Case history : A 44-year-old female presented with 6-week history of gradual deterioration in her left vision associated with eye pain. She saw an ophthalmologist who noted that she had reduced visual acuity (6/24) and colour vision (Ishihara colour vision score 3/15) in her left eye with left relative afferent pupillary defect (RAPD). It was thought that she may have a left retrobulbar neuritis so she was advised to attend A&E for an urgent neurology assessment. On examination, she was noted to have diplopia on left lateral gaze, with left sided RAPD and a left nasal hemianopia.
Investigations: MRI of her orbits and brain demonstrated a 15 x 18 x 20 mm lobulated suprasellar mass compressing the optic chiasm (OC) and left optic radiation. Blood tests on admission showed a normal anterior pituitary profile other than a very mildly elevated prolactin of 581 mIU/L (102-496).
Results & treatment : Her case was discussed in the regional pituitary multidisciplinary team (MDT) meeting. Her imaging findings were in keeping with cystic craniopharyngioma. As she had visual impairment with OC and left optic radiation compression, she underwent urgent Extended Endoscopic Transsphenoidal removal of suprasellar Craniopharyngioma (EETS). Histopathology showed adamantinomatous craniopharyngioma (ACP) with no unusual features. She initially suffered from post operative complication with CSF leak that was promptly repaired. After a short period of polyuria which was either due to vasopressin deficiency or overhydration, she developed SIADH. This was managed with strict fluid restriction and resolved promptly. Post- operative imaging demonstrated an excellent resection. As there was a good surgical resection, the plan was to undertake surveillance imaging in the first instance before proceeding to radiotherapy.
Conclusions & points for discussion: Craniopharyngiomas are tumours of low histological malignancy which originate from remnants of the craniopharyngeal duct epithelium. The commonest histological subtype is ACP, which is diagnosed with a bimodal peak of incidence (5-15 years & 45-60 years). Clinical presentation is usually with symptoms of increased intracranial pressure, visual impairment and endocrine abnormalities. Treatment comprises of neurosurgery and radiotherapy. Although long-term survival is high, quality of life is frequently impaired due to their close proximity to the optic chiasm, hypothalamus and pituitary gland. Hypothalamic involvement and treatment-related hypothalamic damage frequently result in hypothalamic obesity, fatigue and psychosocial problems. Pituitary surgery may be associated with vasopressin deficiency in the first few days, followed by SIADH. Electrolytes should be monitored closely to direct active fluid restriction.