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

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

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

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

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

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

CT scan

MRI scan T2FLAIR

MRI scan T1T1 C+

Endocrine function tests Admission6 M follow-upReference range TSH0.08< –4.400 mcUI mL -1 Free T –1.8 ng dL -1 Cortisol1.4na6.2–19.4 mcg dL -1 ACTH*< 5na9–52 pg mL -1 Total testosterone –8.0 ng dL -1 FSH –12.4 mUI mL -1 LH< –8.6 mUI mL -1 GHna< –5.00 ng mL -1 Prolactin –15.2 ng mL -1

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

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

Clinically – Bilateral hemianopsia – Otherwise neurologically intact Follow-up

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

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+

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

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

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

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