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Stereotactic gamma knife radiosurgery. Initial North American experience in 207 patients.

The first North American gamma knife for stereotactic radiosurgery of brain tumors and arteriovenous malformations entered the therapeutic armamentarium at the University of Pittsburgh (Pa) on August 14, 1987. In this article, we report our initial testing and subsequent experience with this technique. In the first 16 months of operation, 207 patients were treated (113 had arteriovenous malformations, 78 had extra-axial skull base neoplasms, 9 had glial neoplasms, and 7 had metastatic tumors). The patients' lesions either were considered previously as "inoperable" or were residual lesions after attempted surgical resection, or the radiosurgery was performed after the patient declined surgical excision. Gamma radiosurgery was associated with no surgical mortality and no significant early morbidity, and the results were encouraging during the minimum follow-up period of 6 months. Compared with treatment by conventional intracranial surgery (craniotomy), both the average length of stay and hospital charges for radiosurgery were significantly lower. Our initial experience further suggests that stereotactic radiosurgery using the gamma knife is a therapeutically effective and economically sound alternative to microneurosurgical removal of selected intracranial tumors and vascular malformations.

Adolescent↗

Chondroid chordoma: fine-needle aspiration cytology with histopathological, immunohistochemical, and ultrastructural study of two cases.

Chondroid chordoma is a controversial and confusing entity that was originally described by Heffelfinger et al. (Cancer 1973; 32:410-420) as a biphasic malignant neoplasm possessing elements of both chordoma and cartilaginous tissue. Fine-needle aspiration (FNA) cytology of chondroid chordoma has not been described. The aim of our investigation was to characterize the chondroid area of chondroid chordoma and to compare the FNA features with those of well-differentiated chondrosarcoma. Clival and cervical spine chondroid chordomas were studied with light microscopy, immunohistochemistry, and electron microscopy. Chondroid chordomas demonstrated an epithelial nature by immunohistochemistry and ultrastructural studies. The FNA smears showed low cellularity, with loosely arranged or dispersed round cells in a myxoid background. Although the smears were similar to those of well-differentiated chondrosarcomas, they showed a positive reaction for epithelial markers. These findings reveal that chondroid chordoma is a variant of chordoma which possesses a hyaline matrix. Immunohistochemical demonstration of epithelial markers is useful to distinguish it from chondrosarcoma. Diagn. Cytopathol. 1999; 21:335-339.

Biomarkers, Tumor↗

Unusual presentation of multiple myeloma with unilateral visual loss and numb chin syndrome in a young adult.

A 39-year-old man presented with unilateral visual loss as the first sign of multiple myeloma (MM). His visual loss was due to a plasmacytoma in the sphenoid sinus compressing the optic nerve with resultant optic nerve atrophy. Shortly after this presentation he developed numb chin syndrome due to a mandibular plasmocytoma compressing the mental nerve. His MM progressed rapidly despite treatment with high-dose steroids and alkylating agents and he required allogeneic bone marrow transplantation in order to achieve a remission. We reviewed the published medical literature on the presentation of MM with visual impairment and present a summary in tabular form in this paper. This is the first reported case of MM presenting with isolated complete visual loss due to intracranial extrinsic compression of the optic nerve since the advent of modern cross-sectional imaging. Multiple myeloma needs to be included in the differential diagnosis of cranial neuropathies and skull base neoplasms even in adults under 40 years of age.

Adult↗

Outcome of craniofacial resection in patients 70 years of age and older.

BACKGROUND: Craniofacial resection (CFR) for patients over 70 years of age is uncommon. This study examines a cohort of 36 patients who had CFR at a single institution with the aim of reporting mortality, complications, and outcome. METHODS: Thirty-six patients 70 years of age and older were identified from a prospective database of 234 patients who had CFR at a single institution. The median age was 72 years (range, 70-87). Seventeen (47%) patients had had prior single-modality or combined treatment, which included surgery in 14 (40%), radiation in 13 (36%), and chemotherapy in 2 (6%). Thirty-five patients had a malignant tumor and 1 patient a benign tumor; 15 (42%) had high-grade, 17 (47%) intermediate-grade, and 4 (11%) low-grade pathology. The margins of resection were close or microscopically positive in 18 (50%). Adjuvant radiotherapy was given in 15 (42%) and chemotherapy in 1 (3%). Complications were classified into overall, local, central nervous system (CNS), systemic, and orbital. Overall survival (OS) and disease-specific survival (DSS) were determined using the Kaplan-Meier method. Outcomes were compared with patients less than 70 years of age. RESULTS: Postoperative mortality occurred in 6 (17%) patients and postoperative complications occurred in 23 (64%) patients. Local wound complications occurred in 11 (30%), CNS in 12 (33%), systemic in 6 (17%), and orbital in 1 (3%). Postoperative mortality and complications were significantly higher in patients 70 years of age and older compared with patients less than 70 years of age (17% versus 1.5%, p = .0005; 64% versus 36%, p = .003, respectively). With a median follow-up of 27 months (range, 1-237), the 3 year OS and DSS were significantly poorer than patients less than 70 years of age (OS: 53% versus 69%, p = .0004; DSS: 61% versus 70%, p = .01) due to increased medical comorbidity (53% versus 24%, p = .001) and poorer histology (high-, intermediate-, low-grade histology: 42%, 47%, 11% versus 26%, 47%, 27%, p = .05, respectively) in patients over 70 years of age. CONCLUSION: CFR in patients 70 years of age and older is associated with increased mortality, increased incidence of complications, and a poorer overall and disease-specific 3-year survival, compared with patients less than 70 years of age. The survival was likely due to increased medical comorbidity and adverse histology. These factors must be taken into account when considering an elderly patient for craniofacial resection.

Age Factors↗

Craniopharyngioma extended into the posterior fossa.

A 7-year-old girl with a craniopharyngioma with a rare extension into the posterior fossa is presented. MRI revealed a solid calcified mass in the sellar and suprasellar region with a cyst expanded to the posterior fossa up to the foramen magnum and causing hydrocephalus and brainstem displacement. The tumor was removed totally with right pterional approach.

Child↗

[Reconstruction of the frontobasal skull after tumor operations and trauma. Transplant selection and functional outcome].

Defects of the frontoorbital complex subsequent to extensive tumor surgery or severe trauma often result in aesthetic and functional disharmonies. The long-term results of rehabilitation related to different materials and techniques are the subject of this study. From 1974 to 1996 altogether 127 patients with bony defects of the orbit and/or the skull base underwent surgery. All patients' data were documented prospectively from 1984 onwards. The results following reconstruction with autologous calvarian transplants in 52 patients were compared to those of a control group which received iliac bone or PMMA implants. Eighty-one bony defects (64%) resulted from tumor resection; 37 patients (29%) suffered from the effects of trauma. In 67 cases (53%) reconstruction was performed primarily, in 51 cases (40%) secondarily. Free or pedicled soft-tissue transplants were necessary in order to separate the orbit and the neurocranium in 26% of the defects. On the other hand, small, isolated defects of the orbital roof (7%) were left without any reconstructive procedure. Contrary to the iliac bone grafts, the calvarian transplants resisted secondary resorption. Postoperative infections appeared in two cases; loss of transplants was avoided entirely in the group of calvarian reconstructions in contrast to the alloplasts. The selection of a suitable donor site area and rigid fixation with microplates led to excellent esthetic results even in cases with large defects.

Adolescent↗

Drop metastases in a patient with a chondroid chordoma of the clivus.

Metastasising chordomas are extremely rare and only four cases with drop metastases have been reported. We report a patient with an intracranial chondroid chordoma, typically involving the clivus, treated by repeated resection, percutaneous transluminal embolisation and radiosurgery. During follow-up with MRI asymptomatic intradural drop metastases were observed throughout the spine, with transgression of the intervertebral foramen, forming a "dumbbell".

Adult↗

MR-guided laser-induced thermotherapy of the infratemporal fossa and orbit in malignant chondrosarcoma via a modified technique.

A 76-year-old patient presented with a recurrent mass of a malignant chondrosarcoma in the right infratemporal fossa and in the left maxillary sinus with orbital invasion. The patient was treated with a palliative intention with MR-guided laser-induced thermotherapy using a modified applicator technique. Following treatment clinical symptoms improved and MRI revealed complete laser-induced tumor necrosis.

Aged↗

Ectopic clival craniopharyngioma.

Ectopic craniopharyngioma is a rare entity. The authors present a very rare case of an ectopic clival craniopharyngioma completely separate from the sella turcica.A 44-year old woman presented with abducens palsy. A MR imaging study and a CT scan revealed a cystic clival lesion separate from the sella turcica. Surgical resection was performed successfully with flexible endoscope-assisted procedure using an endonasal transsphenoidal approach. No evidence of involvement of the sellar region was found according to radiological, intra-operative, and clinical findings.A review of the literature revealed no other such cases. The discussion includes the formation of craniopharyngioma from the ectopic Rathke's pouch remnants and the surgical approach for clival lesions. We believe that our approach provides good results with minimal invasiveness for some clival lesions.

Adult↗

Three-layer reconstruction with fascia lata and vascularized pericranium for anterior skull base defects.

BACKGROUND: We report an assessment of the efficiacy of a triple layer graft composed of fascia lata and vascularized pericranium for anterior skull base reconstruction. This technique is based on the concept that vascularized tissue over a free flap may promote vascularization and rapid wound healing. METHOD: A large fascial graft is prepared from the fascia lata and divided in two pieces and trimmed to a size larger than the bone and dural defect. Vascularized pericranium is harvested after bicoronal incision and elevating the bifrontal scalp flap down to the supraorbital rims. First is dural repair, which is performed with fascia lata placed between the brain and remaining dura. Second, fascia lata is placed over the skull base defect and secured with mini titanium screws over the cranial surface of the orbital ridges. Third, vascularized pericranium is laid between the two layers of fascia lata. FINDINGS: We studied 17 patients of whom 2 had malignancy, 6 had olfactory groove meningioma, 6 had skull base fracture and rhinorrhea, 1 case had orbital meningioma, 1 had invasive pituitary adenoma and 1 had basal encephalocele. The transbasal approach was used as a single procedure in 13 cases. The extended transbasal approach combined with a transfacial approach was used in 3 cases and with a pterional approach in 1 case. In each patient, reconstruction of the cranial base was performed with triple layer graft of fascia lata and vascularized pericranium. The patients were followed-up 2 months to 5 years. None of the patients experienced postoperative cerebrospinal fluid leakage, meningitis, abscess, brain herniation and tension pneumocephalus. INTERPRETATION: Fascia lata with vascularized pericranium is highly reliable, tensile and well suited for reconstruction of the anterior skull base.

Adolescent↗

Radiofrequency thermal ablation for recurrent meningioma extending extracranially.

BACKGROUND: Image-guided and temperature-controlled radiofrequency thermal ablation techniques were applied to reduce tumor volume and relieve the symptoms caused by extracranial extension of recurrent meningioma. METHOD: We treated two patients with recurrent meningioma, an 81-year-old woman presenting with bulging of the temple and a 68-year-old woman presenting with visual disturbance, facial disfigurement, and sensory disturbance. Neuroimaging in both patients, revealed a large tumor extending extracranially and involving the infratemporal fossa. To avoid injury to important anatomical structures either compressed or entrapped by the tumor, the spatial relation between the planned ablation volume and these structures was confirmed by 3-D reconstruction of the ablation target. During the ablation procedure, local temperatures over the tissue being cauterized were continuously monitored to limit the ablation area to that within the planned volume adjusting RF power. FINDING: Radiofrequency ablation produced tumor necrosis as planned without adverse effects and resulted in swift relief of symptoms and signs with shrinkage of the tumor. CONCLUSION: This technique may be an effective alternative for recurrent meningiomas extending extracranially and for which radical surgical procedures are not indicated.

Aged↗

A congenital teratoma with a cleft palate: report of a case.

Teratomas are benign tumors containing cells from ectodermal, mesodermal and endodermal layers. They occur in about 1 in every 4000 births and most commonly in the sacrococcygeal region, followed by the ovaries. Congenital epignathus teratomas are rare embryological neoplasms localised in the region of head and neck. An epignathus is found in approximately 1:35,000 to 1:200,000 live births. This accounts for 2-9% of all teratomas. Size and location of the neoplasm in the oronasopharynx is variable. Teratomas are partly undiagnosed at the time of birth. They may exist with an intracranial extension or as small polyps. Large epignathi can lead to difficult management during and after birth. The case of a newborn girl with a combination of an epignathus and a cleft palate is described. The epignathus presented as a huge mass extending out of the mouth of the infant girl. On the day of birth debulking of the extraoral portion of the tumor, followed by intraoral exstirpation, was performed. The results of the histologic examination indicated a congenital epignathus. Six months later a recurrence was found.

Cleft Palate↗

Chromosomal imbalances in a recurrent solitary fibrous tumor of the orbit.

Using comparative genomic hybridization (CGH), array CGH, fluorescence in situ hybridization, and loss of heterozygosity analysis, we examined a recurrent solitary fibrous tumor of the orbit for chromosomal imbalances. In the primary tumor, loss of chromosomal material was observed at 9p, 9q, and 16q. In the first recurrent tumor, cells with these abnormalities were detected, but in some parts of the tumor, cells with losses at 13q (homozygous deletion at 13qter) and 20p were dominant. In the second recurrence, only cells with losses at 13q and 20p were seen. Although morphologically similar, the second recurrent tumor invaded the anterior cranial fossa and demonstrated considerably faster growth than the first recurrent tumor. Thus, the clone of tumor cells that dominated the second recurrent tumor was shown by cytogenetic analysis to be different from that present in the primary tumor, and was associated with a more aggressive nature of the tumor.

Adult↗

Navigated dorsolateral suboccipital transcondylar (NADOSTA) approach for treatment of hypoglossal schwannoma. Case report and review of the literature.

Hypoglossal schwannoma is a rare skull base neoplasm, which lies in the midst of extremely complex anatomical structures. We report a 39-year-old man who presented with a history of right glossal hemiatrophy, dysesthesia and weakness of the left extremities. These symptoms were caused by a unilateral hypoglossal schwannoma located at the cranial base. This lesion was exposed through a dorsolateral suboccipital transcondylar approach, which was navigated with the frameless stereotaxy (NADOSTA). We also describe the epidemiology, symptomatology and the diagnosis of hypoglossal schwannoma as well as literature review of the operative approach. By introducing this minimally invasive dorsal surgical approach with neuronavigation in treating hypoglossal schwannoma, we believe that safe exposure and complete tumor resection can be achieved with minimal rate of morbidity.

Adult↗

Acute ocular motor mononeuropathies: prospective study of the roles of neuroimaging and clinical assessment.

The role for immediate neuroimaging in patients 50 years of age or older with acute isolated third, fourth, and sixth nerve palsies is controversial. We prospectively evaluated 66 patients, aged 50 years and older (median 67 years, range 50-85), with acute isolated ocular motor mononeuropathies. Our purpose was to evaluate both the role of neuroimaging and the role of clinical assessment in determining etiology. We found that clinical features, including time to maximal diplopic symptoms, were not predictive of etiology (median 2 days to maximal diplopic symptoms for both peripheral microvascular and other etiologies). The presence of any common vascular risk factor, including diabetes mellitus, hypertension, hypercholesterolemia, or coronary artery disease, was significantly associated with peripheral microvascular etiology in this cohort (p=0.0004, Fisher's exact test). Despite the high prevalence of peripheral microvascular ischemia as an etiology in this age group, other causes were identified by magnetic resonance imaging (MRI) or computed tomography (CT) scanning in 14% of patients. Diagnoses included brainstem and skull base neoplasms, brainstem infarcts, aneurysms, demyelinating disease, and pituitary apoplexy. Neuroimaging procedures may have a role in the initial evaluation of patients 50 years of age or older with acute ocular motor mononeuropathies.

Abducens Nerve Diseases↗

Giant myxomas of the maxillofacial skeleton and skull base.

OBJECTIVES: To review our experience with patients diagnosed with giant myxomas of the maxillofacial skeleton. STUDY DESIGN: All patients undergoing excision of myxomas of the head and neck from September 1998 through September 2003 with a minimum follow-up of 1 year by the senior author (YD) were included in the study. METHODS: A retrospective chart review was conducted to select all patients who met the inclusion criteria. Clinical presentation, preoperative radiology findings, excisions performed, reconstruction, and follow-up were recorded and reported. RESULTS: Four patients were identified who met the inclusion criteria. All underwent wide en bloc excision of the tumor with various reconstructions. Complete resection was achieved in each case, and no patients have had evidence of recurrence. CONCLUSIONS: Giant myxomas of the maxillofacial skeleton have been reported to have significant rates of recurrence. Wide en bloc resection with appropriate reconstruction can result in excellent quality of life postoperatively and minimize the risk of recurrence. Lesser resections may not be appropriate especially in giant myxomas because of the potential morbidity that would be associated with a multifocal recurrence. EBM RATING: C-4.

Adult↗

Orbital tumors.

Neoplasms involving the orbit are divided into primary and secondary types. Primary orbital tumors are rare, include a wide variety of histopathologic types, are usually benign, and most often are managed surgically. Secondary orbital tumors are most often malignant, arise from the paranasal sinuses, and are managed with multiple modalities in a planned fashion. This article discusses primary and secondary orbital tumors, including their evaluation and management.

Humans↗