A multidisciplinary team approach to skull base chordomas

2001 ◽  
Vol 95 (2) ◽  
pp. 175-183 ◽  
Author(s):  
H. Alan Crockard ◽  
Timothy Steel ◽  
Nicholas Plowman ◽  
Anoushka Singh ◽  
John Crossman ◽  
...  

Object. A multidisciplinary team devised a protocol for long-term care of patients with skull base chordomas. In this study they describe their approach. Methods. Forty-two patients presented between 1986 and 1998 and were treated by maximum surgical cytoreduction and photon radiation therapy. Tumor volume—doubling time determined on the basis of magnetic resonance imaging, immunostaining, and cell proliferation (Ki67 labeling index [LI]) studies indicated growth rates of individual chordomas. The best outlook was associated with the greatest extent of tumor removal achieved during the first operation. There were no deaths associated with patients who underwent first-time surgery, but there was a 7.1% mortality rate associated with those who underwent subsequent operations. Cerebrospinal fluid leaks, additional cranial nerve palsies, and pharyngeal wound problems were the most difficult management problems encountered after second and subsequent surgeries. The time interval between operations was usually between 2 years and 3 years after the first surgery; very few patients required a second surgery, with a quiescent period in excess of 5 years. Life-table 5- and 10-year survival rates were 77% and 69%, respectively. Conclusions. The authors believe that this series of skull base chordomas provides new insights into the management of these lesions, particularly with regard to techniques that increase survival times and studies that aid in formulating prognoses.

2001 ◽  
Vol 95 (2) ◽  
pp. 184-189 ◽  
Author(s):  
H. Alan Crockard ◽  
Anthony Cheeseman ◽  
Timothy Steel ◽  
Tamas Revesz ◽  
Janice L. Holton ◽  
...  

Object. The authors review their experience with treating skull base chondrosarcomas, which are much rarer than skull base chordomas and differ from them in prognosis and treatment. Methods. Seventeen patients (12 male and five female patients) with histologically verified chondrosarcomas were followed up prospectively over a 12-year period. The mean age at presentation was 35.9 years. Most patients presented with cranial nerve palsies. Seven had undergone surgery prior to referral to the authors' unit. All underwent maximum surgical cytoreduction by the most direct surgical approach; only the two patients harboring the mesenchymal variant underwent radiotherapy. Conclusions. One patient died of a pulmonary embolus; the patients harboring mesenchymal chondrosarcomas died at 20 and 36 months, respectively, after treatment. Of the remaining patients, 93% were alive 5 years postsurgery and had a projected 10-year survival rate of 84% (mean survival time 9.3 years). These data emphasize the very slow progression of this tumor compared with skull base chordoma.


2004 ◽  
Vol 100 (5) ◽  
pp. 883-890 ◽  
Author(s):  
James K. Liu ◽  
Steven D. Schaefer ◽  
Augustine L. Moscatello ◽  
William T. Couldwell

Object. Allergic fungal sinusitis (AFS) is a form of paranasal mycosis that often involves bone destruction and extension into the orbit and anterior skull base. Treatment consists of surgical extirpation and a course of corticosteroids. Despite frequent intracranial involvement, AFS is rarely reported in the neurosurgical literature. Methods. The records of 21 patients with the histological diagnosis of AFS were reviewed. The histological diagnosis was based on findings of branching septated fungi interspersed with eosinophilic mucin and Charcot—Leyden crystals without fungal invasion of soft tissue. The average age of the 21 patients in this study was 25 years (range 9–46) and the male/female ratio was 3.75:1. All patients were immunocompetent. All had a history of chronic sinusitis and imaging findings of expansile disease involving multiple sinuses. Fifteen patients had nasal polyposis, eight had erosion of bone, which was observed on computerized tomography (CT) scans, eight had disease extending intracranially, and six had disease that involved the lamina papyracea. All patients underwent transnasal and/or transmaxillary endoscopic approaches for debridement and irrigation, six underwent orbital decompression, and three underwent a bifrontal craniotomy for removal of intracranial extradural disease. No patient had a cerebrospinal fluid leak. Postoperatively, one patient was treated with amphotericin B and the other 20 were treated with a short course of corticosteroids. The follow-up period ranged from 2 to 19 years. Conclusions. Allergic fungal sinusitis is a unique form of fungal disease that may mimic anterior skull base and paranasal sinus tumors. A cranial base team approach of neurosurgeons and otolaryngologists is recommended. Most cases can be successfully managed with transnasal and/or transmaxillary endoscopic techniques. A craniotomy is rarely indicated unless there is the suspicion of dural invasion or extensive intracranial and/or intraorbital involvement that is inaccessible from below.


2021 ◽  
Vol 9 ◽  
pp. 2050313X2110270
Author(s):  
Ruth Maxwell ◽  
Michelle O’Brien ◽  
Deirdre O’Donnell ◽  
Lauren Christophers ◽  
Thilo Kroll

Formal assessments of cognition that rely on language may conceal the non-linguistic cognitive function of people with aphasia. This may have detrimental consequences for how people with aphasia are supported to reveal communicative and decision-making competence. This case report demonstrates a multidisciplinary team approach to supporting the health and social care decision-making of people with aphasia. The case is a 67-year-old woman with Wernicke’s type aphasia. As the issue of long-term care arose, the speech and language therapist used a supported communication approach with the patient who expressed her wish to go home. A multidisciplinary team functional assessment of capacity was undertaken which involved functional assessments and observations of everyday tasks by allied health, nursing, catering and medical staff. In this way, the patient’s decision-making capacity was revealed and she was discharged home. A collaborative multidisciplinary team approach using supported communication and functional capacity assessments may be essential for scaffolding the decision-making capacity of people with aphasia.


1999 ◽  
Vol 90 (4) ◽  
pp. 651-655 ◽  
Author(s):  
Marc S. Schwartz ◽  
James I. Cohen ◽  
Toby Meltzer ◽  
Michael J. Wheatley ◽  
Sean O. McMenomey ◽  
...  

Object. Reconstruction of the cranial base after resection of complex lesions requires creation of both a vascularized barrier to cerebrospinal fluid (CSF) leakage and tailored filling of operative defects. The authors describe the use of radial forearm microvascular free-flap grafts to reconstruct skull base lesions, to fill small tissue defects, and to provide an excellent barrier against CSF leakage.Methods. Ten patients underwent 11 skull base procedures including placement of microvascular free-flap grafts harvested from the forearm and featuring the radial artery and its accompanying venae comitantes. Operations included six craniofacial, three lateral skull base, and two transoral procedures for various diseases. Excellent results were obtained, with no persistent CSF leaks, no flap failures, and no operative infections. One temporary CSF leak was easily repaired with flap repositioning, and at one flap donor site minor wound breakdown was observed. One patient underwent a second procedure for tumor recurrence and CSF leakage at a site distant from the original operation.Conclusions. Microvascular free tissue transfer reconstruction of skull base defects by using the radial forearm flap provides a safe, reliable, low-morbidity method for reconstructing the skull base and is ideally suited to “low-volume” defects.


1988 ◽  
Vol 69 (6) ◽  
pp. 850-860 ◽  
Author(s):  
Paul C. McCormick ◽  
Jacqueline A. Bello ◽  
Kalmon D. Post

✓ A consecutive series of 14 patients with trigeminal schwannoma managed surgically at the Neurological Institute of New York since 1970 is reported. Nine women and five men (mean age 40 years) were diagnosed following a mean symptom duration of 33 months. Abnormalities of trigeminal nerve function were present in 11 patients on admission examination. Facial pain was a prominent feature in eight patients. Two patients, both with schwannomas arising from the trigeminal root, presented initially with typical trigeminal neuralgia. Additional cranial nerve palsies or cerebellar or pyramidal tract signs were noted in eight patients. The surgical approach to these tumors depends on their anatomical location. Four patients had tumors confined to the middle fossa, three patients had tumors limited to the posterior fossa, and seven patients had both supratentorial and infratentorial components of their tumors. Twenty operative procedures were performed on these patients, resulting in complete extirpation in six patients, nearly complete removal in seven patients, and partial removal in one patient. Adherence of the tumor to the lateral wall of the cavernous sinus or the brain stem precluded total removal. There was one postoperative death. In the immediate postoperative period, abnormalities of cranial nerves controlling the extraocular muscles were common. In general, these deficits were transient; however, some permanent loss of trigeminal nerve function occurred in nine patients. Two patients required tarsorrhaphy for neurotropic keratitis, and two patients underwent cerebrospinal fluid (CSF) shunting procedures for hydrocephalus or for a persistent CSF leak. The follow-up period ranged from 4 to 177 months (mean 47 months). The clinical features, anatomical considerations, and surgical approach to these rare tumors are discussed. A clinical review of 106 additional cases of trigeminal schwannoma, reported in the English literature since 1935, is also presented.


2003 ◽  
Vol 98 (4) ◽  
pp. 812-822 ◽  
Author(s):  
Roberto Pallini ◽  
Giulio Maira ◽  
Francesco Pierconti ◽  
Maria Laura Falchetti ◽  
Ester Alvino ◽  
...  

Object. Chordomas of the skull base are generally regarded as slow-growing tumors; however, approximately 20% of these lesions have been shown to recur as early as 1 year postsurgery. The classic pathological paradigms are poor predictors of outcome, and additional markers are needed to identify patients at risk for early tumor recurrence. In this study the authors describe such a marker. Methods. In a series of 26 patients with chordomas of the skull base, the authors investigated the relationship between the biological behavior of the tumor, which was determined according to the interval for its recurrence and volume doubling time, and several pathological and molecular features, which included the histological variant, proliferative activity, mutation of p53 protein, expression of human telomerase reverse transcriptase (hTERT) messenger (m)RNA, loss of heterozygosity (LOH), and microsatellite instability. The major finding in this study was that hTERT mRNA expression in chordoma cells identifies those tumors that exhibit unusually fast rates of growth. The expression of hTERT mRNA was frequently associated with mutation of p53 protein, indicating that telomerase dysfunction combines with abnormal p53 function to initiate the unrestrained clonal expansion of the tumor cells. In cases in which the tumor was partially removed, mutation of p53 protein and expression of hTERT mRNA predicted increased doubling time for residual tumor as well as the probability of tumor recurrence. Cell proliferation, as investigated using the Ki-67 method, was significantly related to the tumor doubling time; however, the authors found that the pattern of cell proliferation was not homogeneous throughout the chordoma tissue, and that the proliferative index might change by a factor as high as 8 among different regions of the same tumor. The LOH and microsatellite instability do not seem to affect the prognosis of skull base chordomas. Conclusions. Reactivation of telomerase in chordomas is a reliable predictor of outcome. The ability to predict the biological behavior of chordomas might have immediate implications in the management of this disease in patients who undergo surgery.


1976 ◽  
Vol 44 (5) ◽  
pp. 556-561 ◽  
Author(s):  
Joseph C. Maroon ◽  
John S. Kennerdell

✓ The authors describe their microsurgical lateral orbital approach to intraorbital tumors. In seven patients ultrasonic scanning, computerized axial tomography, polytomography, orbital venography, and arteriography have allowed precise intraorbital tumor localization relative to the optic nerve. The authors believe that circumscribed tumors superior, lateral, or inferior to the optic nerve can be safely and completely removed through a 30–35-mm lateral skin incision with microsurgical dissecting techniques. A combined neurosurgical-ophthalmological team approach is emphasized.


1981 ◽  
Vol 54 (3) ◽  
pp. 384-387 ◽  
Author(s):  
A. Leland Albright ◽  
Ryland P. Byrd

✓ Gross and histological pathology of skull sutures affected by craniosynostosis was evaluated. Nineteen sutures were examined: 14 sagittal, three coronal, and two metopic. Sutures were removed en bloc, examined grossly, then sectioned perpendicular to the axis of the suture and examined microscopically. Foci of dural invagination into the sutures occurred in only four of the 19 specimens. In areas of maximum clinical abnormality, there was no microscopic evidence of the suture. The suture adjacent to the fused portion was narrowed by encroaching calvaria, and suture farther away was normal. Suture ossification was never multifocal. In infants less than 1 year old, the extent of suture fusion did not correlate with age. It is concluded that craniosynostosis is characterized by the progressive obliteration of cranial sutures by fusion of adjacent cranial bones, and that changes in the skull base angles are secondary to suture obliteration.


1985 ◽  
Vol 63 (4) ◽  
pp. 500-509 ◽  
Author(s):  
Arnold H. Menezes ◽  
John C. VanGilder ◽  
Charles R. Clark ◽  
George El-Khoury

✓ Lack of correlation between the severity of rheumatoid subluxation of the upper cervical vertebrae and supposed absence of neurological damage has led to the erroneous supposition that this finding is innocuous. Incomplete autopsy studies in rheumatoid arthritis have failed to recognize the cause of death, despite previously proven dramatic occipito-atlanto-axial dislocations. The most feared entity of rheumatoid basilar invagination, namely “cranial settling,” is poorly understood. Between 1978 and 1984, the authors treated 45 rheumatoid arthritis patients who were symptomatic with “cranial settling.” This consisted of vertical odontoid penetration through the foramen magnum (9 to 33 mm), occipito-atlanto-axial dislocation, lateral atlantal mass erosion, downward telescoping of the anterior arch of C-1 on the axis, and rostral rotation of the posterior arch of C-1 producing ventral and dorsal cervicomedullary junction compromise. Cervicomedullary junction dysfunction has mistakenly been called “entrapment neuropathy,” “progression of disease,” or “vasculitis.” Occipital pain occurred in all 45 patients, myelopathy in 36, blackout spells in 24, brain-stem signs in 17, and lower cranial nerve palsies in 10. Four patients had prior tracheostomies. Four previously asymptomatic patients with “cranial settling” presented acutely quadriplegic. The factors governing treatment were reducibility and direction of encroachment determined by skeletal traction and myelotomography. Transoral odontoidectomy was performed in seven patients with irreducible pathology. All patients underwent occipitocervical bone fusion (with C-1 decompression if needed) and acrylic fixation. Improvement occurred during traction, implying that compression might be the etiology for the neurological signs. There were no complications. Thus, “cranial settling” is a frequent complication of rheumatoid arthritis; although it is poorly recognized, it has serious implications and is treatable.


2004 ◽  
Vol 100 (4) ◽  
pp. 652-658 ◽  
Author(s):  
Angelo Pompucci ◽  
Giancarla Rea ◽  
Eugenio Farallo ◽  
Marzia Salgarello ◽  
Antonino Campanella ◽  
...  

Object. The authors investigated whether skull base resection and primary free-flap reconstruction in a single-stage surgery is oncologically effective for treating advanced stages of recurrent skin cancer (RSC) of the head. Methods. Eighteen consecutive patients were surgically treated. Twelve of them underwent an anterolateral skull base resection, which was performed using a pterional craniotomy combined with an orbitozygomatic osteotomy. Six patients underwent a posterolateral skull base resection, which was performed using an asterional craniotomy combined with a retrolabyrinthine petrosectomy. The wide postoperative defects were covered with muscular or myocutaneous free flaps. The main factor influencing survival was the extent of the resection: patients with no or minimal residual disease showed a statistically significant longer survival time than those with consistent residual disease. Basal cell carcinoma had a better prognosis than squamous cell carcinoma. A trend toward improved survival was observed in patients classified as T4M0 with negative lymph nodes (N0), but this trend was not statistically significant. Adjuvant radiotherapy significantly influenced both survival time and the rate of local recurrence. The surgical morbidity rate was 27.8%; there were two transient cerebrospinal fluid leaks and three seventh cranial nerve injuries. Late complications included radionecrosis in one patient and skin erosion requiring a second surgery in another patient. No deaths occurred during a 30-day postoperative period. Conclusions. Advances in skull base surgery and free-flap reconstruction allowed the authors to treat patients with advanced-stage RSC of the head in a rather satisfactory manner. Only when it is impossible to achieve no or minimal residual disease should aggressive treatment be considered.


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