Controversies in management of sellar teratoid/rhabdoid (AT/RT) tumors: report of two cases mimicking pituitary adenomas and review of literature

BACKGROUND: Sellar teratoid/rhabdoid (AT/RT) tumors represent rare lesions that occur mainly in children younger than 3 years of age. They account for 1-2% of pediatric brain tumors and over 10% of central nervous system (CNS) tumors in infants, with a male preponderance up to 3 years of age. Adult cases are exceptional, with only about 40 cases reported. Despite aggressive treatments, AT/RTs present poor prognosis, with a median survival of less than 1 year. Currently, few reports described a survival of more than 2 years with the combination of radiation therapy and high-dose alkylator-based chemotherapy or by a multi-agent approach. AIM: We describe the clinical, neuroradiological and histological features of two cases of adult AT/RT tumors discussing similarities and differences, and speculating about the possibility to perform the most appropriate therapeutic strategy. MATERIALS AND METHODS: We report two similar cases of sellar lesions underwent full endocrinologic and neuroradiologic preoperative workup. In both cases the clinical onset was characterized by oculomotor nerves palsy. Preoperative MR study revealed heterogeneus sellar/suprasellar lesion invading parasellar regions (left cavernous sinuses in one case) with an intense and disomogeneous contrast enhancement. In one of two cases, five years before a diagnosis of micro-prolactinoma was done and she was treated with cabergoline, reaching the complete shrinkage of the lesion and the prolactin (PRL) levels normalization. An endoscopic endonasal procedure was performed in both cases. In one case postoperative fractionated radiation therapy was started after histological examination, six weeks after surgery, whereas in the other case a high-dose chemotherapy with antraciclines was administered. RESULTS: A total resection of lesion was achieved in both cases. The lesion appeared grey coloured, tough and bloody. The histological examination demonstrated an atypical teratoid/rhabdoid (AT/RT) tumor. In both patients, a slight neurological improvement was observed few days after surgery. No abnormalities of pituitary function were registered before and after surgery. A sudden clinical worsenig occurred shortly after surgery; a MR study revealed the regrowth of the sellar masses. Radiation therapy in one case, and antraciclines in the other one were started. After a transient clinical improvement and a slight volume decrease, the tumor showed a rapid progression with cavernous sinus invasion, and in one case with the invasion and the occlusion of left foramen rotondum. Both patients died 3 months after surgery. CONCLUSION: We reported clinical, neuroradiological and histological features of two cases of AT/RT tumors, discussing the different postoperative therapeutic strategies, and speculating about the possible optimal therapeutic protocol.