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Skull base chordomas: a management challenge

Ossama Al-Mefty and Luis A. B. Borba

✓ Because of their critical location, invasive nature, and aggressive recurrence, skull base chordomas are challenging and, at times, frustrating tumors to treat. Both radical surgical removal and high-dose radiation therapy, particularly proton beam therapy, reportedly are effective in tumor control and improve survival rates. The authors posit that these tumors are best treated with radical surgery and proton—photon beam therapy. During the last 5 years, they treated 25 patients (15 females and 10 males) who harbored pathologically diagnosed skull base chordomas. The mean age of the patients was 38.4 years (range 8–61 years). Previous surgery or radiation therapy was performed at other institutions in seven and two patients, respectively. The authors performed 33 surgical procedures on 23 patients. Radical removal (defined as absence of residual tumor on operative inspection and postoperative imaging) was achieved in 10 patients; subtotal resection (defined as resection of > 90% of the tumor) was achieved in 11 patients; and partial resection (defined as resection of < 90% of the tumor) was achieved in two patients. Radical surgical removal included not only the excision of soft-tumor tissue, but also extensive drilling of the adjacent bone. Adjuvant therapy consisted of postoperative combined proton—photon beam therapy (given to 17 patients and planned for one patient) and conventional radiation therapy (two patients); three patients received no adjunct therapy. To date, four patients have died. One patient who had undergone previous surgery and sacrifice of the internal carotid artery died postoperatively from a massive stroke; one patient died from adenocarcinoma of the pancreas without evidence of recurrence; and two patients died at 25 and 39 months of recurrent tumor. Permanent neurological complications included third cranial nerve palsy (one patient) and hemianopsia (one patient); radiation necrosis occurred in three patients. Of the 21 patients followed for more than 3 months after surgery, 16 have had no evidence of recurrence and five (including the two mortalities noted above) have had recurrent tumors (four diagnosed clinically and one radiologically). The mean disease-free interval was 14.4 months. A longer follow-up period will, hopefully, support the early indication that radical surgical removal and postoperative proton—photon beam therapy is an efficacious treatment. The use of skull base approaches based on the tumor classification introduced in this paper is associated with low mortality and morbidity rates.

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The transcondylar approach to craniocervical meningiomas

Marcio S. Rassi, Jean G. de Oliveira, and Luis A. B. Borba

Surgical removal of foramen magnum meningiomas poses great challenges due their deep location within the central skull base and their proximity to vital neurovascular structures. This video depicts the operative nuances of surgical management for a 59-year-old female who presented with a right-sided spinocranial meningioma. Simpson Grade I resection was achieved through a right transcondylar approach. The patient’s postoperative period was unremarkable, and she was discharged home on postoperative Day 5 for periodic follow-up. The transcondylar approach safely exposes the craniocervical junction at the anterior aspect of the neuraxis and still allows the surgeon to access the tumor through a parallel plane, with minimum morbidity.

The video can be found here: https://youtu.be/P0-kXjAkw9U.

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Surgical treatment of glomus jugulare tumors without rerouting of the facial nerve: an infralabyrinthine approach

Luis A. B. Borba, Samir Ale-Bark, and Charles London

Object

Glomus jugulare tumors are benign lesions located in the jugular foramen that may or may not extend into the middle ear, petrous apex, and upper neck; these growths sometimes invade intradurally. The surgical management of these tumors is a challenge to neurosurgeons and skull base surgeons. Because of their abundant vascularity, deep location, complex anatomy, and difficult surgical approach, their treatment, has been a controversial issue for many years. Despite advancements in nonsurgical techniques, the only treatment with proven efficacy is radical surgical removal. The authors present a series of patients treated with radical removal, in which the feasibility of removing glomus jugulare tumors with low morbidity and a surgical approach limited to tumor removal are discussed. The extent of surgical exposure is tailored with emphasis placed on the routine anterior transposition of the facial nerve.

Methods

Between May 1997 and March 2004, 24 patients with glomus jugulare tumors were treated; 17 patients were women and seven were men. Their mean age at the time of diagnosis was 50 years (range 18–71 years). The most common symptom was hearing loss in 77%, followed by dysphagia and dysphonia in 55% of patients. In seven patients the clinical presentation was a facial palsy. Radical tumor removal was achieved in 23 patients. An anterior facial nerve transposition was not needed in any case. No surgery-related death was recorded in this series, although one patient died of a pulmonary embolism 70 days after the procedure. A one-stage procedure was performed in 23 patients and a two-stage procedure was used in the other patient. Cerebrospinal fluid leakage occurred in two patients. The lower cranial nerve function was worse in eight patients; however, only one had a new deficit. The facial nerve was preserved in all patients except one, in whom a large intradural tumor caused a temporary facial palsy. In the patients with pre-operative facial palsy, the tumor only compressed the nerve in three and it invaded the nerve in four. The nerve was decompressed in the cases with no invasion and a graft was placed in the others. The greater auricular nerve was used as a graft in three and the sural nerve was used in one. On follow-up review, the facial nerve function was House–Brackmann Grade 3 in three patients and Grade 2 in three. After 6 months of follow up with no improvement, one patient was referred for a facial muscle transfer.

Conclusions

The surgical technique must be tailored to each case. The authors believe that the standard surgical approach to jugular foramen tumors with anterior transposition of the facial nerve should be avoided, and that the extent of surgical exposure must be tailored to each case based on the extent of the tumor and the clinical symptoms. Lower morbidity rates and radical removal can be achieved with a good surgical plan.

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Cranial chordomas in children and adolescents

Luis A. B. Borba, Ossama Al-Mefty, Robert E. Mrak, and James Suen

✓ Chordomas are rare tumors that usually occur in adults. This report describes four cases of intracranial chordomas treated in patients 20 years of age or younger by the senior author (O.A.M.) during a 4-year period. The authors also reviewed the literature on pediatric patients, which revealed that the clinical presentations, histological patterns, and behaviors of these tumors differ considerably depending on whether the patient is younger or older than 5 years of age. The younger population had a wider range of presenting symptoms, a greater prevalence of atypical histological findings with aggressive behavior, a greater range of cellularity than the classic chordomas, and a higher instance of metastasis; it showed no chondroid component compared to a 17.1% instance in the older patients. The prognosis for patients with a chordoma is related directly to the histological pattern of the tumor; the atypical chordoma carries a poor prognosis. The prognoses for children older than 5 years of age with a classic or chondroid tumor were not significantly different (p = 0.788). At follow up, the difference in survival rates between patients undergoing surgery plus radiation therapy and those who had surgery alone was statistically significant (p = 0.00446). No correlation was found between radical resection or radiation therapy and an improved prognosis for patients younger than 5 years of age who had a tumor with an atypical histological pattern. This study identifies and delineates the distinction between these age groups and provides a review of the potential prognostic factors.

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Microsurgical management of a high-grade brain arteriovenous malformation in the central lobe after unsuccessful radiosurgery

Jean G. de Oliveira, Carlos R. Massella Jr., Carlos Vanderlei M. de Holanda, Miguel Giudicissi-Filho, and Luis A. B. Borba

The recommendation for the great majority of high-grade brain arteriovenous malformations (bAVMs) is observation, except for those patients with recurrent hemorrhages, progressive neurological deficits, steal-related symptoms, or AVM-related aneurysms, for whom intervention should be considered. These are general recommendations, and the decision should be made on an individual basis. Surgical resection of an AVM in the central lobe may cause postoperative sensorimotor deficits since this anatomical region includes the pre- and postcentral gyri on the lateral surface and the paracentral lobule on the medial surface.

The authors present a patient with a ruptured high-grade bAVM in the central lobe who underwent previous surgery for hematoma evacuation and previous radiosurgery, and whose indication for reoperation was proposed based on progressive hemiparesis. Microsurgical resection was possible after a wide frontoparietal craniotomy, which made all the nidus borders accessible. This case illustrates the anatomy and surgical technique for large nidus AVMs in eloquent areas, showing that complete microsurgical resection is possible with good clinical outcome.

The video can be found here: https://youtu.be/Cpd1PK6BLIM.

Open access

Multimodal treatment of a pediatric posterior fossa arteriovenous malformation employing endovascular and microsurgical techniques

Zeferino Demartini Jr., Guilherme H. W. Ceccato, Érico S. G. G. da Trindade, and Luis A. B. Borba

Intracranial hemorrhage is the most common presentation of posterior fossa arteriovenous malformations (AVMs) and may have serious consequences. The authors present a case of a 7-year-old girl with headache, vomiting, dysmetria, and ataxia due to a ruptured cerebellar grade III AVM. After two sessions of embolization, the patient underwent total microsurgical resection through a suboccipital craniotomy. There were no additional postoperative deficits, and the patient improved progressively during 6 months of rehabilitation. These challenging lesions should be removed after rupture, especially in children with long-term cumulative risk of rebleeding. Multimodal treatment reduces the perioperative bleeding, allowing better outcomes for pediatric AVM.

The video can be found here: https://youtu.be/HQWnjD8ENZQ

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The transcondylar approach to extradural nonneoplastic lesions of the craniovertebral junction

Ossama Al-Mefty, Luis A. B. Borba, Nobuo Aoki, Edgardo Angtuaco, and T. Glenn Pait

✓ Ventral extradural lesions at the craniovertebral junction are commonly exposed through the transoral or transmaxillary approach. The disadvantages of these approaches include: 1) difficulty in reaching laterally located lesions; 2) ineligibility of patients with an intradental distance of less than 25 mm or severe macroglossia; 3) the need for a separate procedure for stabilization and fusion; and 4) the risk of infection from transgressing a contaminated field. In this report, the authors describe the use of the transcondylar approach to extradural nonneoplastic lesions of the anterior craniovertebral junction for decompression and stabilization. Advantages of this approach include: 1) a short distance to the lesion; 2) a wide surgical envelope; 3) direct visualization of the dural sac, eliminating manipulation of the brainstem or upper spinal cord; 4) easy identification and control of the ipsilateral vertebral artery; 5) direct visualization and preservation of the lower cranial nerves; and 6) a sterile field. In addition, occipitocervical fusion and instrumentation can be performed during the same procedure. The transcondylar approach, based on anatomical studies in cadavers, was used to treat eight patients with ventral nonneoplastic lesions at the craniocervical junction. The technique and results are described.

Open access

Microsurgical resection of an inferior cerebellar peduncle cavernous malformation: 3-Dimensional operative video

Guilherme H. W. Ceccato, Rodolfo F. M. da Rocha, Julia Goginski, Pedro H. A. da Silva, Gabriel S. de Fraga, Marcio S. Rassi, and Luis A. B. Borba

Brainstem cavernous malformations are especially difficult to treat because of their deep location and intimate relation with eloquent structures. This is the case of a 26-year-old female presenting with dizziness, dysmetria, nystagmus and unbalance. Imaging depicted a lesion highly suggestive of a cavernous malformation in the left inferior cerebellar peduncle. Following a suboccipital midline craniotomy, the cerebellomedullary fissure was dissected and the lesion was identified bulging the surface. The malformation was completely removed with constant intraoperative neurophysiological monitoring. The patient presented improvement of initial symptoms with no new deficits. Surgical resection of brainstem cavernous malformations can be successfully performed, especially when superficial, using the inferior cerebellar peduncle as an entry zone.

The video can be found here: https://youtu.be/-GGZe_CaZnQ.

Open access

Microsurgical resection of foramen magnum meningioma through a transcondylar approach: three-dimensional operative video

Guilherme H. W. Ceccato, Rodolfo F. M. da Rocha, Duarte N. C. Cândido, Wladimir O. Melo, Marcio S. Rassi, and Luis A. B. Borba

Foramen magnum (FM) meningiomas are challenging lesions. We present the case of a 38-year-old female with neck pain, dysphonia, and slight twelfth nerve palsy. Imaging workup was highly suggestive of an FM meningioma, and microsurgical resection with the aid of intraoperative neurophysiological monitoring was indicated. A transcondylar approach was employed, the vertebral artery was mobilized, and the tumor was completely removed. Postoperative MRI demonstrated complete resection. There were no signs of cervical instability. The patient presented with improvement of her symptoms and no new neurological deficit on follow-up. FM meningiomas can be successfully resected using a transcondylar approach, since it increases the exposure of the ventral FM, allowing the surgeon to work parallel to the skull base and flush with the tumor’s attachment. Informed consent was obtained from the patient for publication of this operative video.

The video can be found here: https://youtu.be/itfUOB-6zM0.

Free access

Simultaneous cochlear implantation as a therapeutic option in vestibular schwannoma surgery: case report

Pedro Helo dos Santos Neto, Johnni Oswaldo Zamponi Jr., Rogério Hamerschmidt, Gislaine Richter Minhoto Wiemes, Marcio S. Rassi, and Luis A. B. Borba

Hearing loss is the most common symptom of vestibular schwannomas (VSs). The management of these lesions includes observation, radiosurgery, and microsurgical resection. Hearing preservation and rehabilitation are the major challenges after the tumor treatment. A 43-year-old man with previous left-sided profound hearing loss and tinnitus presented with a 2-mm left-sided intracanalicular VS. The decision was made to perform a simultaneous cochlear implantation (CI) and microsurgical resection of the tumor. The patient did well postoperatively, with significant improvement of tinnitus, sound localization, and speech recognition in noise. Previous reports of simultaneous CI and VS resection in patients with neurofibromatosis type 2 and sporadic VS in the only hearing ear have been described. The role of CI in patients with VS and normal contralateral hearing has been recently described, showing positive outcomes due to the binaural benefits. Tinnitus also can be treated by the implantation of the cochlear device. The simultaneous microsurgical removal of VS and implantation of a cochlear device is a feasible approach in patients with unilateral hearing loss and severe tinnitus.