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Two-stage skull base surgery for tumours extending to the sub- and epidural spaces.

The surgical management of extensive skull base neoplasms, which often extend to both the sub- and epidural spaces, is still a great challenge with considerable risk. The authors report 12 cases in which a two-stage operation was performed for such nonmalignant tumours. The series consisted of four cavernous sinus meningiomas, one sphenoid-ridge meningioma, one cerebello-pontine angle meningioma, three pituitary adenomas, two chordomas, and one fibroma. Our operative strategy involved removal of the epidural part of the tumour and extensive skull base reforming during the first stage. After approximately one month, the second stage operation was performed by removing the residual subdural parts and the affected dura, which were less vascular, with dural plasty and subsequent spinal drainage. No complications such as cerebrospinal fluid (CSF) leakage or infection were observed. During the long-term follow-up (1.4 to 4.6 years, with a mean of 2.7 years), tumour recurrence was observed in a single case. In conclusion, the major advantages of this procedure were as follows: [1] improvement of the total removal rate, [2] prevention of postoperative CSF leak and infection, [3] residual tumours were avascular, necrotic, and dwindling, and also shifted outwards resulting in less adhesions to the brain. Although it may counter the trend toward less invasive procedures, the two-staged skull base surgery warrants serious consideration as an option for the management of patients with such extensive cranial base tumours.

Adenoma↗

Endoscopically assisted anterior cranial skull base resection of sinonasal tumors.

The traditional approach to sinonasal tumors involving the base of skull has been the anterior craniofacial resection. Endoscopic techniques have created the potential to approach the intranasal aspect of skull base lesions without external incisions and still develop an en bloc resection when removed. We report our initial experience with skull base neoplasms in which the otolaryngic portion of the standard resection was accomplished instead through an endoscopic approach. The nature of lesions favorable for this approach and associated technical issues are discussed. Although we do not consider this approach a replacement for the traditional anterior craniofacial resection, it is an important adjunct in the skull base surgeon's armamentarium.

Adenocarcinoma↗

Use of preoperative MR to predict dural, perineural, and venous sinus invasion of skull base tumors.

PURPOSE: To assess the accuracy of MR imaging in predicting dural, venous sinus, and perineural invasion by skull base tumors. METHODS: The preoperative MR images of 22 patients who had resection of skull base neoplasms were evaluated for the following characteristics: dural enhancement, pial enhancement, local perineural invasion by adjacent tumor, and venous sinus invasion by tumor. The greatest width of dural enhancement was measured, and the character of dural enhancement was noted. The pathologic and surgical reports were reviewed retrospectively with specific attention to dural, venous, and local perineural invasion. RESULTS: Of the 22 patients studied, dural invasion by tumor was confirmed in eight patients, vascular invasion in six patients, and perineural invasion in four patients. The sensitivity of dural enhancement in predicting invasion was 88%, the specificity 50%, and the accuracy 64%. When enhancement and focal nodularity were present, the sensitivity remained at 88%; however, specificity was 100% and accuracy 95%. If the dural enhancement was more than 5 mm thick, sensitivity, specificity, and accuracy were 75%, 100%, and 91%, respectively. Predicting tumor invasion of the dura by the presence of pial enhancement was 50% sensitive and 100% specific. Venous sinus/jugular vein invasion was predicted with 100% sensitivity, 94% specificity, and 95% accuracy. Local perineural invasion was predicted with 100% sensitivity, 50% specificity, and 59% accuracy. CONCLUSIONS: The presence of pial enhancement, focal dural nodules, or dural thickening of more than 5 mm is highly accurate in predicting the presence of neoplastic dural invasion. Linear enhancement of dura does not imply dural infiltration by tumor. Venous invasion by tumor can be predicted accurately with preoperative MR imaging.

Adult↗

Surgical management of intraosseous skull base tumors with aid of Operating Arm System.

Invasion of bone and critical neurovascular structures often impedes complete resection of intraosseous skull base neoplasms, and these lesions tend to recur unless all infiltrated bone is removed. Evolving experience with image guidance over the past few years indicates the potential value of neuronavigation in skull base lesions diffusely infiltrating or fixed to bone structures. We report our early experience with the Radionics Operating Arm System (OAS), specifically emphasizing its utility as an adjunct in the treatment of intraosseous skull base tumors, mainly meningiomas. In April 1995 the OAS was introduced into clinical use at the neurosurgical university clinic in Münster, Germany. Since then, the system's utility has been explored in 10 patients out of the total neuronavigation series presenting with intraosseous skull base tumors (nine females and one male, mean age 47 years; nine meningiomas, one chordoma). For navigational planning, both 3-mm computed tomography scans and a set of 3-mm fat-suppression magnetic resonance images were chosen. At least four adhesive skin markers were used for system calibration. The system was technically usable in all cases in this small series. Because of the relative immobility of the bone structures and/or the tumor, no significant deviation from the preoperative registration accuracy was noted at the end of the procedures. The main advantages were easier localization and resection of infiltrated bone, which is often not grossly identifiable, even under the microscope. Our preliminary experience with the OAS suggests that image guidance is helpful in this type of lesion, providing better anatomical orientation during surgery and delineating tumor margins and their relation to critical neurovascular structures. The problem of a possible intracranial tumor and brain shift can be neglected in these lesions. The system facilitates resection by volumetric contour information, allowing more aggressive and complete resection.

Adult↗

Loss of heterozygosity in the retinoblastoma tumor suppressor gene in skull base chordomas and chondrosarcomas.

BACKGROUND: The retinoblastoma (Rb) gene is a well characterized tumor suppressor gene in which loss of heterozygosity has been implicated in a number of malignancies including osteosarcoma and breast carcinoma. Chordomas and chondrosarcomas are rare skull base neoplasms with a propensity for local recurrences, resistance to conventional radiotherapy, and a 5%-30% incidence of metastases. Except for the so called "chondroid chordoma," histologic features do not correlate with the clinical behavior or growth patterns of these tumors. No study to date has investigated what role tumor suppressor genes or oncogenes play in the development and continued growth of these rare neoplasms. METHODS: In order to evaluate the role of the retinoblastoma tumor suppressor gene in chordomas and chondrosarcomas we screened seven chordomas and two chondrosarcomas located at the skull base for loss of heterozygosity (LOH) of the Rb gene. Genomic DNA was extracted from tumor specimens as well as matched control tissue and utilizing a polymerase chain reaction technique, intron 17 and 20 were amplified from each specimen. The intron 17 product was then digested with the restriction endonuclease X ba1 followed by electrophoresis on a 1% agrose gel. The intron 20 amplified products were electrophoresed on a nondenaturing 6% polyacrylamide gel. RESULTS: We demonstrated LOH at intron 17 of the retinoblastoma gene in 2/7 chordomas and in 0/2 chondrosarcomas. The two chordomas possessing LOH were particularly aggressive tumors demonstrating extensive involvement of the skull base and rapid recurrences following radical resections. CONCLUSIONS: Alterations of the Rb gene may play a role in the growth of skull base chordomas with LOH of the Rb gene serving as a marker for more aggressive tumors. This report represents the first study evaluating the Rb gene in chordomas or chondrosarcomas and is the first report of allelic loss of the Rb gene in skull base chordomas.

Adolescent↗

Microvascular reconstruction of the skull base: indications and procedures.

PURPOSE: The aim of the current study was to review the use of free tissue transfer for reconstruction of the skull base and for coverage of intracranial contents. PATIENTS AND METHODS: From 1990 until 1996, revascularized flaps were transferred to the skull and the skull base in 11 patients in whom intracranial/extracranial resection of tumors of the skull base was performed in cooperation with the Department of Neurosurgery. The defects resulted from removal of squamous cell carcinomas (n = 4), basal cell carcinomas (n = 4), malignant melanoma, malignant schwannoma, and malignant meningioma. Defect repair was accomplished by revascularized transfer of latissimus dorsi muscle flaps in seven cases and rectus abdominis flaps and forearm flaps in two cases each. In five patients with extensive intracranial tumor spread, reconstruction was performed for palliative reasons. RESULTS: A safe soft tissue closure of the intracranial and intradural space was achieved in all patients, whereas the contour of the facial skull and the neurocranium was satisfactorily restored at the same time. By using the entire length of the grafted muscle, the vascular pedicle could be positioned next to the external carotid artery and conveniently connected to the cervical vessels. The mean survival time of the patients with palliative treatment was 8.4 months, with an average duration of hospital stay of 24.5 days. CONCLUSIONS: Despite the increased surgical effort of revascularized tissue transfer, microvascular reconstruction of large skull base defects appears to be justified, even as a palliative measure.

Adolescent↗

Advances in imaging of the paranasal sinuses.

Imaging technology has played a significant role in the diagnosis and management of sinonasal disorders. Plain sinus films are almost exclusively replaced by CT in the work-up for inflammatory sinus disease. MRI provides complementary information to CT in cases of sinonasal and skull-base neoplasms. The evolution of endoscopic surgical techniques for the paranasal sinuses and skull base is made possible by the parallel advancement of imaging technologies. Recent advances that are currently in clinical use have included multidetector row CT scanners and computer image-guidance systems for surgery. Three-dimensional CT angiography, image-guided CT-MR fusion, and intraoperative image-guidance are new techniques that are currently being evaluated. As imaging technology continues to advance, so does the capability to treat diseases beyond the sinuses and skull base with minimally invasive, endoscopic approaches.

Humans↗

Teratomas of the head and neck region.

BACKGROUND: Teratomas are exceptionally rare malformations in the head and neck region. They are mostly benign but as a direct result of their rarity, most clinician's experience of these tumours is very limited, and consequently most of the associated literature consists of single case reports. In this paper, however, all the cases managed by a major Craniofacial Unit (the Australian Craniofacial Unit) were reviewed to attempt to identify common problems encountered in their management. MATERIAL AND METHODS: All cases managed by the Australian Craniofacial Unit over the last 25 years were reviewed. In total a series of nine cases was identified, but two were seen and operated on in overseas centres and the data in these cases were incomplete, and have been excluded from the study. Case note, radiology and pathology review was undertaken to collect data. RESULTS: In total a series of seven cases was identified as suitable for inclusion in this study. Six of these have had a minimum of 9 years follow-up, three having completed growth. CONCLUSION: The initial and subsequent management demonstrates that these tumours when benign can be successfully removed, but depending on the affected site may require continued multidisciplinary management until growth has finished.

Brain Neoplasms↗

Lateral craniofacial approaches to the skull base and infratemporal fossa.

The field of skull base surgery has expanded dramatically the surgical horizons in the treatment of skull base neoplasms, which, previously, were considered inoperable because of their locations and relation to vital structures. This article aims to offer improved understanding of surgical indications and appreciation for some of the details of surgical techniques involved.

Cranial Fossa, Posterior↗

Variability in the enhancement of the normal central skull base in children.

We studied the signal and enhancement characteristics of the central skull base prospectively in 40 children aged 13 days to 8 years, on a 1.5 T MRI system. Identical standard short TR/TE spin echo sequences in the sagittal plane were performed before and after intravenous gadolinium-DTPA. The sequences used for comparison were filmed at identical window and level settings. Three independent observers assessed (1) the intensity of contrast enhancement of the basisphenoid, basiocciput and presphenoid, (2) the signal intensity of the spheno-occipital synchondrosis, (3) the degree of pneumatization of the sphenoid sinus and (4) the uniformity of signal intensity reflecting fatty replacement of the marrow of the basisphenoid, basiocciput and presphenoid. In 16% and 28% respectively of cases there was no enhancement of the basisphenoid and basiocciput after gadolinium administration; in 42% and 44% there was mild enhancement, and moderate or intense enhancement was observed in 42% and 28% of cases. Even when there was irregular fatty replacement, residual hemopoietic clements could enhance intensely. When skull base neoplasms are being investigated, the normal signal irregularity and enhancement of the central skull base in children must not be confused with pathologic invasion of the marrow.

Child↗

Drop metastasis from sinonasal undifferentiated carcinoma: clinical implications.

STUDY DESIGN: The first reported case of multiple intradural, extramedullary spinal metastasis from sinonasal undifferentiated carcinoma is presented. OBJECTIVES: To elucidate the mechanisms by which metastatic disease invades the spinal axis, and to discuss the possibility of spinal drop metastasis from head and neck tumors that invade the dura. SUMMARY OF BACKGROUND DATA: Sinonasal undifferentiated carcinoma is a rare yet aggressive neoplasm of the upper airways and anterior skull base. This neoplasm is known to invade the cranial vault and brain locally. However, it has not previously been reported to seed the cerebrospinal fluid or result in drop metastasis. Such drop metastasis may result in significant neurologic deficit if not diagnosed and treated in a timely manner. METHODS: This report is based on a single patient treated by a multidisciplinary team from the departments of neurosurgery, otolaryngology, and radiation oncology at the University of Southern California School of Medicine. RESULTS: This patient initially underwent resection and local radiation therapy for sinonasal undifferentiated carcinoma of the anterior skull base. At the time of surgery, the tumor was noted to violate the dura and arachnoid along the subfrontal plane. At 11/2 years after the initial treatment, a bandlike distribution developed at T2 as well as paresthesias and numbness below that level. Imaging of the spine showed an intradural, extramedullary tumor at T2 consistent with a schwannoma or meningioma. The patient underwent a laminectomy and tumor resection, which showed poorly differentiated sinonasal carcinoma. Local radiation therapy was administered, and the patient experienced complete recovery of neurologic function. Bilateral leg pain and weakness developed 14 months later. Magnetic resonance imaging of the spine showed a new intradural, extramedullary lesion at T12, remote from the first lesion. This second metastasis was managed with surgical resection and adjuvant radiation therapy. CONCLUSIONS: This is the first reported case of a sinonasal carcinoma leading to intradural extramedullary metastasis. The primary tumor likely seeded the cerebrospinal fluid, thus resulting in drop metastasis. Patients with sinonasal undifferentiated carcinoma that invades the dura should be monitored closely for evidence of metastasis before symptoms develop.

Carcinoma↗

The role of chemotherapy for skull base carcinomas and sarcomas.

The rarity of most primary skull base carcinomas and sarcomas has prevented a careful examination of the role of chemotherapy in these diseases. For advanced nasopharyngeal cancer, however, convincing data have now been generated supporting the role of systemic chemotherapy in conjunction with definitive locoregional treatment. Chemotherapy also seems to have a role in the management of other squamous cell head and neck cancers, and its use concurrently with radiation may be appropriate when extension to the base of the skull is identified. The role of chemotherapy in the management of the other skull base neoplasms remains less well established. Existing evidence is little more than anecdotal, and the use of systemic chemotherapy should generally be restricted to the palliative treatment setting. The ultimate role of this intervention in the aggressive sinonasal undifferentiated carcinomas and primary neuroendocrine carcinomas is unknown, but for these diseases, chemotherapy is a reasonable addition to locoregional treatment.

Antineoplastic Agents↗

Local invasivity of glioblastoma multiforme with destruction of skull bone. Case report and review of the literature.

Primary tumors originating from cells of the glial lineage usually affect predominantly the white matter of the brain. Only rarely do gliomas destroy the surrounding bone by invasion of the extracellular matrix, especially without prior surgery. This paper describes the unusual case of a 66-year-old female patient with a left-sided intra- and extracranial tumor involving the temporal lobe, destroying the underlying skull base, and growing into the paranasal sinuses, orbit, and temporal bone. Biopsy revealed glioblastoma multiforme with strong GFAP positivity. Molecular biologic investigations of the p53, EGFR, and mdm2 genes showed functional inactivation of the p53 gene but no overexpression of oncogenes. Because the tumor was considered inoperable, palliative irradiation was carried out. The patient died 7 months after diagnosis. The causes of this phenomenon are discussed and the literature reviewed.

Aged↗

[Recostructive-restorational operations in patients with locally spread extracerebral tumors].

Issues of the restoration treatment optimization of patients with locally spread extracerebral tumors (LSET) were discerned basing on analysis of 54 observations. The role of reconsructive-restoration operations, depending on the tumor localization, size and invasiveness was shown, as well as the role of radiation therapy in complex restorational treatment. Rational complex approach to conduction of rehabilitation measures constitute the factor, promoting the treatment efficacy raising and providing for satisfactory quality of life in patients with LSET.

Adult↗