MON-424 Suprasellar Immature Cystic Teratoma: A Case Report

Background: Intracranial teratomas are rare and comprise about 0.5% of all intracranial tumors. Actually, a total of 15 cases of sellar-suprasellar teratoma have been described in the last 26 years. Teratomas occur more frequently in children and young adults than in the older population and in males as compared to females. Symptoms at diagnosis are usually neurological defects, diabetes insipidus, and hypopituitarism. Teratoma diagnosis can be suggested through neuroimaging findings. Magnetic Resonance Imaging (MRI) remains the preferred modality. Neuro-radiological findings of mixed-density mass, usually with mixed cystic and solid components inclusions of teeth, fat, and calcification can be suggestive. Tumor markers as beta-HCG and alpha-FP can be useful. Optimal treatment for mature teratoma is neurosurgical excision and in cases of immature teratomas or mixed germ cells tumors teratomas, a multi-step treatment is required with neurosurgical excision, adjuvant or neo-adjuvant radiotherapy and chemotherapy. Clinical Case: A 15-year-old woman, with a headache, secondary amenorrhea, syncope, progressive visual loss, polydipsia, and polyuria due to diabetes insipidus. A craniopharyngioma or another parasellar mass was suspected, an MRI showed an expansive suprasellar process with a cystic component with chiasm compression. A hormone panel showed corticotrophic and thyrotrophic deficiencies. Hormone replacement was started and the patient was referred for surgery. The immediate postoperative progressed well, although one month later she presented recurrence of the compressive symptoms (a headache and visual loss). She was submitted to second surgery. According to the pathology, the mass was identified as a suprasellar immature teratoma, with germ component, as well as cystic areas (ki67: 80%). In addition, tumor markers tests were requested: beta-HCG 5.47mIU/mL (<5.0), alpha-FP 3.1ng/mL (<7.0), DHL 635U/L (240-480). The patient was staged and referred to oncology unit that indicated radiotherapy, as a postoperative adjuvant therapy. At the most recent follow-up, approximately 9 months later, MRI shows a residual, stable tumor and patient presents with bitemporal hemianopsia. Conclusion: Intracranial germ cell tumors are uncommon, with only 15 cases of sellar-suprasellar teratoma described in the literature. It is important to recognize this entity in the differential diagnosis once they mimic many other lesions. Future reports on this rare location for a teratoma may help ascertain long-term outcomes. Reference: Chiloiro, S., Giampietro, A., Bianchi, A., & De Marinis, L. (2015). Clinical management of teratoma, a rare hypothalamic-pituitary neoplasia. Endocrine, 53(3), 636-642. doi:10.1007/s12020-015-0814-4