LINC-27. PAEDIATRIC SUPRASELLAR TUMOURS: CLINICAL EXPERIENCE FROM A SINGLE TERTIARY CENTRE IN KUALA LUMPUR, MALAYSIA

INTRODUCTION The outcome of suprasellar tumours in children varies with the diagnosis and morbidity is significant. We herein report the clinical features of children with suprasellar tumours treated at our centre. METHODS Clinical data were collected by retrospective review from January 2000 to December 2019. The patients were identified from the paediatric haematology and oncology unit registry. RESULTS There was a total of 103 children with brain tumour and suprasellar tumours comprise of 14.6% (n=15). Median age at presentation was 7 years old. Male to female ratio was 3:2. Majority of cases was low grade glioma, 40% (n=6) and germ cell tumour(GCT) 33.3% (n=5) followed by craniopharyngioma, 13.3% (n=2) and Rathke cleft cyst, 6.7% (n=1). All patients had tissue diagnosis except one with secreting GCT and one with unsatisfactory tissue sample. Mean duration of follow up was 7.4 years. One patient with germinoma was lost to follow-up after radiotherapy. Three out of 13 (23%) patients died; 2 with GCT from disease progression; 1 craniopharyngioma after 11 years of unknown cause. All survivors have significant morbidity; 70% have moderate to severe visual impairment, 90% have at least two pituitary hormones deficiency, 20% have neurological deficit and 1 was surgically related. Two boys have precocious puberty not related to disease progression. Two with GCT with diabetes insipidus had history of thromboembolism (stroke and pulmonary embolism). CONCLUSIONS Suprasellar tumours in children at our centre pose a significant long-term complications and multidisciplinary team management and follow up is required to improve the morbidity.

Primary glomus tumour of the pituitary gland: diagnostic challenges of a rare and potentially aggressive neoplasm

Primary non-neuroendocrine tumours of the pituitary gland and sella are rare lesions often challenging to diagnose. We describe two cases of clinically aggressive primary glomus tumour of the pituitary gland. The lesions occurred in a 63-year-old male and a 30-year-old female who presented with headache, blurred vision and hypopituitarism. Neuroimaging demonstrated large sellar and suprasellar tumours invading the surrounding structures. Histologically, the lesions were characterised by angiocentric sheets and nests of atypical cells that expressed vimentin, smooth muscle actin and CD34. Perivascular deposition of collagen IV was also a feature. Case 2 expressed synaptophysin. INI-1 (SMARCB1) expression was preserved. Both lesions were mitotically active and demonstrated a Ki-67 labelling index of 30%. Next-generation sequencing performed in case 1 showed no mutations in the reading frame of 37 commonly mutated oncogenes, including BRAF and KRAS. Four pituitary glomus tumours have previously been reported, none of which showed features of malignant glomus tumour. Similar to our two patients, three previous examples displayed aggressive behaviour.

Surgical management of sellar and suprasellar tumours

Despite their shared location, sellar and suprasellar tumours are an extremely diverse group of lesions with multiple sources of origin. These tumours may be congenital lesions, such as lipomas, Rathke’s cleft (pars intermedia) cysts, arachnoid cysts, and hamartomas, or they may develop from any of the surrounding structures, resulting in pituitary adenomas, meningiomas, craniopharyngiomas, chordomas, optic nerve gliomas, among others. Non-neoplastic lesions, such as cavernous malformations, fibrous dysplasia, and inflammatory lesions such as sarcoidosis also present here, and tumours from remote sites are found in the form of metastasis in both the pituitary gland and the cavernous sinus. This extraordinary variety can make management of cases with these tumours more complicated because of the myriad of pathologies encountered. To add to the complexity of treatment for sellar and suprasellar tumours, there are numerous critical structures in this region, including the pituitary gland, critical vessels, and multiple cranial nerves. Avoiding injury to these important structures is a key component of treatment planning. In this chapter, we will discuss the anatomy of the sellar and suprasellar region with these factors in mind. We will discuss the workup, management, operative strategy, postoperative care, and complication management of these tumours then elaborate on controversies related to these issues.

Loss of horizontal macular ganglion cell complex asymmetry: an optical coherence tomography indicator of chiasmal compression

ObjectiveTo estimate the macular ganglion cell complex (GCC) asymmetry in patients with suprasellar tumours, to compare its diagnostic performance to the nasal GCC thickness and visual field (VF) and to investigate how the parameters correlate with magnetic resonance imaging (MRI) findings.Methods and analysisCross-sectional study of patients with suprasellar tumours affecting the optic chiasm. Macular optical coherence tomography (OCT) scans were evaluated for nasal GCC sector thinning and loss of normal GCC asymmetry between corresponding nasal-temporal sectors. Equivalently, VFs were analysed for defects compatible with chiasm dysfunction. The relationship between optic chiasm and tumour was measured on MRI.ResultsThirty-three eyes of 33 patients were included. There were OCT findings in 14 eyes. Nasal GCC thinning was found in 9 eyes and loss of GCC asymmetry in 12 eyes; the two parameters were not significantly different with respect to number of positive findings (p=0.45). Loss of GCC asymmetry, however, occurred in 5 eyes among 24 without GCC thinning (proportion 0.21; 95% confidence interval 0.071 to 0.42). In 8 eyes, VF indicated pathology; of these, 7 had concurring OCT findings. The prevalence of OCT and VF findings increased significantly with suprasellar tumour extension on MRI.ConclusionThe diagnostic capabilities of nasal GCC thinning and loss of GCC asymmetry were comparable, whilst their complementary performances increased the proportion of eyes in which OCT suggested compression. The prevalence of both OCT and VF findings grew with suprasellar tumour extension. In several cases, however, structural findings on OCT preceded detectable VF deficits.