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

• DOI: 10.1007/s00428-020-02923-4
• Publication Date: 2020-09-12T03:47:28Z
• Authors: Boon Leong Quah; Carmine Antonio D...; Stefano La Rosa; Jean-Philippe Bro...; Giulia Cossu; Ibrahim Djoukhadar; Helen Mayers; Patrick Shenjere; Marta Pereira; Omar N. Pathmanaban; Muhammed O. Murtaza; Rao Gattamaneni; Federico Roncaroli; Konstantina Karab...
• Keywords: Synaptophysin; Glomus tumor; SMARCB1

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