gms | German Medical Science

Objective: Atypical teratoid rhabdoid tumors (AT/RT) are very rare tumors of early childhood usually arising in the posterior fossa between the age of 2 to 5 years. Here we describe two cases of adult sellar AT/RT treated in our department. In additional, the literature searched to analyze clinical, radiological and histopathological features.

Methods: We evaluated the clinical presentation and course, the radiological imaging, and the histopathological features of our two patients. Furthermore a search in the literature was conducted to identify a total of additional four cases with a sellar manifestation of an AT/RT.

Results: All six patients with sellar manifestation of AT/RT were females in their 3rd to 6th decade of life. Clinical presentation was typical for a large sellar tumor with beginning visual impairment and slight headache. The first radiological diagnosis was usually pituitary adenoma, MRI scan showing a heterogeneously enhancing intrasellar mass with suprasellar extension, invasion into the cavernous sinus was evident, the optic chiasm appeared elevated. Histological examination revealed rhabdoid cells with a primitive neuroectodermal component. Immunohistochemical examination showed typical inactivation of INI1 gene. After surgery and histological examination, all six patients were treated with radiation therapy. All patients died within 2 to 28 month after diagnosis usually after having developed either local recurrence or cerebral or spinal metastasis.

Conclusions: Sellar manifestation of AT/RT in adults occurs extremely rarely. If histological examination is inconclusive in an expected pituitary adenoma, clinicians should be aware of this entity. However, despite surgery and radiation therapy, the documented outcome is poor.