Primary glomus tumour of the pituitary gland: diagnostic challenges of a rare and potentially aggressive neoplasm
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ORIGINAL ARTICLE
Primary glomus tumour of the pituitary gland: diagnostic challenges of a rare and potentially aggressive neoplasm Boon Leong Quah 1 & Carmine Antonio Donofrio 1,2 & Stefano La Rosa 3 & Jean-Philippe Brouland 3 Giulia Cossu 4 & Ibrahim Djoukhadar 5 & Helen Mayers 6 & Patrick Shenjere 7 & Marta Pereira 8 & Omar N. Pathmanaban 1 & Muhammed O. Murtaza 9 & Rao Gattamaneni 10 & Federico Roncaroli 2 & Konstantina Karabatsou 1
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Received: 5 June 2020 / Revised: 14 August 2020 / Accepted: 24 August 2020 # The Author(s) 2020
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-yearold 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 Ki67 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. Keywords Pituitary gland . Sella . Non-neuroendocrine tumour . Glomus tumour . Malignant
Introduction 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
* Federico Roncaroli [email protected]
5
Department of Radiology, Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK
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Department of Neurosurgery, Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK
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Department of Cellular Pathology, Salford Royal Foundation Trust, Salford, Manchester, UK
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2
Division of Neuroscience and Experimental Psychology, Faculty of Biology, Medicine and Health, Manchester Centre for Clinical Neuroscience and Manchester Centre for Clinical Neuroscience, Sal
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