Minireviews
Copyright ©The Author(s) 2025. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Oncol. May 24, 2025; 16(5): 106292
Published online May 24, 2025. doi: 10.5306/wjco.v16.i5.106292
Importance of symptoms acuity for clinical diagnosis of primary sellar atypical teratoid/rhabdoid tumor
Run Yu
Run Yu, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA 90095, United States
Author contributions: Yu R conceived and wrote the papers.
Conflict-of-interest statement: The author reports no relevant conflicts of interest for this article.
Open Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Run Yu, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine, 200 Medical Plaza Driveway, Los Angeles, CA 90095, United States. runyu@mednet.ucla.edu
Received: February 21, 2025
Revised: March 26, 2025
Accepted: April 8, 2025
Published online: May 24, 2025
Processing time: 87 Days and 5 Hours
Abstract

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”).

Keywords: Sellar atypical teratoid/rhabdoid tumor; Sellar compression symptoms; Headache; Visual symptoms; Symptom acuity; Clinical diagnosis

Core Tip: The biological and anatomical characteristics of the sella turcica and its surrounding structures determine the nature, order of manifestation, and acuity of the sellar compression symptoms. The acuity of sellar compression symptoms is critically important to make a correct diagnosis of a non-adenoma sellar mass. In particular, rapidly progressive headache and subsequent similarly rapidly progressive visual symptoms in a female with a large sellar mass are pathognomonic of sellar atypical teratoid/rhabdoid tumor.