1. SUPRASELLAR PITUICYTOMA PRESENTING WITH SPONTANEOUS HEMORRHAGE Pedro S. Pinto 1, André Couto Carvalho 2, Bruno Moreira 1, Gustavo Melo-Rocha 2, Alfonso Velasco 3, M. Melo-Pires 4, João Xavier 1 1 Department of Neuroradiology 2 Department of Endocrinology, Diabetes & Metabolism 3 Department of Neurosurgery 4 Division of Neuropathology Centro Hospitalar do Porto, Porto, Portugal

2. Introduction Pituitary gland –2 lobes – Anterior lobe or adenohypophysis Anatomy Embriology Functional Chromophobes, acidophils and basophils Relation with hypothalamus Copyright © 2001, BIOS Scientific Publishers Limited

3. Introduction Pituitary gland –2 lobes - Neural lobe or neurohypophysis Anatomy Embriology Functional Copyright © 2001, BIOS Scientific Publishers Limited

4. Introduction Pituitary gland –2 lobes - Neural lobe or neurohypophysis Anatomy Embriology Functional Astrocytes / Pituicytes Copyright © 2001, BIOS Scientific Publishers Limited

5. Pituicytomas –Low-grade astrocytoma of the pituicytes –<30 cases reported –Middle decades of life –Headaches and endocrine dysfunction –Surgical resection Introduction

6. 63 year-old man – ED Past history tiredness and decreased libido Abrupt decrease of consciousness level Neurological exam –Eyes open –Flexion of the limbs in response to painful stimuli –No verbal response –Bilateral hemianopsia –GCS: 6 Case Report

7. CT scan

9. MRI scan T2FLAIR

10. MRI scan T1T1 C+

11. Endocrine function tests Admission6 M follow-upReference range TSH0.08< 0.0050.400–4.400 mcUI mL -1 Free T40.71.10.9–1.8 ng dL -1 Cortisol1.4na6.2–19.4 mcg dL -1 ACTH*< 5na9–52 pg mL -1 Total testosterone0.0210.0622.8–8.0 ng dL -1 FSH0.41.31.5–12.4 mUI mL -1 LH< 0.10.41.7–8.6 mUI mL -1 GHna< 0.050.06–5.00 ng mL -1 Prolactin5.319.04.04–15.2 ng mL -1

12. Presumptive diagnosis – Hemorrhagic metastasis Surgery – Round reddish mass with thin capsule – Partial resection vigorous bleeding attachment to the pituitary stalk proximity with multiple blood vessels Surgery

13. Glial tumour composed of elongated or plump looking cells with a fibrillary background No mitosis / necrosis Immunostaining – GFAP + – S100 +++ – Vimentin +++ – EMA – Diagnosis – Pituicytoma Histology

14. Clinically – Bilateral hemianopsia – Otherwise neurologically intact Follow-up

15. World Health Organization Classification of Tumors of the Nervous System (2007) – “Grade 1 tumor that involves the posterior pituitary and/or its stalk, solid in architecture, composed of spindle cells, and presumably derived from pituicytes” Only second case of hemorrhagic pituicytoma Discussion

16. How to diagnose a pituicytoma? – Focal, well-circumscribed and strongly enhancing solid posterior sellar mass with suprasellar extension / suprasellar lesion – Orientation and bulk of the stalk – Absence of the neurohypophysis “bright spot” Discussion T1 T1 C+

17. Why diagnose a pituicytoma? – Highly vascularized and firm tumors Neurosurgery Neuroradiology Discussion

18. Pilocytic astrocytoma Spindle cell oncocytoma Pituitary adenoma Meningioma Lymphocytic hypophysitis Metastasis Discussion – differential diagnosis

19. Pituicytoma is a rare low-grade astrocytoma Differential diagnosis of a hemorrhagic suprasellar mass MRI has a central role Preoperative angiography with possible embolization Pathologic correlation Conclusion

20. Thanks!