Oral Abstract Abstract

(Paediatric), #220531327122

A Case of Recurrent Craniopharyngioma Post-Operative with Residual Disease and GH Deficiency

Ahmad Fahmi bin Abdullah Asuhaimi, Hospital UiTM Puncak Alam; Mazidah binti Noordin, Hospital UiTM Puncak Alam; Asc. Prof Noor Shafina binti Md Noor, Hospital UiTM Puncak Alam

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Introduction

Craniopharyngioma is an uncommon intracranial tumor in childhood. Even though it is a benign tumour, recurrence of disease may occur which is commonly complicated with endocrinopathy. We present a case of recurrent craniopharyngioma post-resection with residual tumor complicated with multiple pituitary hormone deficiencies, including growth hormone (GH) deficiency.

Methods

A 7-year-8-month-old boy presented with persistent headache and symptoms of increased intracranial pressure. MRI Brain showed suprasellar cystic mass. He underwent total resection of the tumor. HPE confirmed craniopharyngioma. He developed central diabetes insipidus, central hypothyroidism and ACTH deficiency post-operatively. 11 months later, presented with blurring of vision and increased sleepiness. MRI Brain confirmed recurrence of the tumor. Near-total-excision of the tumour was done as the tumor was adhered to the optic nerve and chiasma. After the surgery, he was under close surveillance for recurrence of disease. Annual MRI Brain surveillance showed stable residual disease.

Results

The patient is currently 12-year-old. Apart from the endocrinopathies mentioned, he is now showing signs of growth hormone deficiency such as hypothalamic obesity with weight BMI at +3.35SDS. He has poor height velocity at 3 cm/year. He has metabolic syndrome including dyslipidaemia, and fatty liver. He also has delayed bone age and poor IGF-1 level. Family counseling was done to explain the role of GH therapy for him, including the risks and benefits.

Conclusion

This case highlights the challenge of initation of GH therapy, in a patient with a background history of recurrent craniopharyngioma and residual disease. Proper counseling with the patient and family is crucial to explain the clinical indications, risks and benefits of the GH therapy. A multidisciplinary approach of the management involving the paediatric endocrinologists, oncologists, neurosurgeons, radiologists, rehab physicians and dietitians together with close surveillance of primary disease are extremely important.

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