Clin Neuropathol. 2021 Sep-Oct;40(5):262-270. doi: 10.5414/NP301352.
An atypical teratoid rhabdoid tumor (ATRT) is a pediatric embryonic tumor of the central nervous system and is uncommon in adults. We report a case of a 33-year-old female who presented with multiple dural lesions that were diagnosed as ATRT. She had a past history of endoscopic transnasal transsphenoidal and subsequent transcranial decompression of suprasellar lesion 6 months prior, with a presumptive diagnosis of atypical pituitary adenoma, which on retrospective evaluation was confirmed as sellar ATRT. Adult sellar ATRT, though rare, has now been proposed as a distinct clinicopathological and genetic variant that is predominantly seen in middle-aged women. We discuss the uniqueness of this rare aggressive tumor with reference to the age, location, and the challenges faced in the clinical and pathological diagnosis.
非典型畸胎样/横纹肌样瘤(ATRT)是一种儿童中枢神经系统胚胎性肿瘤,在成人中较为罕见。我们报告了一例 33 岁女性患者,其表现为多个硬脑膜病变,被诊断为 ATRT。患者 6 个月前曾因鞍上病变行经鼻蝶内镜下和随后的经颅减压,术前诊断为不典型垂体腺瘤,回顾性评估后证实为鞍内 ATRT。成人鞍内 ATRT 虽然罕见,但现在已被认为是一种独特的临床病理和遗传变异型,主要见于中年女性。我们讨论了这个罕见侵袭性肿瘤的独特性,包括年龄、位置以及在临床和病理诊断中面临的挑战。