Moretti Costanzo, Lupoi Domenico, Spasaro Francesca, Chioma Laura, Di Giacinto Paola, Colicchia Martina, Frajoli Mario, Mocini Renzo, Ulisse Salvatore, Antonelli Manila, Giangaspero Felice, Gnessi Lucio
Unit of Endocrinology and Diabetes, Section of Reproductive Endocrinology University of Tor Vergata, San Giovanni Calibita Fatebenefratelli Hospital Tiber Island, Rome, Italy.
Clin Med Insights Case Rep. 2013 Nov 27;6:177-82. doi: 10.4137/CCRep.S12834. eCollection 2013.
Here we present the case of a 60-year-old woman with a rare sellar region atypical teratoid/rhabdoid tumor (AT/RT), complicated by lung metastasis and treated with neurosurgery, radiotherapy, and chemotherapy. The patient had recurrent headache associated with left cavernous sinus syndrome after a previous endonasal transsphenoidal resection for a presumptive pituitary macroadenoma. Pituitary magnetic resonance imaging showed a tumor regrowth in the original location with a haemorrhagic component involving the left cavernous sinus. A near complete transsphenoidal resection of the sellar mass was performed followed by 3 months of stereotactic radiotherapy. Because of a worsening of the general clinical conditions, respiratory failure, and asthenia, the patient underwent a contrast enhanced computer tomography of the whole body which showed the presence of lung metastasis. The histopathological diagnosis on samples from pituitary and lung tissues was AT/RT. The patient survived 30 months after diagnosis regardless chemotherapy. In the adult, the AT/RT should be considered as a possible rare, aggressive, and malignant neoplasm localized in the sella turcica.
在此，我们报告一例60岁女性患有罕见的鞍区非典型畸胎样/横纹肌样瘤（AT/RT），并伴有肺转移，接受了神经外科手术、放疗和化疗。该患者曾因疑似垂体大腺瘤接受经鼻蝶窦切除术，术后出现复发性头痛并伴有左侧海绵窦综合征。垂体磁共振成像显示原部位肿瘤复发，伴有出血成分累及左侧海绵窦。对鞍区肿物进行了近乎完全的经蝶窦切除术，随后进行了3个月的立体定向放疗。由于一般临床状况恶化、呼吸衰竭和乏力，患者接受了全身增强计算机断层扫描，结果显示存在肺转移。垂体和肺组织样本的组织病理学诊断为AT/RT。无论是否进行化疗，该患者在诊断后存活了30个月。在成年人中，AT/RT应被视为一种可能罕见、侵袭性强且恶性的肿瘤，定位于蝶鞍。
