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[A case of Collet-Sicard syndrome caused by skull base metastasis of prostate carcinoma].

A case of Collet-Sicard Syndrome caused by skull base metastasis of prostate carcinoma is reported. A fifty-five years old man presenting multiple lymph node and bone metastases of prostate carcinoma was treated with LH-RH agonist and Flutamide, which induced transient decrease in serum PSA levels and size of lymph node metastases. After 8 months of the treatment, the patient started complaining headache, dysphagia and dysarthria. Brain CT and MRI demonstrated a soft tissue mass replacing left pyramidal bone and occipital bone around left jugular foramen. The tumor was diagnosed as skull base metastasis of the prostate carcinoma and was treated with 50Gy of radiation. The symptom improved after the radiation but died of the disease in 4 months. The autopsy revealed the skull base metastasis of the prostate carcinoma and the tumor was proved to be poorly differentiated adenocarcinoma, which was positively stained by anti-PSA antibody. The case showed cranial nerve palsy of IX to XII, which is usually called Collet-Sicard syndrome. This is the third case report of Collet-Sicard syndrome caused by the skull base metastasis of prostate carcinoma, and it is the first case in Japan.

Adenocarcinoma↗

LINAC radiosurgery in skull base meningiomas.

LINAC radiosurgery has provided increasing access and changed treatment strategies in patients with benign skull base meningiomas in recent years. From January 1996 to January 2003, 37 patients with skull base meningiomas were treated with LINAC radiosurgery. A combination of the University of Florida system and the X Knife System, developed by Radionics, was used in all patients. Eight patients were treated by microsurgical resection before radiosurgery, in two patients tumor recurrence was treated and in 29 patients LINAC radiosurgery was the initial treatment procedure. The median treatment volume was 5.9 mL (0.7 to 22 mL) and the median given dose was 14.6 Gy (12.8 to 18 Gy) prescribed to the 80 percent isodose line. The follow-up period is between 12 and 96 months, median 66 months. 12 patients (32.4 %) showed a significant reduction in tumor size, in 11 patients (29.7 %) there was significantly less contrast enhancement and in 13 patients (35.1 %) the skull base meningioma was without any change. In one patient (2.8 %) there was tumor growth 48 months after radiosurgery, so the tumor control rate was 97.2 %. Two patients (5.6 %) showed neurological worsening after radiosurgery, whereas the symptoms were transient in one patient. LINAC radiosurgery offers us an effective treatment modality and changes treatment strategies in skull base meningiomas towards a less aggressive surgical approach. By combining microsurgery and radiosurgery we can achieve high tumor control rates with an acceptable low morbidity and a high level of quality of life.

Adult↗

The surgical management of patients with glomus tumours of the skull base.

Total surgical excision is the only treatment modality that offers a cure for patients with glomus tumours of the skull base. The vascularity, inaccessibility and frequently extensive local spread, all contribute to the difficulties encountered in the management of patients with these complex lesions. Owing to the rarity of skull base glomus tumours, experience in their management can only be attained over long periods of time. We describe the surgical management of 20 patients with large glomus tumours of the skull base treated by an otoneurosurgical team over an 11-year period. Overall, 70% of patients had an excellent outcome, 10% a good outcome and 20% a poor outcome at a mean follow-up of 3.1 years. Poor outcomes were due to severe facial nerve palsies in two cases, and poorly accommodated palsies of the bulbar cranial nerves in a further two patients. The management of postoperative neurological deficits is discussed in detail. We conclude that in the majority of patients with skull base glomus tumours, complete surgical excision can be safely achieved.

Adult↗

Interventional neuroradiology of lesions of the skull base.

Preoperative embolization has been incorporated definitively into the multidisciplinary therapy strategy for lesions of the skull base. Clinical and anatomic knowledge of the pathology of the skull base, as well as experience with embolization, is the basis for therapeutic success. Clinical knowledge will lead to adequate patient selection, whereas profound anatomic knowledge and mastery of endovascular techniques will avoid predictable complications. Provided these conditions are taken into consideration, embolization of lesions of the skull base yields convincing therapeutic and technical results. Preoperative embolization of skull base tumors should not be associated with mortality or morbidity because most of these diseases are histologically benign and the interventional procedure is done as a preoperative step and not as a curative one. Major complications such as cerebral stroke, blindness, or cranial nerve palsies are results of the application of inappropriate techniques or of poor evaluation of angiographic findings, and should be avoided in the majority of cases. Minor complications are related to inadvertent occlusion of cutaneous, muscular, or osseous branches of the ECA. They are sometimes unavoidable and may include pain, skin necrosis, and trismus. Nevertheless, state-of-the-art external carotid embolization should--in skilled hands--have a permanent morbidity below 1% and no mortality.

Bone Diseases↗

Malignant tumors of the anterior skull base.

BACKGROUND: Cancers of the paranasal sinuses or nasal cavity are the most common malignant tumors of the anterior skull base. Several types of tumors occur in this location, including cancers of endodermal, mesodermal, and epidermal origins. Although anterior skull base surgery is a relatively recent approach in treating these tumors, widespread changes have already occurred in procedural methods and treatment goals. METHODS: We review the tumor types that occur in the anterior skull base and discuss the current treatment options, including multimodal therapy and the team approach to surgery. Surgical techniques are also described. RESULTS: Management of anterior skull base cancer is complex due to the anatomic detail of the region and the variety of cancers that occur in this area. Currently, the "gold standard" for surgery is the anterior craniofacial approach. Combined with adjuvant radiation therapy, 5-year disease-free survival rates have increased to 50%, with some tumors such as adenocarcinomas and esthesioneuroblastomas reaching up to 80% 5-year survival rates. Potential complications include cerebrospinal fluid leakage, meningitis, abscess formation, and pneumocephalus. CONCLUSIONS: Treatment of anterior skull base cancer is complex due to the significant anatomic detail of the region and the variety of cancers that occur in this area. Multimodal therapy through a team approach is the optimal management approach for these tumors.

Adenocarcinoma↗

Endoscopic management of skull base osteoradionecrosis.

OBJECTIVE: Osteoradionecrosis is one of the most serious complications in radiotherapy of nasopharyngeal carcinoma. We describe a new endoscopic approach to resolve resultant skull base osteoradionecrosis. The objective of this study is to evaluate the efficacy of endoscopic management of skull base osteoradionecrosis. STUDY DESIGN: A prospective study of the outcome of endoscopic management for patients with skull base osteoradionecrosis. METHODS: Between 1994 and 1998 six patients who had irradiation previously for nasopharyngeal carcinoma had skull base osteoradionecrosis. A sinoscopic approach was applied for diagnosis and sequestrectomy. This diagnosis was based on the criterion of exposed necrotic bone after removing all crust in the nasopharynx and further confirmed on pathological examination after sequestrectomy. Effective cure was defined as intact mucosal coverage without any ulcer or exposed necrotic bone observed in the nasopharynx and the absence of antecedent accompanying symptoms after management. RESULTS: Six patients (10%) were symptom free. Five (83.3%) patients had effective cure. There was no surgical morbidity or mortality. CONCLUSION: Endoscopic sequestrectomy is a justified approach to skull base osteoradionecrosis.

Adult↗

Management of the internal carotid artery in tumors of the lateral skull base: preoperative permanent balloon occlusion without reconstruction.

OBJECTIVE: To present our experience with permanent preoperative balloon occlusion of the internal carotid artery while dealing with different abnormalities of the lateral skull base and a comparison with the results mentioned in the literature. STUDY DESIGN: Retrospective case review. SETTING: Private neurotologic and skull base tertiary referral center. PATIENTS: Fifteen patients who underwent preoperative balloon occlusion of the internal carotid artery and surgery subsequently for various abnormalities of the lateral skull base between 1989 and 2002. INTERVENTIONS: Each patient was subjected to four-vessel angiography along with the manual cross-compression test and balloon test occlusion to assess the efficacy of the collateral circulation. After angiography, each patient underwent a preoperative balloon occlusion, after which a lateral skull base procedure was performed for removal of the abnormality. MAIN OUTCOME MEASURES: Only those patients showing evidence of adequate collateral cerebral circulation and a less than 1-second delay between the angiographic phases of the two cerebral hemispheres on angiography were considered fit for preoperative balloon occlusion. While under going the preoperative balloon occlusion, the patients were clinically assessed for the development of any neurologic symptoms and signs. Long-term follow-up after surgery was also based on the development of symptoms and signs of neurovascular compromise. RESULTS: A major complication in the form of long-lasting hemiplegia occurred in one patient (6.7%). This complication was the result of technical factors rather than an effect of cerebral ischemia, because it was caused by an intimal dissection produced by the catheter. A defect in the visual field occurred in one patient (6.7%) that resolved partially after antiplatelet therapy. There was no mortality in our series related to preoperative balloon occlusion of the internal carotid artery. CONCLUSION: Preoperative balloon occlusion of the internal carotid artery can still be considered a viable option for the management of the internal carotid artery during lateral skull base surgery. Proper preoperative evaluation of the adequacy and efficacy of the collateral cerebral circulation reduces the chances of postoperative neurovascular complications.

Adolescent↗

Comparison of surgical approaches to the skull base.

The surgical accessibility of many complex areas of the base of the skull has expanded rapidly in recent years, as has the literature on these procedures. Several routes to each anatomic region have been described, although little has been written or illustrated comparing and contrasting the different approaches to the skull base. Thirteen representative surgical approaches will be discussed, accompanied by diagrams of accessible anatomic regions. Key features of each procedure will be compared and contrasted. A uniform presentation will be used, showing the incision for each procedure as well as the accessible anatomic areas in three views. Modern imaging techniques permit CT and MR data to be viewed in similar projections, facilitating the choice of surgical procedures.

Craniotomy↗

Skull base growth in craniosynostosis.

Although considerable scientific work has been published on the role of the skull base in craniosynostosis, the changes with age throughout childhood have not been fully outlined. The realisation that little attention has been paid to the posterior skull in craniosynostosis, resulted in renewed interest in skull base growth. The availability of computer-based image analysis provides a new accurate method of study in three dimensions. Using three-dimensional visualisation techniques, 34 points of the skull base were identified on CT scans of 50 children with craniosynostosis of various types, aged from 1 month to 5 years. Several distances and angles between the various landmarks were measured in an attempt to quantify the growth of skull fossae with age. Comparisons were made with normal controls. In children with craniosynostosis, the anterior fossa was overdeveloped in the males, whereas in the females remained underdeveloped throughout the first 2 years of life. The body of the sphenoid showed moderate underdevelopment in the first 2 years in both sexes, the effect being more prominent in the males. The middle fossae showed overdevelopment in both sexes in the first 2 years of life. The posterior fossa was underdeveloped in both sexes in the first 2 years of life, the effect being more prominent in the females. Craniosynostosis seems to affect both sexes to a similar degree, but there are regional differences in the growth pattern. Better understanding of the normal growth pattern of the skull base and the effect of craniosynostosis upon it may assist our approach to surgical treatment and in particular the role of anterior and posterior skull expansive surgery.

Child, Preschool↗

[The results of gamma knife radiosurgery for malignant skull base tumors].

The results of gamma knife radiosurgery for malignant skull base tumors were analyzed using repeated magnetic resonance imagings and neurological examinations. Nineteen malignant skull base tumors were treated and followed up for 22.3 months (5-40 months) using MR imagings. The mean age was 54.4 years old (ranging from 16-85). Ten were male and 9 were female. Prior to the radiosurgery, removal of the tumors in 17 cases, conventional radiation therapy in 7, and chemotherapy in 4 etc. were performed. The pathological diagnoses were chordoma in 6 cases, metastatic tumors in 5, epipharyngeal carcinoma in 2, adenoid cystic carcinoma in 2, and others in 4. The locations of tumors were the clivus in 8, the parasellar region in 5, the epipharynx in 2, the paranasal sinus in 2, C-P angle in 1, and intraorbital region in 1 (14 were intracranial and 5 were extracranial). The mean diameter of the tumor was 33.5mm. The mean maximum dose was 26.8Gy and the mean marginal dose was 12.9Gy during treatment. Repeated MR imagings revealed decrease of tumor size in 12 cases, no change in 1, and increase of tumor size in 5 (unknown in 1). Follow-up neurological examinations showed improvement in 3 cases, no change in 9, and deterioration in 7. There were 11 deaths during a mean follow-up period of 17.8 months (5-32 months) and another 8 cases are alive for a mean follow-up of 30.5 months (20-40 months) after the radiosurgery. Although the tumor size was large at the time of treatment, the results of gamma knife radiosurgery were promising. Considering the quality of life of patients with malignant skull base tumors, it is emphasized that gamma knife treatment is the method of choice compared with radical removal of the tumors.

Adolescent↗

Osteomyelitis of the skull base.

Three cases of osteomyelitis of the skull base with associated problems in diagnosis and therapy are discussed. Patients with atypical skull base osteomyelitis are difficult to diagnose as they have no ear abnormalities, but they often develop multiple cranial nerve deficits mimicking symptoms of a posterior fossa mass. We conclude that computed tomographic scans, magnetic resonance imaging studies, bone scans indium-labeled white blood cell scans, and gallium scans are useful in making the diagnosis. A biopsy of the bony lesion often is needed to identify the causative organism and to rule out a tumor. Intravenously administered antibiotics are the mainstay of therapy and should be continued until 1 week after the gallium scan shows no abnormalities. Follow-up gallium scans then are done at 1 week and 3 months after the cessation of antibiotic therapy to search for a recurrence.

Adult↗

Gorham's disease of skull base and cervical spine--confusing picture in a two year old.

The unusual presentation of Gorham's disease of skull base and cervical spine in a two-year-old female child with radiological signs mimicking those of raised intracranial pressure is discussed. The differential diagnosis consists of skull base tumours, meningitis, osteomyelitis of the base of skull, congenital hydrocephalaus and congenital syndromes involving the skull base. Pathologically it can be very difficult to differentiate it from lymphangioma of the bone. Difficulty in establishing the diagnosis is discussed along with failure of radiotherapy and palmidronate therapy to cause arrest of the disease process and failure of surgery to provide stabilisation. We describe the course of the disease in this child over the period of last eight years. To the best of our knowledge this is the youngest case of Gorham's described so far.

Bone Density Conservation Agents↗

Craniofacial resection for malignant tumors of ethmoid and anterior skull base.

A combined craniofacial approach is described for resection of tumors that involve the ethmoid sinuses and adjacent base of the skull in the frontal fossa. This operation provides a more thorough en bloc excision than by conventional methods with reasonable safety since both the neurosurgeon and the head and neck surgeon are guided by each other during the mobilization of the specimen, and adequate repair of dural defects can be accomplished. Reconstruction of the bony defect in the skull base is performed with a pedicled periosteal flap from the scalp and a skin graft.

Adult↗

The zygomatico-temporal approach to the skull base: a critical review of 11 patients.

The zygomatico-temporal approach to the base of the skull is a relatively new but established surgical technique. The approach involves the removal of the zygomatic bone to provide access to the skull base, middle cranial fossa, parasellar region and interpeduncular cistern with minimal brain retraction. An excellent view of the bifurcation of the basilar artery and suprasellar region is provided. The outcome of 11 patients undergoing this procedure is reported with particular reference to the post-operative morbidity and the cosmetic result.

Adenoma↗

Prognostic factors and outcome in craniofacial surgery for malignant cutaneous tumors involving the anterior skull base.

BACKGROUND: We retrospectively reviewed the medical charts of 57 patients with advanced malignant cutaneous tumors involving the anterior skull base who underwent combined craniofacial resection in our department from January 1, 1981, to December 31, 1994. METHODS: The data regarding histological results, demographic aspects, clinical presentation, previous treatment, extent of the disease, extent of surgical procedure, type of reconstruction, complications, and follow-up were analyzed using the chi 2 method. Survival analysis was performed using the Kaplan-Meier method. RESULTS: We found prevalence of the male sex (60%) and white race (86%), with a mean age of 62 years. Thirty-five patients (61%) showed extensive lesions directly invading the anterior skull base (type II); 46 patients (81%) had been treated previously; 10 patients (18%) had dural invasion; 29 patients (51%) could not have the eyeball preserved; 32 patients (56%) underwent microsurgical reconstruction; and 45 patients (79%) underwent pericranial or galeal-pericranial flap reconstruction for the anterior skull base defect. Postoperative complications occurred in 29 patients (51%). The most common complication was postoperative infection (17 patients [59%]). Thirty-two patients (56%) were free of disease, and 4 (7%) were alive with disease at the time of our study. CONCLUSIONS: The extent of facial disease (P = .005) and the type of facial reconstruction (P = .01) were the most important risk factors in the development of infectious complications. Invasion of the dura and the type of reconstruction of the anterior skull base were the most important factors related to cerebrospinal fluid leakage (P = .003) and meningoencephalitis (P = .06). Invasion of the dura mater significantly affected survival (P = .005).

Adult↗

Plasma cell myeloma of the skull base: report of two cases.

Plasma cell myeloma (PCM) of the skull base is rarely encountered in neurosurgical practice. PCM has a wide spectrum of pathology, including a quite benign, solitary plasmacytoma (SPC), and an extremely malignant, multiple myeloma (MM) at the two ends of the spectrum. We have described two patients with PCM of the skull base, of which one harbored SPC, and the other progressed to MM. In case 1, a 46-year-old man presented with left multiple cranial nerve impairments and had a large skull base tumor. Subtotal removal was performed. The specimen and general examination, including bone marrow aspiration, revealed SPC. Postoperatively 50Gy of external radiotherapy was administered. The patient has no manifestation of MM 24 months after the initial presentation. In case 2, a 53-year-old woman presented with left abducens palsy and had a left petroclival osteolytic mass. Gross total resection was performed. The specimen revealed a plasmablastic tumor, i.e., myeloma. General examination established the diagnosis of MM. She was administrated adjuvant chemotherapy and autologous bone marrow transplantation. She is alive without local recurrence 30 months after the initial presentation.

Diagnosis, Differential↗

[CT and MRI and clinical study of chordoma in the skull base].

OBJECTIVE: To investigate the diagnostic features of CT and MRI in chordoma of the skull base (CSB) and to estimate their value of clinical application. METHODS: Sixteen patients with CSB were performed CT and MRI examinations of the head, and 13 patients with nasopharyngeal carcinoma (NPC) and 11 patients with pituitary adenoma (PA) which invaded the skull base were selected as control. In addition, 7 cases of chordoma were performed multiple planar reconstruction (MPR) of MRI. RESULTS: MRI was superior to CT in depicting the location and the extent of CSB. The bone destruction at mid-line skull base was demonstrated equally well on both CT and MRI. Based on the location of the tumor displayed on MRI, the CSB could be classified into selloclival, nasopharyngeal, and occipito-temperal types. The T2 weight imaging(T2WI) signal intensity of chordoma was significantly higher than those of NPC and PA (P < 0.01). On MPR images of MRI, oppressed and displaced optic nerves caused by chordoma were noted in 3 cases and encased optic nerves in 4 cases, encased abducent nerves in 7 cases with abducent paralysis and mildly oppressed oculomotor nerves in 3 cases. CONCLUSION: The extremely high T2WI signal intensity is the characteristic of MRI finding in CSB and has great differential value for the tumors of the skull base. The MPR of MRI plays an important role in the surgical treatment for these tumors.

Adult↗

Petroclival meningioma. An attempt to define the role of skull base approaches in their surgical management.

BACKGROUND: Petroclival meningiomas represent a difficult surgical challenge. The introduction of modern techniques of skull base surgery has stimulated the interest of the neurosurgical community in the surgical management of these lesions, although very good results have been obtained by experienced neurosurgeons in cases of petroclival meningioma operated using traditional surgical approaches. METHODS: Thirty-one cases of petroclival meningioma have been operated on during a 4-year period using two different philosophies as far as the approach. Group A patients (13 cases) have been operated on using mostly either a subtemporal transtentorial or a retrosigmoid approach. Group B patients (18 cases) have been managed using a lateral skull base approach, either the anterior transpetrosal or the presigmoid approach. Translabyrinthine/transcochlear approaches have been used occasionally. RESULTS: Tumor dissection and removal seemed to be easier in skull-base operated patients. However, operations take longer and surgical complications such as cerebrospinal fluid leak and hearing loss were increased. Radical tumor removal could be achieved in an equal percentage of cases of both groups (77% vs. 83%). The early postoperative course was more favorable in group B patients. However, late permanent morbidity appeared to be similar in the two groups of patients. CONCLUSIONS: Skull base approaches facilitate tumor dissection and removal at the expense of increased surgical complications. However, the widened surgical field and increased angle of approach that the techniques of skull base surgery may offer can play a significant role in the removal of sizeable, infiltrative, and/or recurrent petroclival meningiomas.

Adult↗