Imaging of carotid canal sympathetic plexus schwannoma.

One case report of a schwannoma involving the sympathetic plexus in the carotid canal has been reported previously. This article presents 2 additional cases of this rare entity with associated clinical and unique radiographic findings. All patients presented with diplopia among other symptoms, and they had enhancing masses that smoothly expanded the carotid canal on cross-sectional imaging. Comparison with the index case and differential diagnosis of more common pathologies in this area are discussed.

FACT-MNG: tumor site specific web-based outcome instrument for meningioma patients.

To formulate Functional Assessment of Cancer Therapy-Meningioma (FACT-MNG), a web-based tumor site-specific outcome instrument for assessing intracranial meningioma patients following surgical resection or stereotactic radiosurgery. We surveyed the relevant literature available on intracranial meningioma surgery and subsequent outcomes (38 papers), making note of which, if any, QOL/outcome instruments were utilized. None of the surgveyed papers included QOL assessment specific to tumor site. We subsequently developed questions that were relevant to the signs and symptoms that characterize each of 11 intracranial meningioma sites, and incorporated them into a modified combination of the Functional Assessment of Cancer Therapy-Brain (FACT-BR) and SF36 outcome instruments, thereby creating a new tumor site-specific outcome instrument, FACT-MNG. With outcomes analysis of surgical and radiosurgical treatments becoming more important, measures of the adequacy and success of treatment are needed. FACT-MNG represents a first effort to formalize such an instrument for meningioma patients. Questions specific to tumor site will allow surgeons to better assess specific quality of life issues not addressed in the past by more general questionnaires.

Surgical resection of intrinsic insular tumors: complication avoidance.

OBJECT: Surgical resection of tumors located in the insular region is challenging for neurosurgeons, and few have published their surgical results. The authors report their experience with intrinsic tumors of the insula, with an emphasis on an objective determination of the extent of resection and neurological complications and on an analysis of the anatomical characteristics that can lead to suboptimal outcomes. METHODS: Twenty-two patients who underwent surgical resection of intrinsic insular tumors were retrospectively identified. Eight tumors (36%) were purely insular, eight (36%) extended into the temporal pole, and six (27%) extended into the frontal operculum. A transsylvian surgical approach, combined with a frontal opercular resection or temporal lobectomy when necessary, was used in all cases. Five of 13 patients with tumors located in the dominant hemisphere underwent craniotomies while awake. The extent of tumor resection was determined using volumetric analyses. In 10 patients, more than 90% of the tumor was resected; in six patients, 75 to 90% was resected; and in six patients, less than 75% was resected. No patient died within 30 days after surgery. During the immediate postoperative period, the neurological conditions of 14 patients (64%) either improved or were unchanged, and in eight patients (36%) they worsened. Deficits included either motor or speech dysfunction. At the 3-month follow-up examination, only two patients (9%) displayed permanent deficits. Speech and motor dysfunction appeared to result most often from excessive opercular retraction and manipulation of the middle cerebral artery (MCA), interruption of the lateral lenticulostriate arteries (LLAs), interruption of the long perforating vessels of the second segment of the MCA (M2), or violation of the corona radiata at the superior aspect of the tumor. Specific methods used to avoid complications included widely splitting the sylvian fissure and identifying the bases of the periinsular sulci to define the superior and inferior resection planes, identifying early the most lateral LLA to define the medial resection plane, dissecting the MCA before tumor resection, removing the tumor subpially with preservation of all large perforating arteries arising from posterior M2 branches, and performing craniotomy with brain stimulation while the patient was awake. CONCLUSIONS: A good understanding of the surgical anatomy and an awareness of potential pitfalls can help reduce neurological complications and maximize surgical resection of insular tumors.

Surgical resection of calvarial metastases overlying dural sinuses.

OBJECTIVE: Few reports have addressed the surgical management of cranial metastases that overlie or invade the dural venous sinuses. To examine the role of surgery in the treatment of dural sinus calvarial metastases, we reviewed retrospectively 13 patients who were treated with surgery at the University of Texas M.D. Anderson Cancer Center between 1993 and 1999. We compared them with 14 patients who had calvarial metastases that did not involve a venous sinus. METHODS: Clinical charts, radiological studies, pathological findings, and operative reports were analyzed retrospectively. RESULTS: The median age of patients with dural sinus calvarial metastases was 54 years. Nine patients were men and four were women. Renal cell carcinoma and sarcoma were the most common primary tumors. Similar features were noted in the 14 patients with nonsinus calvarial metastases. Of the 13 dural sinus calvarial metastases, 11 involved the superior sagittal sinus, and 2 involved the transverse sinus. In nine patients, the involved sinus was resected, and in four patients, the sinus was reconstituted after tumor removal. Nine patients in the dural sinus calvarial metastases group received en bloc resection, and four received piecemeal resection. No operative deaths occurred. The overall median actuarial survival was 16.5 months. The survival times of the two groups were comparable. In the group with dural sinus calvarial metastases, transient postoperative neurological deficits occurred in two patients (15%), and a permanent deficit occurred in one patient (8%). No patients in the group with nonsinus calvarial metastases experienced deficits after resection. Compared with piecemeal resection, en bloc resection was associated with significantly less blood loss. CONCLUSION: Complete extirpation of calvarial metastases that overlie or invade a dural sinus can be achieved with only slightly more morbidity than complete removal of calvarial metastases that are located away from the sinuses. En bloc resection is as safe as piecemeal resection and is more effective in limiting operative blood loss. The overall recurrence and survival rates of patients with dural sinus calvarial metastases are similar to those of patients with calvarial metastases that do not involve the sinuses. Therefore, involvement of a dural venous sinus should not discourage resection of calvarial metastases. In carefully selected cancer patients, surgery provides effective palliation of symptomatic calvarial metastases that overlie or invade the venous sinuses.

Ophthalmological outcome following orbital resection in anterior and anterolateral skull base surgery.

OBJECT: Partial resection of the orbital bones is not uncommon during the excision of anterior and anterolateral skull base tumors. Controversy exists regarding the need for and extent of the reconstruction necessary following this resection. The authors studied this factor in a series of patients. METHODS: The authors conducted a retrospective review of 56 patients in whom resection of 57 anterior or anterolateral skull base tumors and partial excision of the orbital bone were performed. Adverse ophthalmological outcomes were noted in 16 patients, in nine of whom adverse outcomes were believed to be directly related to resection of the orbital walls. Some degree of orbital reconstruction was performed during 23 of the 57 procedures. An adverse orbit-related outcome was strongly associated with resection of the orbital floor and with resection of two thirds or more of two or more orbital walls but not with the presence of absence or orbital reconstruction. The latter finding, however, is likely a function of selection bias. CONCLUSIONS: In most patients after partial excision of the orbital bones, elaborate reconstruction is not necessary. Isolated medial and lateral orbital wall defects or combined superior and lateral orbital wall defects, especially in cases in which the periorbita is intact, probably do not require primary reconstruction. In cases of orbital floor defects, whether isolated or part of a multiple wall resection, primary reconstruction is recommended.

Transmandibular, circumglossal, retropharyngeal approach for chordomas of the clivus and upper cervical spine. Technical note.

Extensive clival tumors that involve both the midline and lateral skull base compartments, or those that extend inferiorly to the anterior cervical spine, are difficult to expose in a wide fashion using any of the transmaxillary, transoral, or transcervical routes. In the transmandibular, circumglossal, retropharyngeal (TCR) approach wide access of this region can be obtained, thus allowing for a more complete resection of tumor and infiltrated bone. It also provides for an improved ability to perform dural reconstruction, should it be necessary. Over the past 4 years four patients with extensive clival chordomas underwent resection via the TCR approach. Gross-total resection was achieved in two patients, a greater than 98% resection in one patient, and a greater than 95% resection in the fourth patient. The surgical technique, all approach-related complications and morbidity, and patient outcome are discussed. If an expanded exposure of the clivus is necessary, the TCR approach is a good choice as well as a useful surgical technique to have available.

A multivariate analysis of 416 patients with glioblastoma multiforme: prognosis, extent of resection, and survival.

OBJECT: The extent of tumor resection that should be undertaken in patients with glioblastoma multiforme (GBM) remains controversial. The purpose of this study was to identify significant independent predictors of survival in these patients and to determine whether the extent of resection was associated with increased survival time. METHODS: The authors retrospectively analyzed 416 consecutive patients with histologically proven GBM who underwent tumor resection at the authors' institution between June 1993 and June 1999. Volumetric data and other tumor characteristics identified on magnetic resonance (MR) imaging were collected prospectively. CONCLUSIONS: Five independent predictors of survival were identified: age, Karnofsky Performance Scale (KPS) score, extent of resection, and the degree of necrosis and enhancement on preoperative MR imaging studies. A significant survival advantage was associated with resection of 98% or more of the tumor volume (median survival 13 months, 95% confidence interval [CI] 11.4-14.6 months), compared with 8.8 months (95% CI 7.4-10.2 months; p < 0.0001) for resections of less than 98%. Using an outcome scale ranging from 0 to 5 based on age, KPS score, and tumor necrosis on MR imaging, we observed significantly longer survival in patients with lower scores (1-3) who underwent aggressive resections, and a trend toward slightly longer survival was found in patients with higher scores (4-5). Gross-total tumor resection is associated with longer survival in patients with GBM, especially when other predictive variables are favorable.

Primary extradural meningiomas: a report on nine cases and review of the literature from the era of computerized tomography scanning.

OBJECT: Primary meningiomas arising outside the intracranial compartment (primary extradural meningiomas [PEMs]) are rare tumors. To develop a better understanding of these tumors and to establish a comprehensive classification scheme for them, the authors analyzed a series of patients treated at the M. D. Anderson Cancer Center (MDACC) and reviewed all cases reported in the English-language literature since the inception of the use of computerized tomography (CT) scanning. METHODS: Clinical records, results of radiographic studies, and histological slides were reviewed for all cases of PEM at MDACC. Demographic features, symptoms, tumor location, histological grade, and patient outcome were assessed in all cases. A comprehensive literature search identified 168 PEMs in 142 patients reported during the CT era. These reports were also analyzed for common features. Tumors for both data sets were classified as purely extracalvarial (Type I), purely calvarial (Type II), and calvarial with extracalvarial extension (Type III). Type II and Type III tumors were further categorized as convexity (C) or skull base (B) lesions. The incidence of PEMs at MDACC was 1.6%, which was consistent with the rate reported in the literature. In both data sets, the male/female ratio was nearly 1:1. The most common presenting symptom was a gradually expanding mass. The age of patients at diagnosis of PEM was bimodal, peaking during the second decade and during the fifth to seventh decades. In all MDACC cases and in 90% of those reported in the literature the PEMs were located in the head and neck. The majority of tumors originated in the skull (70%). In the MDACC series and in the literature review, the majority (67% and 89%, respectively) of tumors were histologically benign. Although fewer PEMs were malignant or atypical (33% at MDACC and 11% in the literature), their incidence was higher than that observed for primary intracranial meningiomas. Distant metastasis was not a common feature reported for patients with PEMs (6% in the literature). Outcome data were available in 96 of the cases culled from the CT-era literature. The combination of the MDACC data and the data obtained from the literature demonstrated that patients with benign Type IIB or Type IIIB lesions were more likely to experience recurrence than patients with benign Type IIC or Type IIIC tumors (26% compared with 0%, p < 0.05). The more aggressive atypical and malignant tumors were associated with a statistically significant higher death rate (29%) relative to benign tumors (4.8% death rate, p < 0.004). CONCLUSIONS: Defining a tumor as a PEM is dependent on the tumor's relation to the dura mater and the extent and direction of its growth. Classification of PEMs as calvarial or extracalvarial and as convexity or skull base lesions correlates well with clinical outcome.

Primary malignant tumors of the sphenoidal sinus.

OBJECTIVE: Neoplasms of the paranasal sinuses account for 0.2 to 0.8% of all cancers and for 2 to 3% of head and neck cancers. Primary involvement of the sphenoidal sinus has been reported to occur in only 1 to 2% of all paranasal sinus tumors. Tumors at this site commonly present with cranial neuropathies and require multidisciplinary treatment. METHODS: During a 21-year period, 27 patients with primary sphenoidal sinus tumors were evaluated. Those with pituitary tumors and fibro-osseous lesions (fibrous dysplasia) were excluded. The records of all remaining patients were reviewed for the following parameters: age, sex, and ethnic origin; presenting symptoms and signs; radiological features and sites of tumor extension; pathological findings; prior treatments; surgical approaches and extent of resection; adjuvant therapy; complications; and patient outcome. RESULTS: A malignant pathological process was found in 26 of the 27 patients. The most common diseases were squamous cell carcinoma (n = 9), adenoid cystic carcinoma (n = 4), chondrosarcoma (n = 3), and neuroendocrine carcinoma (n = 3). Treatments included surgery (n = 1), surgery and radiotherapy (n = 6), surgery and chemotherapy (n = 3), surgery, radiotherapy, and chemotherapy (n = 4), chemotherapy (n = 5), chemotherapy and radiotherapy (n = 3), and radiotherapy (n = 2). The mean follow-up period from initial evaluation was 41 months (range, 2-199 mo). At the last follow-up, 12 patients (48%) were still alive and 13 (52%) had died. The 2-year survival rate for patients with squamous cell carcinoma was 44%. CONCLUSION: Headache and visual disturbances are common symptoms of sphenoidal sinus tumors. The presence of cranial neuropathies, although common, suggests a less successful outcome. It is advantageous if the surgical team has had experience with both open and endoscopic approaches to the sphenoidal sinus. Patient outcome is maximized by the use of aggressive multidisciplinary therapy.

Thermally induced transient trigeminal sensory neuropathy: imaging findings.

We report the clinical and imaging features of a patient with transient partial trigeminal sensory neuropathy thought to have been induced by thermal injury to the tongue. Abnormal thickening and enhancement of the mandibular division of the trigeminal nerve was revealed by MR imaging. The diagnostic considerations for mass-like enlargement of the trigeminal nerve should include transient/inflammatory processes, as well as more common and sinister conditions, such as tumor.

Postoperative swelling of pericranial pedicle graft producing intracranial mass effect. Report of two cases.

Two cases of florid swelling of pericranial pedicle grafts are reported. Intracranial mass effect produced by the grafts necessitated reoperation with graft removal in one case and graft revision in the other. No permanent neurological deficits were incurred by either patient. Venous congestion and associated swelling within the graft were considered to be related to constriction of the graft base at the frontal bone flap-skull base interface in one patient and torsion of the graft base in the other.

Benign nonmeningeal tumors of the cavernous sinus.

OBJECTIVE: Resection of benign cavernous sinus tumors with minimum morbidity and mortality is increasingly more common. Although meningiomas have dominated most discussions, numerous nonmeningeal tumors also deserve attention because they are generally more amenable to radical surgical resection. METHODS: We reviewed the records of 40 patients (19 female and 21 male patients) who were treated surgically during a 15-year period for benign, nonmeningeal tumors of the cavernous sinus. Invasive pituitary adenomas (14 cases) and trigeminal schwannomas (13 cases) comprised the majority of tumors; the remaining tumor types were hemangioma, neurofibroma, juvenile angiofibroma, dermoid tumor, giant cell tumor, chondromyxofibroma, and chondroma. Patient age ranged from 7 to 65 years (mean, 37 yr). RESULTS: All patients underwent surgery, the intent being total resection. Total resection was achieved in 33 (82.5%) of the 40 patients. Follow-up was achieved via a combination of direct patient visit or phone interview or via the referring physician. Eight patients had undergone previous surgery elsewhere, which correlated closely with complications and neurophthalmological outcomes but did not affect resectability. Postoperatively, 89.7% of the patients had either stable or improved extraocular muscle function compared with their preoperative statuses. Forty percent of the patients experienced improvement of their preoperative extraocular muscle deficits. Complications included cerebrospinal fluid leak (three cases), postoperative hemorrhage (one case), fat embolism (one case), perforator distribution infarct (one case), hydrocephalus requiring ventriculoperitoneal shunting (two cases), transient hemiparesis (one case), and diabetes insipidus (one case). CONCLUSION: We conclude that benign nonmeningeal tumors of the cavernous sinus can be safely and radically removed and result in good long-term neuro-ophthalmological function and low morbidity and mortality. Furthermore, when compared with our previously reported results for cavernous sinus meningiomas, benign nonmeningeal tumors of the cavernous sinus carry a better chance of total removal, a lower incidence of postoperative ocular dysfunction, and a higher rate of recovery of preoperative cranial nerve deficits.

Aggressive fibromatosis involving the cranial base in children.

OBJECTIVE: Aggressive fibromatoses are proliferations of fibrous tissue that infiltrate surrounding tissues and tend to recur after surgical resection. We report a series of five children with aggressive fibromatosis involving the cranial base. METHODS: A search of the database maintained by the Medical Informatics Department at The University of Texas M.D. Anderson Cancer Center identified 78 patients with aggressive fibromatosis of the head and neck. Of these patients, five demonstrated involvement of the cranial base. All five were children. A retrospective review of the charts, diagnostic images, and pathological findings for these five patients was performed. RESULTS: Surgery, radiotherapy, chemotherapy, and hormonal therapy were the treatment methods used. Four patients underwent gross total resections, but tumor cells were present at the margins in all cases. Two of these four patients experienced recurrences; one patient was cured of disease by a second surgical resection and radiotherapy, and the other underwent a second gross total resection. The fifth patient underwent a subtotal resection. He has experienced four recurrences and continues to live with residual disease. One transformation to a Grade 2 fibrosarcoma was observed. CONCLUSION: Fibromatoses of the cranial base are rare and seem to be most common in the pediatric age group. Gross total resection, preferably in the first attempt, should be the goal. Radiotherapy and hormonal therapy are useful adjunctive methods for the treatment of aggressive fibromatosis. Fibromatoses that involve the cranial base are most likely to require a multidisciplinary treatment approach.

Imaging of perineural tumor spread from palatal carcinoma.

Carcinomas of the hard or soft palate are known to spread perineurally along palatine branches of the maxillary nerve. Imaging of perineural tumor spread from the palate has been underemphasized in the imaging literature. We report the findings from eight patients in whom spread from primary cancers of the palate was seen along the palatine nerves. Indications of perineural spread include enlargement or excessive enhancement of a nerve, or abnormal density/signal intensity, enhancement, or widening of the pterygopalatine fossa, cavernous sinus, or Meckel's cave.

Palatal adenoid cystic carcinoma presenting as perineural spread to the cavernous sinus.

Adenoid cystic carcinoma of the hard or soft palate is a slow-growing, insidious disease with a tendency to spread via a perineural mechanism along the palatine branches of the maxillary division of the trigeminal nerve. Such spread may present first as a tumor in the pterygopalatine fossa or cavernous sinus. The purpose of this manuscript is to report three cases of palatal adenoid cystic carcinoma which presented as a mass in the cavernous sinus prior to discovery of the palate primary tumor. The imaging features of these cases are emphasized as is the need to search for a head and neck primary lesion when faced with a cavernous sinus mass.

Craniospinal dissemination of central neurocytoma. Report of two cases.

Central neurocytoma was first described in the literature in 1982 and has been noted to be a benign neuronal tumor usually located in the ventricular system. Of the more than 100 reported cases, only seven recurrences have been reported, all of which have been local. The authors report two cases of recurrent central neurocytoma that disseminated through the ventricular system with seeding to the spine, as evidenced by magnetic resonance images and positive cerebrospinal fluid cytology. The histological appearance of these two tumors was typical for the lesion and lacked evidence of malignant change. Central neurocytoma may not be as benign as previously thought, and the recognition of this more malignant behavior has implications for patient follow up and therapy.

The treatment of recurrent unresectable and malignant meningiomas with interferon alpha-2B.

OBJECTIVE: Recurrent malignant meningiomas and unresectable meningiomas represent a great therapeutic challenge after the failure of radiation therapy. No effective chemotherapy has been found. We report the preliminary results of the treatment of patients with recurrent unresectable or malignant meningiomas with recombinant interferon alpha-2B (IFN-alpha-2B). METHOD: Each of six patients with either a recurrent malignant meningioma or an unresectable meningioma was treated with IFN-alpha, administered subcutaneously at a dosage of 4 mU/m2 per day, 5 days per week. Two of the six meningiomas were regular, one was atypical, and three were malignant. RESULTS: Five of six patients exhibited positive response to treatment; with stabilization of the size of the tumor in four patients and slight regression in one. The responses observed lasted from 6 to 14 months. The toxicity associated with prolonged use of IFN-alpha was mild and well tolerated. CONCLUSION: These results suggest that IFN-alpha is effective in the treatment of recurrent malignant meningiomas.

Dislocation of the pituitary gland by sphenocavernous meningiomas.

BACKGROUND: Dislocation of the pituitary gland from the sella turcia by tumor has only very rarely been described. To date, only two cases have appeared in the neurosurgical literature. METHODS: After intraoperative identification of pituitary dislocation in five patients with cavernous sinus meningiomas, a review of our most recent 20 patients with cavernous sinus meningiomas was performed. A subgroup of sphenocavernous meningiomas was identified, as were the associations between pituitary dislocation and the patient's endocrinologic status. RESULTS: Of 20 consecutive patients with cavernous sinus meningiomas, seven tumors could be subclassified as sphenocavernous meningiomas due to the significant amount of tumor extension into the sphenoid sinus. At the time of surgery, dislocation of the pituitary gland from the sella into the suprasellar cistern was observed in five of seven of these patients. Four of the five patients with pituitary dislocation had evidence of hypopituitarism, whereas there was no evidence of this condition in the two patients with sphenocavernous meningiomas without pituitary dislocation. CONCLUSIONS: Pituitary dislocation in these patients is likely due to the physical dislocation of the pituitary gland from the sella, through an incompetent diaphragma sella, and by the tumorous extension in the sphenoid sinus. The extension of a cavernous sinus meningioma into the sphenoid sinus, and specifically the sella turcica, may be via the intercavernous venous sinuses. The lack of endocrinologic improvement suggests that the hypopituitarism noted in these patients is likely due to disturbance of the pituitary microcirculation with subsequent pituitary ischemia. The possibility of pituitary dislocation in patients with sphenocavernous meningiomas, especially those with panhypopituitarism, should be considered and searched for at the time of surgery.