Shitara Satoshi, Akiyama Yoshinori
Department of Neurosurgery, Tenri Hospital, Tenri, Nara, Japan.
Surg Neurol Int. 2014 May 23;5:75. doi: 10.4103/2152-7806.133105. eCollection 2014.
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.
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.
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.
非典型畸胎样/横纹肌样瘤(AT/RT)是一种罕见的中枢神经系统肿瘤,由原始横纹肌样细胞组成,这些细胞可能沿神经外胚层、间充质和上皮谱系分化。成人AT/RT罕见但并非完全没有。它通常起源于婴儿的后颅窝,但报告的大多数成人AT/RT发生在幕上,仅有4例起源于小脑,2例起源于脊髓。
一名44岁女性主诉视力障碍。我们进行了两次开颅手术,每次均部分切除,但标本中未发现任何恶性细胞。最后,我们从扩大切除的病变中确定了组织学诊断。她在初始治疗17个月后死于呼吸衰竭。
在成年患者鞍区病变的鉴别诊断中应考虑AT/RT。然而,成人AT/RT罕见,应进行适当的免疫组化评估以诊断这种罕见疾病。