BACKGROUND Immunoglobulin G4 related disease(Ig G4-RD)is a fibroinflammatory disease with markedly elevated serum Ig G4 levels and fibrous tissue proliferation,accompanied by numerous plasma cells.Ig G4 related hypert...BACKGROUND Immunoglobulin G4 related disease(Ig G4-RD)is a fibroinflammatory disease with markedly elevated serum Ig G4 levels and fibrous tissue proliferation,accompanied by numerous plasma cells.Ig G4 related hypertrophic pachymeningitis(Ig G4-RHP)is relatively rare and indistinguishable from other phymatoid diseases before the operation.The risk of long-term immunosuppression needs to be balanced with disease activity.CASE SUMMARY A 40-year-old man presented with headache and bilateral abducent paralysis.He was also diagnosed with pulmonary tuberculosis 10 years ago and was on regular treatment for the same.Before the operation and steroid therapy,the patient was suspected of having tubercular meningitis at a local hospital.A clivus lesion was found via brain magnetic resonance imaging(MRI)at this presentation.He was preliminarily diagnosed with meningioma and underwent Gamma Knife Surgery.Transnasal endoscopic resection was performed to treat deterioration of nerve function.Postoperative pathologic examination suggested Ig G4-RD.Moreover,the serum Ig G4 was elevated at 1.90 g/L(reference range:0.035-1.500 g/L).After steroid therapy for 2 mo,the lesion size diminished on MRI,and the function of bilateral abducent nerves recovered.CONCLUSION Ig G4-RHP is relatively rare and indistinguishable before the operation.Elevated serum Ig G4 levels and imaging examination help in the diagnosis of Ig G4-RHP.Surgery is necessary when lesions progress and patients start to develop cranial nerve function deficit.展开更多
Chordomas are dysembryogenic tumors originating from the notochordal process [1] [2]. They are aggressive tumours with unique diagnostic and management challenges. Primary therapy is complete surgical removal of the t...Chordomas are dysembryogenic tumors originating from the notochordal process [1] [2]. They are aggressive tumours with unique diagnostic and management challenges. Primary therapy is complete surgical removal of the tumour as much as possible. The likelihood of recurrence is high in spite of complete surgical resection. A 52-year-old female patient presented with complaints of decreased vision in right eye, nasal bleeding, nasal blockage and difficulties in swallowing. CT scan and nasal biopsy were performed which confirmed the diagnosis of clivus chordoma. The CT scan showed extension into nasopharynx, nasal cavity and oropharynx pushing onto the soft palate. Surgical excision of the mass was performed with coblator by both intraoral and intra nasal approach [3]. On follow-up, nasal endoscopy and CT were done;the patient was relieved of the symptoms and was clinically better.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
Objective: To describe the radiological and pathological presentation of a salivary heterotopia in clivus. The lesion was detected incidentally in a 57-year-old woman. Material and Methods: Radiologic imaging (CT scan...Objective: To describe the radiological and pathological presentation of a salivary heterotopia in clivus. The lesion was detected incidentally in a 57-year-old woman. Material and Methods: Radiologic imaging (CT scan and MRI) and histological picture obtained from a clival lesion. Results: Radiologically the lesion presented as a well-limited lytic lesion with a diameter of 1.5 cm and accompanied by a cerebrospinal fluid secondary fistula. Histological examination revealed non-pathological salivary gland tissue. Conclusion: The differential diagnosis of this rare entity mandates a histological examination of the lesion.展开更多
基金Supported by 1·3·5 Project for Disciplines of Excellence–Clinical Research Incubation Project,West China Hospital,Sichuan University,No.2019HXFH018。
文摘BACKGROUND Immunoglobulin G4 related disease(Ig G4-RD)is a fibroinflammatory disease with markedly elevated serum Ig G4 levels and fibrous tissue proliferation,accompanied by numerous plasma cells.Ig G4 related hypertrophic pachymeningitis(Ig G4-RHP)is relatively rare and indistinguishable from other phymatoid diseases before the operation.The risk of long-term immunosuppression needs to be balanced with disease activity.CASE SUMMARY A 40-year-old man presented with headache and bilateral abducent paralysis.He was also diagnosed with pulmonary tuberculosis 10 years ago and was on regular treatment for the same.Before the operation and steroid therapy,the patient was suspected of having tubercular meningitis at a local hospital.A clivus lesion was found via brain magnetic resonance imaging(MRI)at this presentation.He was preliminarily diagnosed with meningioma and underwent Gamma Knife Surgery.Transnasal endoscopic resection was performed to treat deterioration of nerve function.Postoperative pathologic examination suggested Ig G4-RD.Moreover,the serum Ig G4 was elevated at 1.90 g/L(reference range:0.035-1.500 g/L).After steroid therapy for 2 mo,the lesion size diminished on MRI,and the function of bilateral abducent nerves recovered.CONCLUSION Ig G4-RHP is relatively rare and indistinguishable before the operation.Elevated serum Ig G4 levels and imaging examination help in the diagnosis of Ig G4-RHP.Surgery is necessary when lesions progress and patients start to develop cranial nerve function deficit.
文摘Chordomas are dysembryogenic tumors originating from the notochordal process [1] [2]. They are aggressive tumours with unique diagnostic and management challenges. Primary therapy is complete surgical removal of the tumour as much as possible. The likelihood of recurrence is high in spite of complete surgical resection. A 52-year-old female patient presented with complaints of decreased vision in right eye, nasal bleeding, nasal blockage and difficulties in swallowing. CT scan and nasal biopsy were performed which confirmed the diagnosis of clivus chordoma. The CT scan showed extension into nasopharynx, nasal cavity and oropharynx pushing onto the soft palate. Surgical excision of the mass was performed with coblator by both intraoral and intra nasal approach [3]. On follow-up, nasal endoscopy and CT were done;the patient was relieved of the symptoms and was clinically better.
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.
文摘Objective: To describe the radiological and pathological presentation of a salivary heterotopia in clivus. The lesion was detected incidentally in a 57-year-old woman. Material and Methods: Radiologic imaging (CT scan and MRI) and histological picture obtained from a clival lesion. Results: Radiologically the lesion presented as a well-limited lytic lesion with a diameter of 1.5 cm and accompanied by a cerebrospinal fluid secondary fistula. Histological examination revealed non-pathological salivary gland tissue. Conclusion: The differential diagnosis of this rare entity mandates a histological examination of the lesion.
