellow arrows) in sellar-suprasellar region with calcification (red open arrow) within. (a) (b) Figure 4. Adamantinomatous craniopharyngioma. (a) Sagittal (c) (d) T1-weighted, (b) coronal T2-weighted, and contrast- enhanced (c) axial and (d) sagittal magnetic resonance images showing a solid cystic mass (arrows) in the sellar- suprasellar region, appearing hypointense on T1-weighted images with hyperintense focus (arrowhead) suggestive of motor oil appearance, hyperintense on T2-weighted images showing homogenous contrast enhancement of solid component (open arrows) and peripheral enhancement of the cystic component (thin arrow). Synchronous involvement of the pineal and suprasellar germinomas show only the absence of a posterior regions may be evident in up to 15% of cases and are pituitary bright spot on MRI with an infundibular mass termed bifocal germinomas9 (Figure 7). Cases of occult seen on follow-up scans after 14 months10 (Figure 8). Hong Kong J Radiol. 2021;24:129-40 131 Sellar and Parasellar Masses (a) (b) (c) (d) Figure 5. Germ cell tumours. (a) Sagittal T1-weighted and (b to d) axial T2-weighted magnetic resonance imaging images showing a solid lesion (arrow) in the sella-suprasellar region appearing hypointense on T1-weighted images and isointense on T2-weighted images. Encasement of bilateral proximal middle cerebral arteries (open arrows), bilateral internal carotid arteries (thin arrows), and posterior encasement of the basilar artery (arrowhead) are evident. (a) (b) Figure 6. In the same case, T1-weighted post-contrast (a) axial and (b) sagittal spine magnetic resonance images show intense enhancement of the mass (yellow arrow) with intense leptomeningeal enhancement along the spinal cord (red arrows), indicating metastasis. 132 Hong Kong J Radiol. 2021;24:129-40 S Gupta, S Singh, R Dixit, et al (a) (b) Figure 7. Bifocal germinoma. (a) Sagittal and (b) axial post- contrast brain magnetic resonance imaging images showing a solid enhancing lesion (arrow) in the sellar-suprasellar region with a similar enhancing lesion in the pineal region (thin arrow). (a) (b) (c) Figure 8. Occult germinoma. (a, b) Sagittal T1-weighted and (c) coronal post-contrast T1-weighted brain magnetic resonance images showing absence of the normal pituitary bright spot (arrows) with no lesion in the pituitary gland. Hypothalamic Chiasmatic Glioma associated with NF1.11 Distinguishing hypothalamic Hypothalamic chiasmatic glioma (HCGs) are mostly gliomas from optic chiasm gliomas can be challenging low-grade World Health Organization grade I and is typically determined by locating the epicentre of astrocytomas and invade the brain along the optic the lesion and determining optic nerve involvement. pathways. Around 20% to 50% of the HCGs are associated with
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