The management of chordomas and chondrosarcomas involving the cranial base remains controversial. The options for therapy include biopsy, partial resection, radical resection, and various forms of radiotherapy. In this article, we analyze the outcome of 60 patients with cranial base chordoma or chondrosarcoma treated with extensive surgical resection between 1984 and 1993. Forty-six patients had chordomas, and 14 had low-grade chondrosarcomas; 50% of these patients had been treated previously. Preoperative studies included computed tomography, magnetic resonance imaging, cerebral angiography, and balloon occlusion test of the internal carotid artery, as indicated. Magnetic resonance imaging was performed on all patients during follow-up. The surgical approaches used for tumor resection were predominantly the following: subtemporal, transzygomatic, transcavernous, and transpetrous apex; subtemporal and infratemporal; extended frontal; and extreme lateral transcondylar. Staged operations with a combination of approaches were used when necessary (52% of cases) to remove a tumor more completely. Statistical analysis was done by the chi 2 test and correlation matrix. Sixty-seven percent of the patients had total or near-total resection. Twenty percent of the patients received postoperative radiotherapy. Eleven patients died during the postoperative follow-up period, nine with chordomas and two with chondrosarcomas. Three patients died because of systemic complications within 3 months after surgery, five died because of tumor recurrence, one died from unrelated causes, and two died from late complications of radiotherapy. The recurrence-free survival rate for all tumors was 80% at 3 years and 76% at 5 years. Chondrosarcomas had a better prognosis than chordomas (recurrence-free survival rates, 90% at 5 years and 65% at 5 years, respectively; P = 0.09). Patients who had undergone previous surgery had a greater risk of recurrence (5-year recurrence-free survival rate, 64%) than did patients who had not undergone previous surgery (5-year recurrence-free survival rate, 93%; P < 0.05). Patients with total or near-total resection had a better 5-year recurrence-free survival rate (84%) than did patients with partial or subtotal resection (64%) (P < 0.05). Postoperative leakage of cerebrospinal fluid was the most frequent complication (30% of patients) and was found to increase the risk of permanent disability. Patients who had undergone previous radiotherapy had a greater risk of death in the postoperative period (within 3 months of their operations) and during follow-up. However, total or near-total resection did not increase the rate of postoperative disability.(ABSTRACT TRUNCATED AT 400 WORDS)

A subtemporal-preauricular infratemporal fossa approach to remove 22 large neoplasms involving the lateral and posterior cranial base is detailed. The areas from which a neoplasm could be removed by this approach included the sphenoid and clival bone; the medial half of the petrous temporal bone; the infratemporal fossa; the nasopharynx; the retro- and parapharyngeal area; the ethmoid, sphenoid, and maxillary sinuses; and the intradural clivus-foramen magnum area. The pathology of the neoplasms included benign tumors such as meningioma, malignant cartilaginous neoplasms such as chordoma, and other malignant lesions such as nasopharyngeal carcinoma. This approach offers many advantages over other anterior and lateral approaches to the lateral and posterior cranial base: these include minimal brain retraction; direct access to the ipsilateral petrous and upper cervical internal carotid artery; reconstruction of extensive cranial base defects, often with the use of a vascularized rectus abdominus flap; preservation of the hearing conduction mechanism when it is not involved by tumor; and the maintenance of excellent facial nerve function postoperatively. The use of an anterior extradural approach (transethmoidal) and of an intradural approach (frontotemporal or retromastoid), either concurrently or separately, is necessary in some patients to effect total tumor removal. The most serious complication in this series was the death of a patient due to postoperative infection and bilateral carotid artery rupture, which may have been avoided by the use of a rectus abdominis muscle flap for reconstruction. Among the 21 surviving patients, 18 had a good outcome, two had a fair outcome, and one with preexisting neurological deficits had a poor outcome. One of the surviving patients with a chordoma died of pulmonary metastases 1 year later, without evidence of local recurrence. The length of postoperative follow-up evaluation in these patients is insufficient to make any judgment about the effectiveness of this surgical approach in achieving a cure or long-term control of the tumors described.

Intracranial hemorrhage (ICH) from an intracranial aneurysm or arteriovenous malformation is a grave complication of pregnancy and is responsible for 5 to 12% of all maternal deaths. We critically analyzed 154 cases of verified ICH during pregnancy from an identified intracranial lesion, including 2 patients treated at our institution and 152 cases previously reported in the literature in English. Aneurysms were responsible for ICH in 77% of patients, and arteriovenous malformations in 23%. Hemorrhage occurred antepartum in 92% of patients and postpartum in 8%. Women with angiomatous hemorrhage were younger than those with aneurysmal hemorrhage; however, in contrast to previous reports, we found no differences between angiomatous and aneurysmal hemorrhage with respect to parity or gestational age at the time of the initial hemorrhage. Hypertension and/or albuminuria were present at some time during the pregnancy in 34% of patients with documentation, which sometimes made it difficult to differentiate angiomatous or aneurysmal ICH from that associated with eclampsia. In a logistic regression analysis, surgical management of aneurysms, but not arteriovenous malformations, was associated with significantly lower maternal and fetal mortality, independent of other covariants. For those patients with a lesion not operated on, cesarean delivery afforded no better maternal or fetal outcome than did vaginal delivery. We conclude that the decision to operate after ICH during pregnancy should be based upon neurosurgical principles, whereas the method of delivery should be based upon obstetrical considerations. The perioperative and anesthetic management of the pregnant patient with a neurosurgical complication is discussed.

Aggressive microsurgical resection of chordomas can be followed by long-term, tumor-free survival with good functional outcome. A more conservative strategy is recommended in reoperation cases, especially after previous radiotherapy, to reduce postoperative complications.