The clivus is an atypical metastatic site for renal clear cell carcinoma(RCCC). Here we report a 54 year old man with acute cavernous sinus syndrome. Brain magnetic resonance imaging identified a clival-based lesion w...The clivus is an atypical metastatic site for renal clear cell carcinoma(RCCC). Here we report a 54 year old man with acute cavernous sinus syndrome. Brain magnetic resonance imaging identified a clival-based lesion with associated bony erosion. The patient underwent endoscopic endonasal biopsy and partial resection of the clival mass. Because histologic examination of the resected specimen resulted in a diagnosis of RCCC, contrast-enhanced computed tomography scan of the abdomen was performed and showed an enhanced left renal mass. The patient subsequently underwent laparoscopic left radical nephrectomy and gamma knife was planned for the residual clival lesion. We also retrospectively reviewed available published reports on clival metastases, specifically those from RCCC, since 1990.展开更多
<strong>Objective:</strong> To evaluate the role of expanded endoscopic endonasal approach in removal of clival chordomas. <strong>Patients &</strong> <strong>Methods:</strong> ...<strong>Objective:</strong> To evaluate the role of expanded endoscopic endonasal approach in removal of clival chordomas. <strong>Patients &</strong> <strong>Methods:</strong> Nine patients with clival chordomas were operated upon in Cairo University hospital from September 2015 to September 2018 using the EEEA a recurrent case and seven new cases were involved in these study and ten operations were done. All patients had preoperative neurological and radiological examination. The study was focusing on the approach, efficacy of tumor removal, reconstruction of the base and complications related to this approach. <strong>Results:</strong> Nine patients were operated in this study in which ten operations were done. It included six males (66.6%) and three females (33.3%) with age ranging from 4 years to 63 years with average age 40.7 years. Headache and diplopia were the most common symptoms found in six patients (66.6%). Brainstem affection was found in two patients (22.2%). Lower cranial nerves affection was found in two patients (22.2%). One case developed CSF leakage postoperatively (11.1%). Two patients underwent tracheostomy. We achieve total removal in four patients (44.4%), near total removal in one patient (11.1%) and subtotal tumor resection in four patients (44.4%). <strong>Conclusion:</strong> EEEA for clival chordomas is safe and effective approach regarding the results of the incidence of complications, and the percentage of tumor resection.展开更多
Godtfredsen syndrome or clival syndrome is a rare syndrome of abducens and hypoglossal nerve palsies (cranial nerve 6th and 12th respectively) that localizes to a clival mass. There are few reported cases of this clin...Godtfredsen syndrome or clival syndrome is a rare syndrome of abducens and hypoglossal nerve palsies (cranial nerve 6th and 12th respectively) that localizes to a clival mass. There are few reported cases of this clinical presentation. The aim of this case report is to describe this rare manifestation observed in a woman with clival metastases arising from a thymoma. A previously well 34-year-old native lady presented to a district hospital in Sabah, Malaysia, with history of blurring of vision and headache for 1 month. Cranial nerve examination reveals right abducens nerve palsy (right 6th CN) and right hypoglossal nerve palsy (right 12th CN). Initial imaging with CT brain reveals a subtle extra-axial hyperdense mass adjacent to the clivus and a routine chest x-ray reveals a mediastinal mass. Further imaging of thorax showed right anterior mediastinal mass, which then proceeded with Video-Assisted Thoracoscopic Surgery (VATS) guided biopsy. The biopsy result was consistent with the finding of thymoma, type B2. She was then diagnosed with aggressive form of thymoma, which unfortunately has metastasized to the bone, lung, liver and brain. Despite prognosis at the time of diagnosis is guarded, she still opted to undergo chemotherapy. Despite the completion of 6 cycles of chemotherapy, her disease progressed, and she eventually succumbed to the illness. In short, the presence of combined 6th and 12th palsy should alert clinician to the possibility of clival mass or metastases and hence could earlier workup with appropriate imaging can lead to earlier diagnosis and better treatment outcome.展开更多
Background: Until recently, tumors of the clivus and the anterior region of the posterior cranial fossa were considered extremely difficult to access and often inoperable using standard transcranial approaches. With t...Background: Until recently, tumors of the clivus and the anterior region of the posterior cranial fossa were considered extremely difficult to access and often inoperable using standard transcranial approaches. With the introduction into the neurosurgical practice of minimally invasive methods utilizing endoscopic techniques, it became possible to effectively remove hard-to-reach tumors, including central tumors of the anterior region of the posterior cranial fossa. Methods: From 2008 to the present time, the inpatient institution has operated on 140 patients with various tumors of the base of the skull, localized to the clivus and anterior region of the posterior cranial fossa (65 men and 75 women). The age of patients ranged from 3 to 74 years. Tumor distribution according to the histopathological features was as follows: chordomas, 103 (73.57%);meningiomas, 12 (8.57%);pituitary adenomas, 9 (6.43%);fibrous dysplasia, 4 (2.86%);cholesteatoma, 3 (2.14%);craniopharyngiomas, 2 (1.43%);plasmacytomas, 2 (1.43%);and other tumors (giant cell tumor, neurohypophyseal glioma, osteoma, carcinoid, chondroma), 5 (3.57%). The tumors had the following size distribution: giant (more than 60 mm), 35 (25%);large (35–59 mm), 83 (59.3%);medium (21–35 mm), 21 (15%);and small (less than 20 mm), 1 (0.7%). In 11 cases, intraoperative monitoring of the cranial nerves was performed (21 cranial nerves were identified). Results: Upper, middle, and lower transclival approaches provide access to the anterior surface of the upper, middle, and lower neurovascular complexes of the posterior cranial fossa. The chordoma cases were distributed as follows according to extent of removal: total removal, 68 (66.02%);subtotal removal, 25 (24.27%);and partial removal, 10 (9.71%). The adenomas of the pituitary gland were removed totally in 6 cases, subtotally in 1 case and partially in 2 cases. The meningiomas were removed totally in 1 case, subtotally in 5 cases, and partially in 5 cases, with less than 50% of the tumor removed in 1 case. Other tumors (cholesteatoma, craniopharyngioma, fibrous dysplasia, giant cell tumor, glioma of the neurohypophysis, osteoma, plasmacytoma, carcinoid, and chondroma) were removed totally in 9 cases and subtotally in 7 cases. Postoperative CSF leaks occurred in 9 cases (6.43%) and meningitis in 13 cases (9.29%). Oculomotor disorders developed in 19 patients (13.57%), 12 of which regressed during the period from 4 to 38 days after surgery, and 7 of which were permanent. In 2 cases, surgical treatment had a lethal outcome (1.43%). (Continued on next page) (Continued from previous page) Conclusion: The endoscopic endonasal transclival approach can be used to obtain access to the centrally located tumors of the posterior cranial fossa. It is an alternative to transcranial approaches in the surgical treatment of tumors of the clivus. The results of using this approach are comparable with the results of transcranial and transfacial approaches and, in some cases, surpass them in effectiveness. The extended endoscopic endonasal posterior (transclival) approach, considering its minimally invasive nature, allows fora radical and low-risk (in terms of postoperative complications and lethality) removal of various skull base tumors of central localization with the involvement and without the involvement of the clivus, which, until recently, were considered to be almost inoperable.展开更多
文摘The clivus is an atypical metastatic site for renal clear cell carcinoma(RCCC). Here we report a 54 year old man with acute cavernous sinus syndrome. Brain magnetic resonance imaging identified a clival-based lesion with associated bony erosion. The patient underwent endoscopic endonasal biopsy and partial resection of the clival mass. Because histologic examination of the resected specimen resulted in a diagnosis of RCCC, contrast-enhanced computed tomography scan of the abdomen was performed and showed an enhanced left renal mass. The patient subsequently underwent laparoscopic left radical nephrectomy and gamma knife was planned for the residual clival lesion. We also retrospectively reviewed available published reports on clival metastases, specifically those from RCCC, since 1990.
文摘<strong>Objective:</strong> To evaluate the role of expanded endoscopic endonasal approach in removal of clival chordomas. <strong>Patients &</strong> <strong>Methods:</strong> Nine patients with clival chordomas were operated upon in Cairo University hospital from September 2015 to September 2018 using the EEEA a recurrent case and seven new cases were involved in these study and ten operations were done. All patients had preoperative neurological and radiological examination. The study was focusing on the approach, efficacy of tumor removal, reconstruction of the base and complications related to this approach. <strong>Results:</strong> Nine patients were operated in this study in which ten operations were done. It included six males (66.6%) and three females (33.3%) with age ranging from 4 years to 63 years with average age 40.7 years. Headache and diplopia were the most common symptoms found in six patients (66.6%). Brainstem affection was found in two patients (22.2%). Lower cranial nerves affection was found in two patients (22.2%). One case developed CSF leakage postoperatively (11.1%). Two patients underwent tracheostomy. We achieve total removal in four patients (44.4%), near total removal in one patient (11.1%) and subtotal tumor resection in four patients (44.4%). <strong>Conclusion:</strong> EEEA for clival chordomas is safe and effective approach regarding the results of the incidence of complications, and the percentage of tumor resection.
文摘Godtfredsen syndrome or clival syndrome is a rare syndrome of abducens and hypoglossal nerve palsies (cranial nerve 6th and 12th respectively) that localizes to a clival mass. There are few reported cases of this clinical presentation. The aim of this case report is to describe this rare manifestation observed in a woman with clival metastases arising from a thymoma. A previously well 34-year-old native lady presented to a district hospital in Sabah, Malaysia, with history of blurring of vision and headache for 1 month. Cranial nerve examination reveals right abducens nerve palsy (right 6th CN) and right hypoglossal nerve palsy (right 12th CN). Initial imaging with CT brain reveals a subtle extra-axial hyperdense mass adjacent to the clivus and a routine chest x-ray reveals a mediastinal mass. Further imaging of thorax showed right anterior mediastinal mass, which then proceeded with Video-Assisted Thoracoscopic Surgery (VATS) guided biopsy. The biopsy result was consistent with the finding of thymoma, type B2. She was then diagnosed with aggressive form of thymoma, which unfortunately has metastasized to the bone, lung, liver and brain. Despite prognosis at the time of diagnosis is guarded, she still opted to undergo chemotherapy. Despite the completion of 6 cycles of chemotherapy, her disease progressed, and she eventually succumbed to the illness. In short, the presence of combined 6th and 12th palsy should alert clinician to the possibility of clival mass or metastases and hence could earlier workup with appropriate imaging can lead to earlier diagnosis and better treatment outcome.
文摘Background: Until recently, tumors of the clivus and the anterior region of the posterior cranial fossa were considered extremely difficult to access and often inoperable using standard transcranial approaches. With the introduction into the neurosurgical practice of minimally invasive methods utilizing endoscopic techniques, it became possible to effectively remove hard-to-reach tumors, including central tumors of the anterior region of the posterior cranial fossa. Methods: From 2008 to the present time, the inpatient institution has operated on 140 patients with various tumors of the base of the skull, localized to the clivus and anterior region of the posterior cranial fossa (65 men and 75 women). The age of patients ranged from 3 to 74 years. Tumor distribution according to the histopathological features was as follows: chordomas, 103 (73.57%);meningiomas, 12 (8.57%);pituitary adenomas, 9 (6.43%);fibrous dysplasia, 4 (2.86%);cholesteatoma, 3 (2.14%);craniopharyngiomas, 2 (1.43%);plasmacytomas, 2 (1.43%);and other tumors (giant cell tumor, neurohypophyseal glioma, osteoma, carcinoid, chondroma), 5 (3.57%). The tumors had the following size distribution: giant (more than 60 mm), 35 (25%);large (35–59 mm), 83 (59.3%);medium (21–35 mm), 21 (15%);and small (less than 20 mm), 1 (0.7%). In 11 cases, intraoperative monitoring of the cranial nerves was performed (21 cranial nerves were identified). Results: Upper, middle, and lower transclival approaches provide access to the anterior surface of the upper, middle, and lower neurovascular complexes of the posterior cranial fossa. The chordoma cases were distributed as follows according to extent of removal: total removal, 68 (66.02%);subtotal removal, 25 (24.27%);and partial removal, 10 (9.71%). The adenomas of the pituitary gland were removed totally in 6 cases, subtotally in 1 case and partially in 2 cases. The meningiomas were removed totally in 1 case, subtotally in 5 cases, and partially in 5 cases, with less than 50% of the tumor removed in 1 case. Other tumors (cholesteatoma, craniopharyngioma, fibrous dysplasia, giant cell tumor, glioma of the neurohypophysis, osteoma, plasmacytoma, carcinoid, and chondroma) were removed totally in 9 cases and subtotally in 7 cases. Postoperative CSF leaks occurred in 9 cases (6.43%) and meningitis in 13 cases (9.29%). Oculomotor disorders developed in 19 patients (13.57%), 12 of which regressed during the period from 4 to 38 days after surgery, and 7 of which were permanent. In 2 cases, surgical treatment had a lethal outcome (1.43%). (Continued on next page) (Continued from previous page) Conclusion: The endoscopic endonasal transclival approach can be used to obtain access to the centrally located tumors of the posterior cranial fossa. It is an alternative to transcranial approaches in the surgical treatment of tumors of the clivus. The results of using this approach are comparable with the results of transcranial and transfacial approaches and, in some cases, surpass them in effectiveness. The extended endoscopic endonasal posterior (transclival) approach, considering its minimally invasive nature, allows fora radical and low-risk (in terms of postoperative complications and lethality) removal of various skull base tumors of central localization with the involvement and without the involvement of the clivus, which, until recently, were considered to be almost inoperable.