Atypical teratoid/rhabdoid tumor in sellar turcica in an adult: A case report and review of the literature

Abstract

Background: Atypical teratoid/rhabdoid tumor (AT/RT) is a rare central nervous system tumor composed of primitive rhabdoid cells that may differentiate along neuroectodermal, mesenchymal and epithelial lineages. AT/RT in adults is rare but not completely exceptional. It generally arises from the posterior fossa of infants, but the broad majority of the reported AT/RT in adults manifested supratentorially with the exception of four cases that arose in the cerebellum and two that arose in the spinal cord.

Case description: A 44-year-old female complained of visual disturbance. We performed craniotomies twice and removed partially for each time, but any malignant cells were not found in the specimens. Finally, we determined histological diagnosis from the extended lesion. She died of respiratory failure 17 months after the initial treatment.

Conclusion: AT/RT should be considered in the differential diagnosis of a sellar lesion in adult patients. However AT/RT is rare in adults, the appropriate immunohistochemical evaluation should be performed to diagnose this rare entity.

Keywords: Adult; atypical teratoid/rhabdoid tumor; sellar turcica.

Figure 1

T1-weighted MRI coronal (a) and sagittal (b) images clearly show inhomogeneously gadolinium enhancing mass in sellar turcica extending into suprasellar lesion

Figure 2

T1-weighted MR coronal (a) and sagittal (b) images show a partial removal of the tumor after the first operation via translabial transsphenoidal approach

Figure 3

T1-weighted MR coronal (a) and sagittal (b) images show a partial removal of the tumor after the second operation via a basal interhemispheric approach

Figure 4

Photomicrographs of tumor specimens obtained in the patient from the first operation. There was no malignant cells. H and E. Original magnification ×100 (a) and ×400 (b)

Figure 5

T1-weighted MR coronal (a) and sagittal (b) images show an aggressive extension to the left cavernous sinus, the left orbital fossa and the left forehead with no regrowth in the sellar region

Figure 6

T1-weighted MR images (a-c) and contrast computed tomography image (d) show intracranial dissemination (arrow head) and distant metastasis in the lung (arrow head)

Figure 7

Histologically, the tumor tissue is mainly composed of hyperchromatic small cells with a relatively high nuclear/cytoplasm ratio and rhabdoid cells (H and E. Original magnification ×100 (a) and ×400 (b)). In immunohistochemical studies, Nestin (c), S-100 (d) and Vimentin (e) were positive and EMA (f) was focally positive. The proliferation index measured by Ki-67 was 85% in the areas of highest mitotic activity (g) and the tumor cells lack nuclear staining for the INI1 protein (h). Pathological findings were consistent with those of atypical teratoid/rhabdoid tumor