• ATRT
• Chemotherapy
• Infratentorial neoplasms
• MRI findings
• Neurosurgery
• Pediatric brain tumors

Primary sellar atypical teratoid/rhabdoid tumor (AT/RT) is the most aggressive sellar mass. Although rare, sellar AT/RT exhibits a very relentless clinical course and usually results in death within months to a few years after diagnosis. The best clinical evidence suggests that surgical debulking and timely adjuvant chemoradiation are most effective in prolonging survival. A preoperative radiological diagnosis of sellar AT/RT thus is crucial in informing patients and physicians about this devastating disease. This minireview summaries the imaging features of sellar AT/RT. magnetic resonance imaging features of sellar AT/RT and the much more common sellar mass, pituitary macroadenoma, are similar in most aspects: They are both isointense to brain gray matter on T1 and T2 imaging and enhance upon gadolinium administration. Suprasellar extension and cavernous sinus invasion are present in practically all cases of sellar AT/RT, but are also present in 50%-75% of pituitary macroadenomas, especially in large ones, suggesting that suprasellar extension and cavernous sinus invasion disproportionate to the tumor size may favor sellar AT/RT diagnosis. Since sellar AT/RT grows very rapidly and does not allow significant remodeling of perisellar structures, the imaging features of perisellar structures such as optic chiasm and cavernous sinus may be key for imaging diagnosis of sellar AT/RT although they have not been well described in sellar AT/RT. In limited cases of sellar AT/RT, optic chiasm degeneration and thinning, which are very common in pituitary macroadenoma, are not present, giving hope for using features of perisellar structures to diagnose sellar AT/RT by imaging.

• Primary sellar atypical teratoid/rhabdoid tumor
• Sellar imaging
• Sellar mass
• Pituitary macroadenoma
• Optic chiasm
• Cavernous sinus

The predominance of pituitary adenoma in the etiology of sellar masses often leads to the diagnostic fallacy of “availability bias” so that pituitary adenoma is almost always considered the most likely diagnosis of all sellar masses, even when clinical evidence suggests otherwise. Primary sellar atypical teratoid/ rhabdoid tumor (AT/RT) is the most aggressive sellar tumor. Most patients with sellar AT/RT are initially misdiagnosed with pituitary macroadenoma. Early diagnosis of sellar AT/RT is of paramount importance to counsel patients and family on the grave prognosis and to avoid futile surgical procedures. Since there are no discerning imaging features to differentiate AT/RT from other sellar tumors, the acuity of sellar compression symptoms characteristic of AT/RT is the only evidence indicative of the AT/RT diagnosis. Based on the biological and anatomical properties of the sella turcica and its surrounding structures, the nature, order of manifestation, and acuity of the sellar compression symptoms in response to sellar content expansion are mostly predictable. It is concluded that rapidly progressive headache and subsequent similarly rapidly progressive visual symptoms in a female with a large sellar mass are pathognomonic of sellar AT/RT (the “Yu rule”).

• Sellar atypical teratoid/rhabdoid tumor
• Sellar compression symptoms
• Headache
• Visual symptoms
• Symptom acuity
• Clinical diagnosis

• AT/RT
• Atypical teratoid/rhabdoid tumor
• Brain tumors
• SMARCB1
• Sellar region tumors

• Child
• Adult
• Humans
• Female
• Rhabdoid Tumor/surgery
• Teratoma/surgery
• Central Nervous System Neoplasms
• Prognosis
• Central Nervous System

• ATRT
• adult
• sellar ATRT
• sellar atypical teratoid/rhabdoid tumor

• DICER1-mutant
• case report
• methylation tumor classifier
• primary intracranial sarcoma