Sellar Mass in 2 Patients With Acute-Onset Headache and Visual Symptoms: Not Your Usual Pituitary Adenoma

Abstract

Background/objective: Clinical diagnosis of rare aggressive sellar malignancies requires a high index of suspicion. The objective was to report 2 patients with primary sellar atypical teratoid (AT)/rhabdoid tumor (RT) who presented with acute-onset headache and visual symptoms.

Case report: Patient 1 was a 45-year-old woman who presented with 3 weeks of headache and 1 week of eye pain and diplopia. Magnetic resonance imaging (MRI) identified a 2.2-cm sellar mass. Pituitary hormone testing showed elevated prolactin and suppressed luteinizing hormone, follicle-stimulating hormone, and estradiol levels. Patient 2 was a 32-year-old woman who presented with 1 month of headache and 1 week of diplopia. MRI showed a 2.1-cm sellar mass. Hormonal test results were reportedly unremarkable. Both patients did not have a significant medical history. They each underwent transsphenoidal resection. Surgical histology and molecular studies were consistent with primary sellar AT/RT. After surgery, patient 1 developed bilateral blindness and was lost to follow-up. Patient 2 developed hypopituitarism; her visual symptoms improved temporarily but recurred 2 weeks later. Pituitary MRI showed sellar recurrence. She underwent further debulking, but the tumor recurred promptly again. Despite radiation therapy, she died 4 months after the original presentation.

Discussion: AT/RT appears to be the most aggressive sellar malignancy.

Conclusion: Based on the 2 cases presented and the literature, I conclude that rapidly progressive headache with subsequent visual impairment in women with large sellar masses is almost pathognomonic of sellar AT/RT.

Keywords: acute-onset headache and visual symptoms; atypical teratoid/rhabdoid tumor; sellar compression symptoms; sellar mass.

Figure 1

Pituitary magnetic resonance imaging of patient 1 at initial presentation. Left, coronal view; right, sagittal view. The tumor invaded into the right cavernous sinus, circumferentially encased the cavernous right internal carotid artery, abutted the left cavernous sinus and the left internal carotid artery, and slightly displaced the optic chiasm. Arrows indicate the tumor.

Figure 2

Pituitary magnetic resonance imaging of patient 2 after the first transsphenoidal operation. Left, 24 days after the first transsphenoidal operation. The tumor splayed and compressed the optic chiasm with associated edema of the optic tracts, optic chiasm, and prechiasmatic optic nerves. There was mass effect on the right cavernous sinus and dural thickening along the planum sphenoidale. Middle and right, 1 and 12 days after the second operation, respectively. During the 11 days, the residual tumor in the right sella grew from 8 mm × 6 mm to 18 mm × 11 mm, and the residual tumor in the suprasellar cistern involving the optic chiasm grew from 3 to 6 mm in maximum thickness. Arrows indicate the tumor.