Federico Roncaroli1, Debajyoti Chatterjee2, Caterina Giannini3, Marta Pereira4, Stefano La Rosa5, Jean-Philippe Brouland5, Kanna Gnanalingham6, Carlo Galli7, Bethania Fernandes7, Andrea Lania8, Bishan Radotra2. 1. Division of Neuroscience and Experimental Psychology, Faculty of Biology, Medicine and Health, School of Biology, University of Manchester, M13 9PT, UK, Manchester. 2. Deparment of Histopathology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. 3. Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA. 4. Manchester Centre for Genomic Medicine, St Mary's Hospital, Division of Evolution and Genomic Science, University of Manchester, Manchester, UK. 5. Institute of Pathology, University Hospital and University of Lausanne, Lausanne, Switzerland. 6. Department of Neurosurgery, Manchester Centre for Clinical Neurosciences, Salford Royal Foundation Trust, Stott Lane, Salford, Manchester, M6 8HD, UK. 7. Department of Histopathology, Humanitas University, Milan, Italy. 8. Department of Endocrinology, Humanitas University, Milan, Italy.
Abstract
AIM: To describe fournovel primary epithelial tumours of the sella with papillary architecture and TTF-1 expression. METHODS: Paraffin embedded tissue from the four cases and recurrence of patient 1was investigated with haematoxylin-eosin, special histochemical stains, immunohistochemistry with a broad panel of antibodies,and next generation sequencing. The ultrastructure of one tumour was studied in tissue retrieved from paraffin. RESULTS: The lesions occurred in three females aged 20, 26 and 42 years and a male aged 49years. They presented with signs and symptoms secondary to pituitary stalk compression. Pre-operative neuroimaging documented mixed solid and cystic, enhancing sellar masses with suprasellar extension. Histologically, the tumours showed thin papillae lined by a single layer of cytokeratin and TTF-1 positive cuboidal and cylindrical cells with mildly atypical nucleus. Next generation sequencing performed in three cases did not identify any mutations. The main differential diagnosis included metastasis from lung or thyroid carcinoma,extra-ventricular choroid plexus papilloma,and sellar ependymoma. CONCLUSION: We suggest the descriptive term of primary papillary epithelial tumour of the sella (PPETS) for this entity and propose that it could represent the intracranial equivalent of thyroid-like low-grade nasopharyngeal papillary adenocarcinoma. The cell of origin of PPETS remains undetermined although the intense and ubiquitous expression of TTF-1 may suggest a derivation fromthe infundibulum or ventricular recess. Our study expands the spectrum of sellar TTF-1-positive tumour and challenges the view that they all derive from pituicytes. This article is protected by copyright. All rights reserved.
AIM: To describe fournovel primary epithelial tumours of the sella with papillary architecture and TTF-1 expression. METHODS:Paraffin embedded tissue from the four cases and recurrence of patient 1was investigated with haematoxylin-eosin, special histochemical stains, immunohistochemistry with a broad panel of antibodies,and next generation sequencing. The ultrastructure of one tumour was studied in tissue retrieved from paraffin. RESULTS: The lesions occurred in three females aged 20, 26 and 42 years and a male aged 49years. They presented with signs and symptoms secondary to pituitary stalk compression. Pre-operative neuroimaging documented mixed solid and cystic, enhancing sellar masses with suprasellar extension. Histologically, the tumours showed thin papillae lined by a single layer of cytokeratin and TTF-1 positive cuboidal and cylindrical cells with mildly atypical nucleus. Next generation sequencing performed in three cases did not identify any mutations. The main differential diagnosis included metastasis from lung or thyroid carcinoma,extra-ventricular choroid plexus papilloma,and sellar ependymoma. CONCLUSION: We suggest the descriptive term of primary papillary epithelial tumour of the sella (PPETS) for this entity and propose that it could represent the intracranial equivalent of thyroid-like low-grade nasopharyngeal papillary adenocarcinoma. The cell of origin of PPETS remains undetermined although the intense and ubiquitous expression of TTF-1 may suggest a derivation fromthe infundibulum or ventricular recess. Our study expands the spectrum of sellar TTF-1-positive tumour and challenges the view that they all derive from pituicytes. This article is protected by copyright. All rights reserved.