Laghari Altaf Ali, Javed Gohar, Khan Muhammad Faheem, Ahmed Syed Ijlal, Nathani Karim Rizwan, Ahmed Riyasat
Department of Surgery, Aga Khan University Hospital, Karachi, Pakistan.
Department of Surgery, Aga Khan University Hospital, Karachi, Pakistan.
World Neurosurg. 2017 Mar;99:811.e1-811.e5. doi: 10.1016/j.wneu.2016.12.086. Epub 2016 Dec 29.
Chondrosarcomas are very rare malignant, slow-growing tumors that develop in or near the petroclival region of the brain. We report a very rare case in which the tumor originated from left petrous bone and induced intraventricular hemorrhage leading to an acute comatose presentation.
A 28-year-old man initially presented to the outpatient department with a 1-month history of headache, vomiting, vertigo, and left facial numbness. A lesion at the cerebellopontine angle with extension into the middle cranial fossa was demonstrated on computed tomography and magnetic resonance imaging. The following night his condition worsened, and he presented to the emergency department with intraventricular hemorrhage with hydrocephalus. An external ventricular drain was placed in the emergency department to relieve hydrocephalus, and definitive surgical resection of the tumor was subsequently. Postoperatively, his Glasgow Coma Scale score improved, and he was transferred to the surgical intensive care unit where he remained for 3 days. He was subsequently stepped down to a special care unit and then to a ward room. The patient is currently awake and has grade II facial palsy (House-Brackmann), demonstrates spontaneous purposeful eye opening, inconsistently obeys single-step orders, demonstrates no meaningful phonation or vocalization, and has at least grade 4 power in all 4 extremities. He is currently fed through a nasogastric tube and is in rehabilitation.
Our experience of petroclival junction chondrosarcoma causing intraventricular hemorrhage may be the first to be documented. Preferred treatment of this highly malignant lesion is radical removal with postoperative radiotherapy.
软骨肉瘤是一种非常罕见的恶性、生长缓慢的肿瘤，发生于脑桥小脑角区或其附近。我们报告了一例极为罕见的病例，肿瘤起源于左侧岩骨并导致脑室内出血，进而引发急性昏迷表现。
一名28岁男性最初因1个月的头痛、呕吐、眩晕及左侧面部麻木症状就诊于门诊。计算机断层扫描和磁共振成像显示桥小脑角区有一病变并延伸至中颅窝。次日夜间，其病情恶化，因脑室内出血伴脑积水就诊于急诊科。在急诊科放置了外置脑室引流管以缓解脑积水，随后进行了肿瘤的根治性手术切除。术后，他的格拉斯哥昏迷量表评分有所改善，并被转入外科重症监护病房，在那里停留了3天。随后他被转至特殊护理病房，然后再转至病房。患者目前已清醒，有Ⅱ级面神经麻痹（House-Brackmann分级），能自主睁眼，对单步指令的服从不稳定，无有意义的发声，四肢肌力至少为4级。他目前通过鼻饲管进食，正在接受康复治疗。
我们关于岩斜区软骨肉瘤导致脑室内出血的经验可能是首例有文献记载的病例。这种高度恶性病变的首选治疗方法是根治性切除并术后放疗。
