Skull Base Meningioma: Delayed Extracranial Presentation

Meningiomas are common intracranial tumors that rarely extend to extracranial sites. When they occur extracranially near the skull base, their diagnosis and management represent tremendous challenges. Although the extracranial component tends to manifest at the same time as the intracranial component, this is not always the case. In the patient reported in this article, 13 years separated the diagnosis of the intracranial tumor from the appearance of an extracranial component. The clinical presentation, radiologic findings, and management of this large skull base meningioma are discussed with reference to the literature. A high index of suspicion and clinical acumen are needed to ensure that the extracranial component of these frequent tumors is discovered, especially when it presents later than the intracranial component.     

Jugular foramen schwannomas

Thirteen patients with schwannomas of the jugular foramen were operated on at the Cleveland Clinic between 1974 and 1983. The authors' experience in managing these rare tumors is presented. Three major growth patterns of jugular foramen schwannoma were seen, and it is postulated that the position of the tumor depends on its point of origin from the nerves as they pass through the pars nervosa of the jugular foramen. The more distal lesions will expand inferiorly out of the base of the skull, and the more proximal lesions will enlarge into the posterior fossa. Tumors in the mid region will tend to expand primarily into bone. The schwannoma was primarily intracranial in six patients. In five patients the tumor expanded the bone at the base of the skull, with only a small intracranial component, and in two patients the tumor was primarily extracranial, with a small extension into the bone or posterior fossa. The presentation of the patients varied according to the tumor growth pattern. Deafness, vertigo, and ataxia were present in all patients with a major intracranial component, and in most of these there were only minimal deficits of the jugular foramen nerves. By contrast, lower cranial nerve involvement, including hoarseness and weakness of the trapezius and sternocleidomastoid muscles, occurred in patients in whom the tumor was primarily, within the bone or extracranial. Three of the five patients with the major component of the schwannoma within the bone also had deafness. Symptomatic history was longest in those with tumor mainly involving the bone at the base of the skull, and shortest in patients with entirely extracranial tumor. Surgical resection was accomplished with a joint neurosurgical-otological approach, usually combining a posterior fossa exploration with either a translabyrinthine transcochlear or infralabyrinthine procedure. The exact nature of the operation depended upon the presence of intracranial tumor and on the extent of bone or extracranial involvement. Total excision was performed in all cases. There was no operative mortality, and surgery resulted in loss of function of the ninth, 10th, and 11th cranial nerves in most patients. The major postoperative morbidity consisted of swallowing difficulties and sputum aspiration.     

Cranial and intracranial aspergillosis of sino-nasal origin

Summary This paper is an attempt at defining the most efficacious surgical and antifungal therapy for invasive cranial and intracranial aspergillosis, and is based on experience with nine non-immuno-compromised patients treated and followed-up by the authors between 1983 and 1994; as well as on the summary of previously reported cases and advances in therapy of this condition. Depending on the degree of aspergillar involvement of the cranial base and intracranial structures, a classification, with implications for treatment and prognosis, is also proposed.Two patients had extracranial skull base erosion; whereas relentlessly progressive granulomas, mimicking malignancy, invaded the skull base and intracranial contents in seven cases. Of these seven patients with cranial and intracranial invasion, two died of acute intracranial haemorrhage due to fungal invasion of cerebral blood vessels. In two patients, complete surgical eradication of the disease proved impossible due to cavernous sinus involvement, while residual aspergillomas are still present in orbit and paranasal sinuses (PNS) in a further two patients in spite of multiple surgical procedures and prolonged antifungal chemotherapy (AFC). What appears to be a cure has been effected in one patient only. Multiple therapeutic strategies were used. Biopsy plus systemic AFC was ineffective, surgical drainage and debridement plus systemic AFC resulted in long-term survivals but no cure. Radical surgery in conjunction with systemic and local (intracavitary) AFC should be considered to improve an otherwise poor prognosis.     

Combined use of a radial fore arm free flap for extra-intracranial bypass and for antero-lateral skull base reconstruction – a new technique and review of literature

Summary This article describes a new surgical technique consisting of the combined use of a fascial radial fore arm free flap (RFFF) as vascular graft for extra-intracranial bypass and as dura mater plasty for reconstruction of the antero-lateral skull base. This new technique is illustrated by a case of a complex intracranial meningioma with extracranial extension necessitating resection of internal carotid artery. The technical issues of antero-lateral skull base reconstruction and extra-intracranial bypass are discussed and the literature is reviewed.     

Extending the traditional resection limits of squamous cell carcinoma of the anterior skull base

OBJECTIVES: Preliminary report to evaluate the efficacy of resection of squamous cell carcinomas that demonstrate intracranial invasion. METHODS: A retrospective review of all cases of extracranial squamous cell carcinomas that extend intracranially treated by a single surgeon. RESULTS: A total of 21 cases were reviewed. In 6 cases, there was noted to be overt brain invasion. Complete resection of the intracranial disease was achieved in each of the remaining 15 cases. There were no instances of CSF leak, meningitis, brain abscess, stroke, or other intracranial complication noted either acutely or secondarily. In follow-ups that ranged from 10 months (single patient died of disease at 10 months) to 6 years (average, 3.8 years), there were no instances of intracranial recurrence. There was a disease-free control rate of 67.7% at an average follow-up of 4.1 years. CONCLUSIONS: Extending the resection of squamous cell carcinoma into the intracranial vault judiciously as outlined appears to be associated with acceptable outcomes in the treatment of advanced squamous cell carcinoma of the skull base.     

Lesions of Rathke's duct: another indication for the infratemporal fossa approach

Rathke's duct is a midline structure, arising from the primitive stomodeum and extending upward to the hypophysis. The duct, which normally involutes in utero, may persist on occasion. This most frequently results in intracranial disease. Occasionally, disease may become manifest in the extracranial skull base. We present three such cases: cystic craniopharyngioma, Rathke's cleft cyst, and a persistent cerebrospinal fluid rhinorrhea caused by a patent craniopharyngeal canal. Each lesion involved the clivus and the parasphenoid region. Surgical management of such lesions requires not only extirpation of disease, but also the ability to recognize and preserve the many important regional structures. The procedure that best allows for the wide exposure necessary to accomplish these goals is the infratemporal fossa approach to the lateral skull base.     

Intracranial juvenile angiofibroma with intradural and cavernous sinus involvement

Juvenile angiofibroma is a benign tumor, well known for aggressive extracranial growth, that frequently spreads to involve the skull base and intracranial compartment as well. The meninges, however, provide a strong barrier against intracerebral spread. This article reports an unusual case of angiofibroma with dural penetration, cavernous sinus involvement, and adherence to the temporal lobe of the brain. Controversy exists as to how the intracranial extensions are best managed. Our experience with this case, combined with review of the literature, suggests that these lesions can be removed surgically, eliminating in most cases the need for radiation therapy.     

Intracranial Juvenile Angiofibroma with Intradural and Cavernous Sinus Involvement

Juvenile angiofibroma is a benign tumor, well known for aggressive extracranial growth, that frequently spreads to involve the skull base and intracranial compartment as well. The meninges, however, provide a strong barrier against intracerebral spread. This article reports an unusual case of angiofibroma with dural penetration, cavernous sinus involvement, and adherence to the temporal lobe of the brain. Controversy exists as to how the intracranial extensions are best managed. Our experience with this case, combined with review of the literature, suggests that these lesions can be removed surgically, eliminating in most cases the need for radiation therapy.     

Aneurysm of the extracranial internal carotid artery caused by fibromuscular dysplasia

A case of aneurysm of the extracranial internal carotid artery (ICA) caused by fibromuscular dysplasia (FMD) is reported. The patient also had an aneurysm of the contralateral intracranial ICA, but the renal arteries were normal. A review of the literature shows extracranial ICA aneurysms to be uncommon, with only 3% caused by FMD. The surgical management of such difficult lesions at the base of the skull is discussed.     

Complex cranial base trauma resulting from recreational fireworks injury: case reports and review of the literature.

Two patients who sustained complex skull base trauma secondary to recreational fireworks injuries are reported. Initial assessment and management included axial and coronal computerized tomography, control of hemorrhage, debridement of wound and brain, isolation of brain from external environment, and reconstruction of the cranial base floor. Secondary orbital and facial reconstruction used available bone fragments and iliac bone graft in one patient and vascularized free tissue transfer in the other. In both patients, reconstruction of both the intracranial and extracranial compartments was successful with acceptable cosmetic result. Modification of multiple conventional approaches, along with a multispecialty surgical team, was used to deal effectively with these unique cases.     

Frontal skull base surgery combined with endonasal endoscopic sinus surgery

BACKGROUND: Postoperative infection remains a serious complication after radical resection of anterior skull base lesions because intracranial, nasal, and paranasal cavities are opened during surgery. To prevent complications from postoperative infection, we combined endonasal endoscopic sinus surgery (ESS) with the frontal transbasal approach in patients with skull base lesions. METHODS: Patients (n = 16) with anterior skull base lesions extending to the paranasal or nasal cavity underwent surgical resection via the frontal transbasal approach. After removal of the lesion via the transcranial approach, enlargement of the ostium or sinusotomy was performed bilaterally using our endonasal ESS procedure. The main purpose of ESS is the establishment of a wide drainage route to avoid dead space and postoperative infection. Furthermore, we confirmed the absence of residual lesion and leakage of cerebrospinal fluid (CSF), endoscopically. RESULTS: The frontal transbasal approach combined with endonasal ESS was performed in 16 patients with frontal skull base lesions. There were 8 malignant tumors, 6 benign tumors, and 2 mucoceles. Although 11 patients had preoperative active paranasal sinusitis, most frequently at the ethmoid sinus, none experienced postoperative infection. There was no complication related to ESS procedure. Furthermore, leakage of CSF and extracranial residual tumor were not found. CONCLUSION: Endonasal ESS after frontal skull base surgery is a highly useful technique for preventing postoperative infection, especially for the cases with large skull base tumors extending into other regions involving the paranasal sinuses or nasal cavity and with active paranasal sinusitis.     

Fatal hemorrhage from rupture of the intracranial internal carotid artery caused by aspergillus arteritis

To the best of our knowledge, this is the first reported case of combined intracranial and extracranial hemorrhage due to aspergillus cerebral arteritis. Knowledge of the imaging and the importance of early diagnosis and treatment are emphasized. A 78 year old man developed progressive right-sided visual impairment and diplopia. Magnetic resonance imaging demonstrated a mass lesion located in the right orbital apex, with extension to the cavernous sinus and the right middle cranial fossa. Cerebral angiography showed no aneurysmal dilatation. He was scheduled for transnasal biopsy. However, the patient died of massive epistaxis and intracranial hemorrhage. Postmortem examination revealed an aspergillus granuloma of the orbit and the skull base involving the intracranial and extracranial internal carotid artery. Aspergillus fumigatus was identified by culture. The characteristic feature of the fungal infection is a low-intensity signal on T2-weighted magnetic resonance images. This finding may be useful in diagnosing fungal infection.     

Skull base chondroma of extracranial origin

Methods. Two cases of chondroma at the skull base, most probably of extracranial origin, are presented: one at the apex of the petrous pyramid and the other in the infratemporal fossa. Results. In the second case, the surgical findings suggested that the tumor originated in the cartilagineous part of the eustachian tube. The world literature was reviewed and only three cases of extracranial chondromas at the skull base, and ten cases of chondromas of the nasopharynx have been described so far. Conclusions. The potential origins of chondromas at the skull base are discussed: Cartilagineous remnants at the basisphenoid and basiocciput or the cartilagineous part of the eustachian tube seem to be the most probable origin.     

Surgical technic for removal of solitary destructive neoplastic lesions of the cranium

A method is demonstrated that allows complete extirpation en masse of a single solitary, destructive lesion of the skull, including extracranial and intracranial local extensions. This procedure has been carried out in four cases, with excellent technical results.     

Experience with 24 cases of reconstructive anterior skull base surgery: classification and evaluation of postoperative facial appearance

This article details our experience with 24 cases of anterior skull base reconstruction after tumor resection. They were classified into four types according to the resected region. In 11 cases of type I resection, the orbital part of frontal bone and/or cribriform plate of ethmoid bone were resected. In two cases of type II resection, the orbital contents and partial orbital bone were resected with the addition of type I. In five cases of type III resection, the maxillary bone was resected with the addition of type II. In six cases of type IV resection, the zygomatic bone and/or facial skin were resected with the addition of type III. The tumor originating from intracranial region was 25% of this series and all of them belonged to type I. The tumor originating from extracranial region tumor was 75% and its resected region was more extensive. In type I and II resections, the cranial flap, radial forearm free flap, or a combination of the two was used for reconstruction. The rectus abdominis myocutaneous/muscle free flap was used for reconstruction of massive defects in type III and IV defects. Total incidence of postoperative complications was 16.7%. Donor site deformity of the cranial flap at the frontal and temporal region in types I and II resections and facial contour deformity in zygomatic region and defect of upper and/or lower palpebra in type IV resection were major problems with postoperative facial appearance. Although use of the rectus abdominis myocutaneous free flap combined with costal cartilages improved the midfacial contour, palpebral reconstruction remained an unsolved problem in reconstructive skull base surgery. The reconstructive goals in skull base surgery are not only to obtain safe and reliable skull base reconstruction but also to restore the facial appearance postoperatively.     

Benign extracranial nerve sheath tumors of the skull base: postoperative morbidity and management

The purposes of this retrospective case series study were to examine the outcome of the operative treatment of extracranial nerve sheath tumors (NSTs) of the skull base and to learn the optimal management. The study was conducted at a university teaching hospital and a regional referral center. A total of 19 cases of benign extracranial NSTs of the skull base who presented to the otolaryngology department over a period of 10 years were studied regarding the clinical, radiological, and pathological features and the operative and postoperative management. In the majority, these tumors originated from cranial nerves; postoperative complications were frequent and depended on the nerve of origin. Postoperative nerve deficit was apparent in 10 cases, and a second operation was necessary in 8 cases. The greatest postoperative morbidity was associated with the parapharyngeal NSTs (i.e., dysphagia in 30%, dysphonia in 30%, and Horner's syndrome in 20% of cases). The conclusion from this study is that high postoperative morbidity resulting from surgery on skull base NSTs demands an integrated approach between the otolaryngologist, plastic surgeon, neurosurgeon, speech therapist, physiotherapist, dietician, and occupational therapist and a clear strategy of long-term follow-up.     

Gliosarcoma with infratemporal fossa extension. Case report

Gliosarcomas are bimorphic intraaxial tumors. Involvement of the skull base is highly unexpected. The authors present the case of a temporal lobe gliosarcoma with significant infratemporal fossa extension. This 55-year-old man presented with a 1-month history of severe progressive headache. Neurological examination was unremarkable except for bilateral papilledema. Magnetic resonance imaging revealed a 6-cm right temporal mass with extension into the infratemporal fossa. The patient underwent a right frontotemporal craniotomy together with drilling of the sphenoid ridge and middle fossa floor. The tumor consisted of intraaxial, intracranial as well as extradural, and extracranial components with extension to the posterolateral wall of the sphenoid sinus. It had a relatively well-circumscribed dissection plane. Gross-total resection was achieved, and the middle fossa floor was reconstructed using a rotated temporalis muscle flap. The postoperative course was uneventful except for hypesthesia in the distribution of the maxillary division of the right trigeminal nerve. The histopathological diagnosis was consistent with gliosarcoma. Radiotherapy and chemotherapy consisting of temozolomide were administered subsequently, and the patient was recurrence free 12 months after his initial diagnosis. In the presence of a mass lesion with both intraaxial and extracranial involvement, gliosarcoma should be considered among the differential diagnoses. Aggressive resection should be attempted, including the use of skull base surgical techniques to ensure an optimal outcome. The effect of skull base involvement to the overall treatment and outcome of patients with gliosarcomas would be difficult to determine given the rare occurrence of these lesions in such locations.     

Intracranial epidermoid tumor after subcutaneous lipoma excision

A 66-year-old man presented with complaints of numbness for the past 5 years and progressive motor weakness of the right leg for the previous 2 months. Magnetic resonance imaging revealed large intra- and extracranial tumors in the frontoparietal region. Physical examination suggested the extracranial lesion was a subcutaneous lipoma, which had been partially resected 60 years before, connected to the intracranial lesion via a defect of the skull. Gross total removal of the tumors was performed. Histological examination showed the intracranial lesion was epidermoid tumor, and connected to the extracranial lipoma by a lipoma bridge. The symptoms improved remarkably following surgery. This case of intracranial epidermoid tumor associated with intra-extracranial lipoma indicates that implantation of skin tissue in childhood carries the risk of epidermoid tumor even after several decades. Neuroimaging screening is recommended to detect the development of any intracranial components.     

Subtotal petrosectomy in the treatment of cerebrospinal fluid fistulae of the lateral skull base

Cerebrospinal fluid (CSF) fistulae almost invariably lead to meningitis, even in the absence of other clinically obvious sequelae of the fistula such as a CSF fluid leak. The only effective means of reducing the risk of meningitis is surgical closure of the fistula. If surgery is to be recommended to patients with CSF fistulae even if they are currently asymptomatic, the morbidity of the procedure must be a principal determinant of the chosen technique. Recovery after the extracranial approach to a CSF fistula is much more rapid than after an intracranial procedure. The extracranial route is also free of the long-term risk of epilepsy which accompanies a craniotomy. The principal disadvantage of the lateral extracranial approach, failure of treatment, has been largely eliminated following studies into the obliteration of simple bony cavities using free adipose grafts. This paper describes our use of the extracranial approach to closure of CSF fistulae of the lateral skull base.     

Sinonasal Non-Hodgkin's Lymphoma with Skull Base Involvement

Non-Hodgkin's lymphoma (NHL) is a rare tumor of the skull base. As the incidence of primary central nervous system (CNS) lymphoma has increased, atypical presentations involving the skull or cranial base exclusively have been reported. In immunocompetent patients with no previous history or predisposing factors, the diagnosis of primary NHL of the skull base may be delayed. We present four cases of nasal and paranasal sinus NHL with both skull base and intracranial involvement in immunocompetent patients. Clinicopathologic correlation suggests that cranial base and intracranial involvement with NHL represents advanced-stage primary sinonasal disease. Surgical biopsy before definitive treatment is recommended. Radiation therapy provides local control; adjuvant chemotherapy after primary radiation therapy may be required for recurrent disease.