Hemorrhagic Skull Base Chordoma Presenting As Chordoma Apoplexy

Abstract

Intratumoral hemorrhage/apoplexy in clival chordomas is extremely uncommon, with only 16 reported cases. The average age of patients was 46.2 years and slightly more than half were men. In cases published before 1990, all patients died from their disease without any intervention. Since then, 11 patients have undergone resection by a variety of approaches and there have been no deaths. The diagnosis of skull base chordomas relies on a combination of clinical presentation and radiographic features related to the location and invasion of the tumor. Chordomas presenting with sudden-onset symptoms should alarm the surgeon of a possible hemorrhage. As an illustration of this presentation, we describe a 58-year-old woman who presented with acute-onset headache and cranial nerve deficits. Computed tomography and magnetic resonance imaging demonstrated a hemorrhagic clival lesion with cavernous sinus extension. The patient underwent transsphenoidal resection of the lesion that resulted in the resolution of her symptoms. Histopathological evaluation of the lesion was consistent with chordoma with acute hemorrhagic components. Although intratumoral hemorrhage is rarely detected in chordomas, it should be considered a differential diagnosis of such lesions because prompt recognition and treatment are critical for patient survival.

Adjuvant stereotactic radiosurgery with or without postoperative fractionated radiation therapy in adults with skull base chordomas: a systematic review

“…ntracranIal chordomas are rare tumors that arise from prenatal remnants of the notochord located in the midline skull base clivus. [1][2][3][4] They account for 0.5% of all intracranial tumors and have an incidence of 1 case per million persons. 5,6 Despite initial surgery, chordomas are locally aggressive and have a high rate of local recurrence.…”

Sacrococcygeal chordoma with spontaneous regression due to a large hemorrhagic component

“…The etiology of chordoma hemorrhage is poorly understood but has been reported in the literature, mainly in clival chordomas [3,6]. Some authors have reported that rapid tumor growth without adequate matched blood supply may cause small friable vessels to rupture, and vascular proliferation and subsequent occlusion of smaller vessels may also cause tumor necrosis and hemorrhage [6]. To our knowledge, this is the first case of sacrococcygeal chordoma with SR due to a reduction in its hemorrhagic component described.…”

“…The decrease in size of the hemorrhage occurred as fast as in 4 weeks. The etiology of chordoma hemorrhage is poorly understood but has been reported in the literature, mainly in clival chordomas [3,6]. Some authors have reported that rapid tumor growth without adequate matched blood supply may cause small friable vessels to rupture, and vascular proliferation and subsequent occlusion of smaller vessels may also cause tumor necrosis and hemorrhage [6].…”

The utility of inflammatory biomarkers in predicting overall survival and recurrence in skull base chordoma

“…3,4 ) Chordomas exhibit highly variable clinical behavior, ranging from mild to highly aggressive, which complicates their management. 3,5 Gross-total resection (GTR) accompanied by adjuvant radiation therapy represents the current treatment paradigm for most skull base chordomas (SBCs); however, GTR is challenging given their proximity to critical neurovascular structures such as the cranial nerves and major vessels. [6][7][8][9][10][11] Despite an aggressive treatment regimen, the median overall survival (OS) is only 6-7 years.…”