ESPE Abstracts

Introduction: Rathke's cleft cyst (RCC) is a non-neoplastic epithelial lesion of the sellar or suprasellar region. Although most RCC cases are asymptomatic, they can present with headache, visual defects, and endocrine dysfunction. We present a case diagnosed with a giant RCC postoperatively, characterized by short stature and multiple pituitary hormone deficiencies.

Case: A 15-year-old male, who came with complaints of headache and was found to have an intracranial mass, was referred to us primarily due to his short stature, with craniofaryngioma being the main consideration. In the physical examination, his height was measured as 143.5 cm (-4.48 SDS), his weight as 39.5 kg (-3.46 SDS), his target height was calculated as 173 cm (-0.52 SDS), his bone age was assessed as 10 years, testicles were 3/3 ml in volume, penile length was 6 cm, and pubic hair development was at stage 1. Test results showed fT4:0.33 ng/L, TSH:4.55 mIU/L, cortisol:1.1 μg/dL, ACTH:9.57 ng/L, FSH:1.58 IU/L, LH:1.17 IU/L, total testosterone:<2.5 ng/dL, IGF-1:32 ng/mL (<-2 SDS), prolactin:<2 μg/L. Hydrocortisone at a dose of 10 mg/m²/day was started for the patient with a peak cortisol response of 8.62 µg/dL in the low-dose ACTH stimulation test. Levothyroxine treatment at a dose of 2µg/kg/day was added for the management of central hypothyroidism. Cranial MRI revealed a thick-walled cystic T2 hyperintense lesion measuring 52x33x58 mm with calcifications, extending into the bilateral sphenoid sinus and causing destruction of the sphenoid sinus wall in the sellar region. The lesion was observed to completely fill the sphenoid sinus compartment, displacing the vascular structures within it, extending superiorly to the foramen of Monro, compressing the optic chiasm, and extending superiorly towards the third ventricle in the interhemispheric fissure. The lesion was primarily considered to be consistent with a craniopharyngioma. Due to the presence of polyuria in the postoperative follow-up of the patient, tests were conducted which showed Na:147 mmol/L, K:4.56 mmol/L and urine density:1005. These findings suggested central diabetes insipidus and desmopressin treatment was initiated. The pathology result of the material sent during the operation was reported as a Rathke's cleft cyst. Growth hormone treatment was initiated for the patient due to short stature caused by growth hormone deficiency.

Conclusion: RCC is rarely symptomatic, but when symptomatic, it often presents with headaches and a hypopituitarism. A case of panhypopituitarism due to a giant RCC is presented.