ESPE2024 Poster Category 2 Pituitary, Neuroendocrinology and Puberty (36 abstracts)
1Ankara, Etlik City Hospital, Department of Pediatric Endocrinology, Ankara, Turkey. 2Ankara, Etlik City Hospital, Department of Neurosurgery, Ankara, Turkey. 3University of Health Sciences, Ankara, Turkey. 4Ankara, Etlik City Hospital Department of Pediatric Hematology and Oncology, Ankara, Turkey
Introduction: Intracranial teratomas constitute 0.5% of all intracranial tumors. Teratoma localized in the sellar region is very rare in children. Here, we will present a case in which growth hormone deficiency was detected upon admission with complaints of short stature, craniopharyngioma was considered on cranial imaging, and sellar teratoma was diagnosed during the operation.
Case: A four-year-ten-month-old male patient presented with a complaint of short stature that had been noticed for the last year. He was born with a weight of 2650 grams at the 38th gestational week via vaginal delivery. On physical examination, height: 95 cm (-3.25 sds), body weight: 14 kg (-2.16 sds), prepubertal and systemic examinations were normal. In laboratory tests for short stature: hemogram and biochemistry were normal, thyroid function tests were euthyroid, celiac serology was negative, bone age was 3 years. The peak growth hormone (GH) response to the L-Dopa stimulation test was inadequate with 5.74 ng/ml, and the peak GH response to the clonidine stimulation test was inadequate with 3.39 ng/ml. IGF1:43 ng/ml (<-2 sds), IGFBP3:2.32 ug/ml (mean/+1 sds). In cranial imaging, a bilobed lesion of approximately 24 × 12 × 15 mm in size, containing densely calcified and cystic areas located central-right paracentrally in the suprasellar cistern, was detected and it was reported that the lesion was compatible with craniopharyngioma. Preoperatively, other anterior pituitary hormones were normal. Following the consultation with the neurosurgery and oncology department, the tumor was almost completely resected. During the operation, it was seen that the calcified areas within the tumor were mature teeth. Pathological examination was compatible with mature cystic teratoma. During the postoperative follow-up of the patient, central hypothyroidism, central adrenal insufficiency, and central diabetes insipidus developed and replacement treatments were started.
Conclusion: Development of mature teratoma in the sellar region has been reported in very few cases in the literature. As in our case, initial diagnosis may be difficult due to similar radiological features to craniopharyngioma, which is more common in the childhood age group. In treatment, the appropriate surgical approach should be chosen according to the spread of the tumor. Although the prognosis for these tumors is generally good, multidisciplinary long-term follow-up of these cases is recommended.
Key Words: Craniopharyngioma, mature cystic teratoma, sellar mass