Abstract
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.
Highlights
Primary non-neuroendocrine tumours of the pituitary gland and sellar region are rare and are often challenging to diagnose preoperatively and at pathological examination
If craniopharyngiomas and meningiomas are excluded, the remaining 1% of sellar non-neuroendocrine neoplasms includes the group of TTF-1 expressing tumours of the posterior pituitary, neuronal and paraneuronal, germ cell, haemopoietic, melanocytic, sellar salivary gland-like tumours, the spectrum of mesenchymal neoplasms including chordoma, and bone and cartilage tumours, sellar atypical
The pituitary gland is an uncommon site for glomus tumour (GT) [7,8,9,10] where it probably derives from the thick tunica media of hypophyseal portal vessels that are similar to glomera of other sites and function as sphincter to regulate blood flow [7, 11, 12]
Summary
Primary non-neuroendocrine tumours of the pituitary gland and sellar region are rare and are often challenging to diagnose preoperatively and at pathological examination. We describe two patients with primary pituitary GT presenting as large, invasive sellar and suprasellar lesions, one with a fluctuating clinical behaviour due to tumour apoplexy and the second with a short history of visual loss and amenorrhoea. Magnetic resonance imaging (MRI) at that time documented an expanded sella and a bulky pituitary gland but no tumour was observed He presented again in August 2018 with worsening intermittent headaches and blurred vision. A 30-year-old female presented with a 10-month history of progressive bilateral visual deterioration and amenorrhoea She initially refused an endocrinological assessment and a brain MRI. A brain MRI showed a solid cyst, heterogeneously enhancing, intra- and suprasellar lesion with pituitary fossa enlargement, optic chiasm compression, third ventricle extension and bilateral cavernous sinus invasion. Right-sided hemiparesis and left-sided crural paresis and died 6 months after the second operation
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