Aggressive fibromatosis (fibrosarcoma) of the facial nerve

Aggressive fibromatosis of the facial nerve is a very rare tumor. Three cases have been previously reported. The tumor is locally recurrent and often has a fatal outcome. This report is of a ten-year-old boy whose tumor originally developed in the parotid area with subsequent spread to the base of the skull, the neck and the cerebellopontine angle. Treatment was by wide surgical excision, radiation therapy and chemotherapy. Despite treatment, the patient died. The clinical features of this case will be described. Only wide surgical excision early in the course of the disease may offer a chance for cure.     

Incorporation of nuclear matrix attachment regions into the herpes simplex virus type 1 genome does not induce long-term expression of a foreign gene during latency

We report two cases of skull base metastasis from renal cell carcinoma. Case 1: A 55-year-old female presented with a skull base tumor located on the clivus. Partial removal of the tumor was performed via the transsphenoidal approach. Case 2: A 44-year-old male presented with a skull base tumor occupying the orbital, nasal, and paranasal cavities. The first operation, partial removal of the tumor, was performed via the transsphenoidal approach. Thereafter, the tumor regrowth and the patient's symptoms were progressive, so a second operation was performed via Le-Forte I craniotomy to prevent cranial nerve dysfunction and air way obstruction. The initial symptom of these two cases was abducens nerve paresis, which is caused by the tumor extending from the clivus to the cavernous sinus. The neuroradiological features were the destruction of skull base bone and angiographic tumor stain. Histopathological examination of the surgically resected specimens revealed clear cell carcinoma, and thereafter they were diagnosed as skull base metastasis from renal cell carcinoma. We emphasize the necessity of a thorough medical workup to enable speedy diagnosis of renal cell carcinoma in such cases.     

Frontometaphyseal dysplasia: evidence for autosomal dominant inheritance

Frontometaphyseal dysplasia is a syndrome that encompasses cranial hyperostosis, abnormal tubulation of cylindrical bones, and other skeletal and extraskeletal abnormalities. The most striking features are overgrowth of the supraorbital ridges which results in a Mephistophelian facial appearance and a radiographic configuration of the skull that has been likened to a soldier's helmet. Most patients have severe hearing loss, defective dentition, poorly developed musculature, and joint contractures. Dominant inheritance has been suggested in previous reports, but an appropriate pedigree has been documented in only one family. This paper describes three additional patients in two unrelated families: (1) an 8-year-old boy whose mother has mild metaphyseal dysplasia and several minor skeletal abnormalities that have occurred in patients with the syndrome; and (2) two maternal half-brothers. These cases provide additional evidence that frontometaphyseal dysplasia is an autosomal dominant trait with variable penetrance.     

Mondini's dysplasia: a rare cause of recurrent meningitis in childhood

Recurrent meningitis in children may be due to immune deficiency or abnormal communication of CSF spaces with mucosal spaces (basal skull fractures, encephalocele, etc.). We report a 6-year-old boy, suffering from recurrent meningitis and sensorineural hearing loss. CT scans of the temporal bones demonstrated a bilateral Mondini dysplasia. In patients with this congenital inner ear anomaly, recurrent meningitis may be due to a CSF-middle ear fistula. We think that it is necessary to investigate all patients with recurrent meningitis and sensorineural hearing loss with CT scans of the temporal bones.     

Cholinesterase activity in human pulmonary arteries and veins

We present two cases of unusually large skull base paragangliomas. The first tumor was accompanied by marked bony destruction of the central skull base and multiple associated cysts. The second tumor arose along the petrous ridge, with a large intracranial component. The CT, MR imaging, angiographic, histologic, and electron microscopic findings of these unusual lesions are described.     

Non-Hodgkin lymphoma of the central skull base: MR manifestations

OBJECTIVE: The aim of this study was to demonstrate the MR characteristics of non-Hodgkin lymphoma of the skull base to help in the differential diagnosis of this neoplasm from other conditions. MATERIALS AND METHODS: MR of five patients, 7-64 years old, with pathologically proved lymphomas of the skull base were reviewed. Three cases had primary skull base lesions involving the sphenoid bone and the cavernous sinus. One case with a nasal cavity lesion involving the skull base and one with a relapsing skull base lesion of previously treated tonsillar lymphoma were included. RESULTS: The lesions had signal intensities that were similar to that of gray matter of brain on both T1- and T2-weighted imaging. Bilateral cavernous sinuses were involved with encasement of internal carotid arteries in every case. Postcontrast MR showed homogeneous enhancement of the tumor with dural infiltration along the planum sphenoidale, clivus, or tentorium. The clivus was destroyed or replaced by tumors in adult cases but in two children the clivus was preserved with intact sphenooccipital synchondrosis. In one case the tumor extended to the extracranial portion through the jugular foramen. CONCLUSION: The MR findings of a permeative lesion of the skull base, invasion of the cavernous sinus without arterial narrowing, infiltration along the dural surface, and an iso- or hypointensity with brain on T2-weighted imaging should suggest lymphoma.     

Striated osteopathy

Striated osteopathy, a roentgenological entity characterized by longitudinal striations, can occur concomitantly with a number of disorders which should be routinely looked for. There are three types of striated osteopathy. The pure form without severe bone disorders is the form originally reported by Voorhoeve. In the second form, there is a concomitant fibrous dysplasia, such as Jaffe-Lichtenstein-Uehlinger disease, Ollier enchondromatosis, sponastrism dysplasia, or osteopetrosis. This group also includes bone dysplasias with sclerosis such as melorheostosis, osteopecilia, and sclerosis of the base of the skull which is sometimes seen in patients with striated osteopathy. In the third form, striated osteopathy occurs concomitantly with a skin disease such as patchy dermal hypoplasia or hyperpigmented skin lesions.     

Radiosurgical management of meningiomas

The management of residual, recurrent, or small skull base meningiomas is controversial. Stereotactic radiosurgery has emerged as an alternative treatment. We report our experience from September 1991 to August 1994 of treatment of 20 such patients [18 females -age 19-82 years, followed for 6-36 months (mean 15.5 months)] with the Leksell Gamma Knife. Nine patients were treated either with recurrent (2 patients-2 operations each) or residual tumor. Twelve patients had skull base, 3 optic nerve, 3 parasagittal, and 1 residual torcular tumor. Mean volume/diameter was 9.172 mm3/25 mm.     

Avid uptake of technetium-99m-HMPAO by an intracranial plasmacytoma during carotid balloon test occlusion

A 56-yr-old woman was evaluated for removal of a tumor at the base of the skull. A test to determine the risk of carotid artery sacrifice was performed prior to surgery using carotid balloon occlusion of the left internal carotid artery and 99mTc-HMPAO perfusion scintigraphy during the occlusion. An unusual intense focus of increased uptake was seen at the site of the primary tumor in the left cavernous sinus. The tumor, found to be plasmacytoma at surgery, demonstrated only mild washout from 30 min to 2 hr after administration of 99mTc-HMPAO, with a tumor-to-cerebellum ratio of 1.6 and 1.5, respectively, and a tumor-to-contralateral cranial ratio of 2.5 and 2.4, respectively. Intracranial plasmacytoma shows good response to radiation therapy, and the differentiation of this tumor from other neoplasms is pertinent to the mode of treatment and surgical approach. Technetium-99m-HMPAO SPECT imaging may be a useful tool in distinguishing these tumors from other neoplasms at the base of the skull.     

Chondroma of the petrous bone. A contribution to differential skull base tumor diagnosis

INTRODUCTION: Cartilaginous tumors of the mid-face and the skull base are rare. CASE REPORT: For the first time, a case report of a chondroma of the base of the ear in a 56-year-old woman is presented. In 1974 the patient developed a facial nerve paralysis while she was pregnant. Twenty-two years later the patient developed persistent headache and CT studies of the head were obtained, which showed an extensive tumorous lesion located at the base of the ear. A tumor was resected through an otoneurosurgical approach. The histological examination showed a chondroma. CONCLUSIONS: Even the rare diagnosis of a chondroma should be considered for a differential diagnosis of skull base tumors.     

Life-size, computer-generated skull replica to assist surgery of craniofacial fibrous dysplasia

Craniofacial fibrous dysplasia is a benign pathological condition but it replaces bone and expands gradually, leading to facial distortion. Surgery is the only effective treatment. The authors describe the usefulness of a life-size, computer-generated skull replica which assists surgeons pre- and intraoperatively. Surgery can be safely performed with less invasion and a shorter operation time by referring to the skull replica. Partial bone resection and bone contouring have been carried out, assisted by skull replicas, in 8 patients with craniofacial fibrous dysplasia, resulting in good cosmesis.     

Vagal paraganglioma: a review of 46 patients treated during a 20-year period

BACKGROUND: Vagal paragangliomas (VPs) arise from paraganglia associated with the vagus nerve. Approximately 200 cases have been reported in the medical literature. Because of their rarity, most information regarding these tumors has arisen from case reports and small clinical series. OBJECTIVE: To detail the clinicopathologic features of 46 patients with VP with an emphasis on the role of a multidisciplinary skull base team in both the successful extirpation and rehabilitation. DESIGN: Retrospective review of 46 patients with VP managed by a single skull base team. SETTING: An academic tertiary medical center. RESULTS: Forty-six patients were treated over a 20-year period (1978-1998). Ten (22%) demonstrated intracranial extension. There was a history of familial paragangliomas in 9 (20%) of the patients. The incidence of multicentric paragangliomas was 78% in patients with familial paragangliomas vs 23% in patients with nonfamilial paragangliomas. Management of this group of 46 patients consisted of surgery (n = 40), radiation therapy (n = 4), and observation (n = 2). The operative approach consisted of a transcervical excision often combined with a transtemporal or lateral skull base approach as dictated by the tumor extent. Postoperative cranial nerve deficits were common, and, as such, aggressive rehabilitation was a vital component in the management of these tumors. CONCLUSIONS: The management of VP and its associated cranial nerve deficits remains a difficult clinical problem. Options for treatment include surgical resection, radiation therapy, and, in selected cases, observation. Surgical extirpation requires a multidisciplinary skull base team to achieve complete tumor resection. Radiation therapy is reserved for elderly patients and patients at risk for bilateral cranial nerve deficits. Rehabilitation of cranial nerve deficits is an integral part of the management of VP.     

Osteoclastoma of temporal and zygomatic bone

A twelve year boy was admitted to the Otolaryngological Clinic of the Medical Academy in Cracow with a paralysis of the peripheral facial nerve and a tumor on the right zygomatic arc. A CT investigation revealed an expansive process in the central fossa of the skull, which continuously passed into the squama of the temporal bone and the are of the zygomatic bone on the right side, causing a paralysis of the nerve VII. A neurosurgical intervention was carried out as well as a subsequent treatment by radiotherapy. A remission of the tumor was achieved. Osteoclastoma was diagnosed histologically.     

Cisplatin-based neoadjuvant chemotherapy and combined resection for ethmoid sinus adenocarcinoma reaching and/or invading the skull base

OBJECTIVE: To review our experience with cisplatin-based neoadjuvant chemotherapy before en bloc resection via a combined neurosurgical and transfacial approach for ethmoid sinus adenocarcinoma reaching and/or invading the skull base. DESIGN: Case series. SETTING: A tertiary care center and university teaching hospital. PATIENTS: Twenty-two patients with primary untreated ethmoid sinus adenocarcinoma reaching and/or invading the skull base consecutively treated between 1984 and 1992 with cisplatin-based neoadjuvant chemotherapy and combined neurosurgical and transfacial approach. MAIN OUTCOME MEASURES: Statistical analysis of survival, local control, nodal recurrence, distant metastasis, and metachronous second primary tumor incidence based on the Kaplan-Meier actuarial method. Univariate analysis was performed to analyze the relationships between various factors, survival, and local recurrence. Clinical response, histological response, toxic effects of chemotherapy, and postoperative course were also reported. RESULTS: The Kaplan-Meier 3-year survival, local control, nodal recurrence, and distant metastasis estimates were 68.1%, 65.7%, 5.3%, and 10%, respectively. Metachronous second primary tumor was not encountered in our series. Survival was statistically more likely to be reduced in patients with intrasphenoidal tumor extent (P = .04) and local recurrence (P = .01). Local recurrence was statistically more likely in patients with intrasphenoidal tumor extent (P = .002) and no response to cisplatin-based neoadjuvant chemotherapy (P = .03). CONCLUSIONS: The results achieved suggest that cisplatin-based neoadjuvant chemotherapy before combined neurosurgical and transfacial approach should be further investigated for the treatment of ethmoid sinus adenocarcinoma reaching and/or invading the skull base.     

Experiences with hemangiopericytoma in cranial base surgery

BACKGROUND: Haemangiopericytomas are rare vascular neoplasms which show either slow local tumour growth or aggressive progression in size with a high tendency of recurrence and metastasis. Manifestations of haemangiopericytomas in the area of the nose and sinuses are supposed to have a relatively benign course. To date clinical and histological parameters for prognosis are uncertain. Therefore in a quite large number of cases only the clinical course allows to evaluate the dignity of a haemangiopericytoma. PATIENTS AND RESULTS: We present our experience with five haemanglopericytomas in contact with the skull base out of a total number of 457 skull base tumours. The site of origin of these five haemangiopericytomas were: nasal septum, pterygopalatine fossa, oronasopharynx, temporal bone, and parotid gland. All neoplasms showed primary or recurrent tumor in contact with the skull base and an intracranial extension was observed twice. One patient died postoperatively of a cerebral infarction. Three patients showed one or more recurrences which were treated surgically. In one case 5 recurrences occurred. Despite of 4 surgical procedures within a period of 15 months, the tumour could be controlled only for a short period of time, and the patient died 16 months after the first operation. CONCLUSION: One has to consider that haemangiopericytomas of the head and neck show a potential malignant course. The vascular tumor may recur decades later. Adequate therapy and life-long follow-up are therefore mandatory in haemangiopericytomas. Complete surgical resection of the tumour is usually the treatment of choice, in spite of the high degree of surgical skill required, especially in advanced tumour and in case of recurrence.     

Hemifacial dismasking flap for anterior skull base tumor--technical note

A new approach to malignant tumor in the anterior skull base using a hemifacial dismasking flap is described. A bicoronal incision is extended unilaterally down to the neck, degloving the craniofacial tissue to widely expose the skeleton underneath, allowing easy resection of an extensive tumor without postoperative scarring of the face. This method has been used successfully on five patients.     

The role of stereotactic radiosurgery in the treatment of malignant skull base tumors

PURPOSE: To determine the efficacy and toxicity of stereotactic radiosurgery in the treatment of malignant skull base tumors. METHODS AND MATERIALS: Thirty-two patients with 35 newly diagnosed or recurrent malignant skull base tumors < or = 33.5 cm3 were treated using the Leksell Gamma unit. Tumor histologies included: adenoid cystic carcinoma [12], basal cell carcinoma [1], chondrosarcoma [1], chordoma [8], nasopharyngeal carcinoma [3], osteogenic sarcoma [2], and squamous cell carcinoma [8]. RESULTS: After a median follow-up of 2.3 years, 83% +/- 15% (+/-95% confidence interval) of patients experienced a symptomatic response to treatment. Local control at the skull base was 95 +/- 9% at 2 years and 78 +/- 23% at 3 years. Local-regional control above the clavicles was 75 +/- 15% at 1 year and 51 +/- 20% at 2 years. Overall and cause specific survival were identical, 82 +/- 13% at 1 year, 76 +/- 14% at 2 years, and 72 +/- 16% at 3 years. One patient developed a radiation-induced optic neuropathy 12 months after radiosurgery. CONCLUSION: Stereotactic radiosurgery using the Leksell Gamma Unit can provide durable tumor control and symptomatic relief with acceptable toxicity in the majority of patients with malignant tumors 4 cm or less in size involving the skull base. Further evaluation of more patients with longer follow-up is warranted.     

Effects of HMG-CoA reductase inhibitors on excitation-contraction coupling of rat skeletal muscle

We report on two unrelated children, a girl and a boy, with regressive metaphyseal dysplasia. Both children had bow legs and a transient growth decline in early childhood. Metaphyseal modifications of the long bones in the children were most conspicuous at an early age and then subsided by age 2 to 3 years. The father of the boy may have had the same disorder, because he was shorter than his sibs and showed mild modifications of the vertebral end plates with mild narrowing of the interpediculate distance of the lumbar spine. The evolution of the metaphyseal dysplasia in the children closely resembled that of metaphyseal anadysplasia (MAD), which is X-linked recessive in inheritance. By contrast, the occurrence of an isolated, affected girl and possible father-to-son transmission reported here were consistent with autosomal dominant transmission, suggesting heterogeneity of MAD. Molecular studies of the type X collagen gene in the boy did not demonstrate any disease-causing mutation.     

High blood pressure in pregnancy: effects of Ca2+ and Mg2+

Mucoepidermoid carcinoma of trachea and bronchi is a rare tumor, especially in children. The authors report a case of 9-year-old boy with mucoepidermoid carcinoma at the carina. His presenting symptoms were hemoptysis of recent onset and intermittent cough of 2 years' duration. Preoperative assessment of the tumor was an intraluminal polypoid mass arising from the carina extending into the trachea and right main stem bronchus. A complete resection with reconstruction of carina was successful. The tumor was 12 mm in size, polypoid with a broad base. It had characteristic features of a low-grade mucoepidermoid tumor, namely, admixture of islands of intermediate cells and glandular components with invasion of submucosa. The patient is now 15 months postsurgery free of disease.     

The system of the modified transcochlear approach: a lateral avenue to the central skull base

OBJECTIVE: This study aimed to update the authors' experience with the modified transcochlear approach for the management of lesions of the central skull base. The surgical technique, classification, indications, and results also are presented. STUDY DESIGN: A retrospective review of the charts of 66 consecutive patients treated in our centers by the modified transcochlear approach was conducted. SETTING: The study was performed in two tertiary referral centers. PATIENTS: All patients treated by the modified transcochlear approach were included. Thirty-five patients had extradural lesions, whereas 31 lesions were intradural. INTERVENTION: All patients were treated surgically using the modified transcochlear approach either in its basic form (type A) or with its extensions (types B, C, and D). MAIN OUTCOME MEASURES: The outcome of surgery is evaluated with particular emphasis on the incidence of morbidity, mortality, and the degree of total tumor removal. RESULTS: Total tumor removal was accomplished in 58 cases either in single or staged procedures. A second-stage procedure for total tumor removal is planned in five other patients. Subtotal tumor removal was performed in three patients. Mortality occurred in two cases. Ipsilateral hearing loss and immediate facial nerve palsy constituted the major drawbacks of this approach. However, 67.5% recovered to grade III facial function or better 1 year after surgery. CONCLUSIONS: The modified transcochlear approach provides a relatively safe, wide, and versatile access to large lesions of the central skull base.