BACKGROUND This report describes a case of intracranial multiple inflammatory pseudotumors(IP)after endoscopic resection of a craniopharyngioma,which is relatively rarely reported in the literature,and neurosurgeons s...BACKGROUND This report describes a case of intracranial multiple inflammatory pseudotumors(IP)after endoscopic resection of a craniopharyngioma,which is relatively rarely reported in the literature,and neurosurgeons should be aware of its existence.CASE SUMMARY Herein,we report the case of a 56-year-old man who developed decreased visual acuity and blurred vision without obvious cause or inducement on April 27,2020.To seek further treatment,he went to the Department of Neurosurgery,Clinical Medical College,Yangzhou University.After falling ill,there was no nausea,vomiting,limb convulsions,obvious disturbance of consciousness,speech disorders,cough,or persistent fever.The neurological examination findings were normal,and pituitary magnetic resonance imaging(MRI)revealed multiple nodules with abnormal signals in the sellar region.The diagnosis was craniopharyngioma.We performed total resection of the tumor via transnasal endoscopy,and the postoperative pathology suggested that the type of tumor was craniopharyngioma.Six months after the operation,the patient experienced sudden hearing loss in the right ear,tinnitus in both ears,and numbness on the right side of the face and head.Meanwhile,cranial MRI showed multiple IP.After steroid hormone and anti-inflammatory therapy,the above symptoms did not significantly improve.Finally,the patient's symptoms were well improved by surgery,and the postoperative pathological diagnosis was multiple IP.CONCLUSION Intracranial inflammatory pseudotumor is a benign disease with slow progression,but the clinical symptoms and imaging findings are not typical,there are no pathological findings,and the diagnosis is relatively difficult.Most of the cases are treated by surgical resection,and the prognosis is good after surgery.展开更多
Craniopharynigoma samples were collected from 36 patients. Out of the 36 samples, 29 achieved successful sub-culturing, with a success rate of 80.6%. Immunohistochemistry staining showed that cytokeratin-7 was positiv...Craniopharynigoma samples were collected from 36 patients. Out of the 36 samples, 29 achieved successful sub-culturing, with a success rate of 80.6%. Immunohistochemistry staining showed that cytokeratin-7 was positively expressed in the cytomembrane and cytoplasm of craniopharyngioma cells at 6-8 passages, confirming that all cultured cells were squamous epithelial cells. The doubling time of craniopharyngioma cells was 3 days, as confirmed by the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide assay. In this study, craniopharyngioma cells cultured in vitro were established; however, establishment of immortalized craniopharyngioma cell lines requires further research.展开更多
BACKGROUND Cerebral venous sinus thrombosis(CVST)is a rare condition in patients with craniopharyngioma following transsphenoidal surgery.CASE SUMMARY A 56-year-old man who underwent transsphenoidal surgery for cranio...BACKGROUND Cerebral venous sinus thrombosis(CVST)is a rare condition in patients with craniopharyngioma following transsphenoidal surgery.CASE SUMMARY A 56-year-old man who underwent transsphenoidal surgery for craniopharyngioma 26 d ago presented gradual headache and cerebrospinal fluid leakage while vomiting 5 d post-discharge and required readmission to our department of neurosurgery.After admission,head imaging examination showed a hyperdense shadow in the superior sagittal sinus and right transverse sinus,edema at the bilateral parietal lobe,and hemorrhage at the left parietal lobe and right occipital lobe;the venous phase of cerebral angiography revealed CVST.The patient was treated immediately by intravenous thrombolysis,endovascular thrombolysis,and mechanical thrombectomy after the definite diagnosis.However,the neurological status of the patient continued to deteriorate and he died on the fourth day after readmission.CONCLUSION For craniopharyngioma undergoing transsphenoidal surgery,it is vital to take an effective strategy to manage the postoperative complications,such as diabetes insipidus,severe electrolyte imbalance,and cerebrospinal fluid leakage.Additionally,the early differential diagnosis of CVST is essential when it develops clinical symptoms,especially in patients following transsphenoidal surgery with a high risk of CVST.Subsequently,the timely and effective treatment of the CVST is critical for preventing neurological deterioration.展开更多
Objective. To explore an effective method of surgical management of craniopharyngioma. Subjects and methods. Fifty patients with craniopharyngioma had total andsubtotal tumor ectomy. There were 29 males and 21 females...Objective. To explore an effective method of surgical management of craniopharyngioma. Subjects and methods. Fifty patients with craniopharyngioma had total andsubtotal tumor ectomy. There were 29 males and 21 females, ranging in age from 15 to 56 years (mean 34.1 years). MR imaging showed that the tumors were locatedin the superior sellar region in 24 cases, in superior sella region and extended into the third ventricular floor in 19 cases, into parasella in 3 cases and down to intrasella in 4 cases. Complete cystic tumors were found in 5 cases, whilethe partial cystic tumor in 24 cases and complete solid tumors in 21 cases. Pterional approach was used in 48 patients and subfrontal approach in 2 patients. Great attention was paid to the preservation of the perforating arteries from thecarotid, posterior and anterior communicating and anterior choroidal arteries to the hypothalamic structures. The clinical outcome was evaluated according to the GOS scale.Results. Of the 50 patients surgically treated, 47 patients obtained total ectomy of the tumor and 3 patients with the secondary surgery had subtotal ectomy of the tumor. The pituitary stalk was preserved in 29(58%) patients, severed in 14 patients and unidentified in 7 patients. Forty-six patients regained a normal life; one patient needed assist in life. Of the 3 deaths, one patient died of diabetes insipidus, one of inhalation asphyxia, and another one of water and sodium disorders. Conclusion. Avoidance of the injury to the neural structures in the thirdventricular floor and preservation of the perforating arteries to hypothalamus are the key to achieve good surgical results in treating craniopharyngioma.展开更多
Objective Typically,the transcranial approach has been used for the treatment of craniopharyngiomas with suprasellar extension,whereas the transsphenoidal approach has been used mostly for infradiaphragmatic craniopha...Objective Typically,the transcranial approach has been used for the treatment of craniopharyngiomas with suprasellar extension,whereas the transsphenoidal approach has been used mostly for infradiaphragmatic craniopharyngioma.Total resection of craniopharyngioma can reduce the recurrence rate,especially in young children,but it may lead to severe complications.Therefore,any benefit of the degree of resection must be weighed against the risk of complications by the surgeons.The purpose of this study was to explore the therapeutic outcome after transsphenoidal microsurgical treatment of infradiaphragmatic craniopharyngioma and share our experiences.Methods Between January 2003 and June 2013,30 patients with infradiaphragmatic craniopharyngioma underwent transsphenoidal microsurgical resection in our hospital.The neurological,visual,and endocrine functions,and extent of resection were analyzed retrospectively.Recurrence or growth of residual tumor tissue during follow-up was assessed using magnetic resonance imaging(MRI).Results Total resection was achieved in 25 patients(83.3%),subtotal resection was achieved in 4 patients(13.3%),and partial resection was achieved in 1 patient(3.4%).There were no perioperative deaths.Cerebrospinal fluid(CSF) leakage occurred in 6 patients,and among them,2 required surgical repair of the sella.New-onset postoperative diabetes insipidus(DI) developed in 8 patients.Vision and visual fields were improved at different levels in 13 out of 16 patients who had sight impediments before treatment.Tumor recurrence and regrowth was observed in 2 patients;1 patient underwent transsphenoidal reoperation,the condition of the other patient who had undergone several craniotomies grew worse over the 6-month follow-up period.Conclusion Transsphenoidal surgery is an ideal choice in treating infradiaphragmatic craniopharyngioma.The transsphenoidal approach,which preserves pituitary function and avoids damage to the hypothalamic structures and optic nerve,is associated with fewer complications than the transcranial approach and a low mortality rate.展开更多
Objective Few studies have investigated the differences in outcomes between primary and repeat surgery for a craniopharyngioma in adults.As a result,a treatment concept for adult patients with a craniopharyngioma has ...Objective Few studies have investigated the differences in outcomes between primary and repeat surgery for a craniopharyngioma in adults.As a result,a treatment concept for adult patients with a craniopharyngioma has not yet been established.The present study aimed to retrospectively analyze adult patients with craniopharyngioma to compare surgical outcomes between primary surgery and surgery for recurrence.Methods The demographic and clinical data of 68 adult patients with craniopharyngioma who had primary surgery(n=50)or surgery for recurrence(n=18)were retrospectively analyzed.In addition,the patients were followed up for an average of 38.6 months(range:1–133 months).Results The cohorts of patients undergoing primary surgery or repeat surgery did not differ preoperatively in terms of demographic data,or radiological tumor features.However,patients with recurrent craniopharyngioma had significantly more pituitary hormone deficits and hypothalamo-pituitary disorders before surgery compared with patients with newly diagnosed craniopharyngioma.The success rate of complete resection in primary surgery was 53.2%.Even after repeat surgery,a satisfactory rate of complete resection of 35.7%was achieved.Operative morbidity was increased neither in patients with repeat surgery compared with those with primary surgery(postoperative bleeding P=0.560;meningitis P=1.000;CSF leak P=0.666;visual disturbance P=0.717)nor in patients with complete resection compared with those with partial resection.We found no difference in recurrence-free survival between initial surgery and repeat surgery(P=0.733).The recurrence rate was significantly lower after complete resection(6.9%)than after partial resection(47.8%;P<0.001).Conclusion Attempting complete resection is justified for not only those with newly diagnosed craniopharyngioma but also for those with recurrent craniopharyngioma.However,the surgeon must settle for less than total resection if postoperative morbidity is anticipated.展开更多
Objective.To study the treatment of the patients with blood sodium disorder after craniopharyngioma surgery. Method.The blood sodium in 44 patients with craniopharyngiomas was daily examined from operative to post-ope...Objective.To study the treatment of the patients with blood sodium disorder after craniopharyngioma surgery. Method.The blood sodium in 44 patients with craniopharyngiomas was daily examined from operative to post-operative days. Hypernatremia is defined as [Na]>145 mmol/l, and hyponatremia as [Na]<135 mmol/L. Results.Of the 44 patients, 36 developed sodium disorder. Among them, simple hyponatremia was 16 patients, simple hypernatremia was 9 patients, and alternative sodium disorder was 11 patients. Conclusion.The blood sodium disorder in patients with craniopharyngiomas after surgery appears to present complicated changes. Three types of blood sodium disorder could be determined as simple hyponatremia, simple hypernatremia, and alternative sodium disorder. Treatment of the sodium disorder in patients with craniopharyngiomas after surgery could be carried in accordance with the above three types.展开更多
Objective: This review article attempts to examine and provide an overview of the risk factors associated with craniopharyngioma recurrence. Methods: A literature review of articles relating to the recurrences of cran...Objective: This review article attempts to examine and provide an overview of the risk factors associated with craniopharyngioma recurrence. Methods: A literature review of articles relating to the recurrences of craniopharyngioma and the clinical, molecular prognostic indicators of recurrence and treatment outcomes was performed retrospectively. Results: A total of 107 studies which described specific risk factors related to craniopharyngioma recurrence were identified which included but not limited to 54 retrospective case series, 7 systematic reviews, 21 laboratory reports, 13 case reports and 12 literature reviews. Conclusion: Based on the evidence identified in this review, the risk factors for recurrence in craniopharyngioma management are interrelated in a complex way, and surgery with or without adjuvant radiotherapy is reported to be of long-term benefit, but a disparity in findings suggests no definitive consensus on the risk factors of craniopharyngioma recurrence. More high-quality research is needed.展开更多
<strong>Background: </strong>Craniopharyngioma is a benign tumor which represents 2% - 3% of all intracranial tumors, there are two types: childhood type which affects children between 5 and 10 years and a...<strong>Background: </strong>Craniopharyngioma is a benign tumor which represents 2% - 3% of all intracranial tumors, there are two types: childhood type which affects children between 5 and 10 years and adulthood type which affects patients 50 - 60 years old. The presenting symptoms develop over years and include visual, endocrine, hypothalamic, neurological and neurophysiological manifestations. Surgery is the treatment of choice. Postoperative radiotherapy, gamma-knife and intra tumoral injection of chemotherapeutic drugs have been used as an adjuvant therapy in some cases. <strong>Objectives: </strong>In this study, we evaluated the role of endoscopy in assisting microscopic surgical removal of craniopharyngioma. <strong>Methods: </strong>Eleven patients were operated upon in Cairo University Hospitals, Egypt. All operations were done using microscope through the subfrontal approach. At the end of surgery, the endoscope was used to detect any residual tumor in the subchiasmatic and retrochiasmatic areas and to visualize the posterior part of the tumor which couldn’t be seen by the microscope to check if it was adherent to the hypothalamus and to evaluate whether to be removed or not. <strong>Results:</strong> The study included eleven cases, four of which were childhood type and seven adult type craniopharyngiomas. Total removal was achieved in six cases (five cases of adulthood type). Oumaya reservoir was inserted in five cases;ventriculoperitoneal shunt was needed in five cases. All cases suffered from temporary diabetes insipidus postoperatively, while only two cases developed permanent diabetes insipidus. Three cases presented preoperatively with pituitary hypofunction and two cases developed postoperative pituitary hypofunction, which necessitated hormone replacement therapy. <strong>Conclusion: </strong>Craniopharyngioma is one of the most difficult and challenging tumors for neurosurgeons due to its relation to optic nerve, hypothalamus and vascular system formed by Willis circle and its perforating branches. Endoscopy has a role in decision making after microscopic removal of craniopharyngioma.展开更多
<strong>Objective:</strong> This retrospective study aims to survey the clinical outcomes of 341 consecutive patients surgically treated for and diagnosed with craniopharyngioma (CP) treated in a 10-year p...<strong>Objective:</strong> This retrospective study aims to survey the clinical outcomes of 341 consecutive patients surgically treated for and diagnosed with craniopharyngioma (CP) treated in a 10-year period in a single institution. <strong>Methods:</strong> The clinical reports of three hundred forty-one patients CP patients treated surgically between January 2006 and December 2016 were reviewed and analyzed retrospectively. <strong>Results:</strong> Our cohort consisted of 341 patients (202 male, 139 female) with a mean age of 34.9 years (range 1 - 74 years);Tumor Features: 129 patients (37.8%) had cystic tumors, 88 (23.8%) had solid tumors whereas 126 (36.4%) had heterogeneous lesions with a solid and cystic portion;calcifications were present in 139 (40.8%);Tumor Topography: Suprasellar 198 (58.1%), Intrasellar 40 (11.7%), Intra-third ventricular 103 (30.2%);Surgical approaches used among the patients included: Pterional 262 (76.8%), Transsphenoidal (TS) 42 (12.3%) Transcallosal 20 (5.9%), Transcortical 16 (4.7%) suboccipital 1 and combined approach 1;Gross total removal (GTR) was achieved in 247 patients (72.4%), Subtotal removal (STR) in 94 patients (27.6%). Good postoperative outcome at discharge was achieved in 324 (95%) patients while 17 (5%) patients had poor outcome including 5 (1.5%) perioperative deaths. Mean hospital length of stay was 21.87 (8 - 129). There were 42 (12.9%) recurrences with a mean time to recurrence of 28.36 (3 - 84) months, among which 37 (88.1%) underwent surgery for recurrence treatment. Follow-up time ranged from 3 months to 10 years. There was a statistical significance between open transcranial surgery and suprasellar tumors (p < 0.0001), TS and intrasellar tumors (p < 0.0001);postoperative diabetes inspidus and gross total resection (p < 0.0001);GTR and cystic tumors (p = 0.034) calcification and GTR (p = 0.0008). <strong>Conclusion:</strong> Good surgical outcome and long-term tumor control can be achieved through individual-based selective resection, whether total or subtotal resection. Whereas surgical prehistory of CP was found to be a significant risk factor to recurrence (p < 0.0001), the clinical risk factors of CP of recurrence are still arguable, including the presence residual tumor due to subtotal resection. More future studies are necessary.展开更多
Background:Adamantinomatous craniopharyngioma(ACP)is the commonest pediatric sellar tumor.No effective drug is available and interpatient heterogeneity is prominent.This study aimed to identify distinct molecular subg...Background:Adamantinomatous craniopharyngioma(ACP)is the commonest pediatric sellar tumor.No effective drug is available and interpatient heterogeneity is prominent.This study aimed to identify distinct molecular subgroups of ACP based on the multi-omics profiles,imaging findings,and histological features,in order to predict the response to anti-inflammatory treatment and immunotherapies.Methods:Totally 142 Chinese cases diagnosed with craniopharyngiomas were profiled,including 119 ACPs and 23 papillary craniopharyngiomas.Whole-exome sequencing(151 tumors,including recurrent ones),RNA sequencing(84 tumors),and DNA methylome profiling(95 tumors)were performed.Consensus clustering and non-negative matrix factorization were used for subgrouping,and Cox regression were utilized for prognostic evaluation,respectively.Results:Three distinct molecular subgroups were identified:WNT,ImA,and ImB.The WNT subgroup showed higher Wnt/β-catenin pathway activity,with a greater number of epithelial cells and more predominantly solid tumors.The ImA and ImB subgroups had activated inflammatory and interferon response pathways,with enhanced immune cell infiltration and more predominantly cystic tumors.Mitogen-activated protein kinases(MEK/MAPK)signaling was activated only in ImA samples,while IL-6 and epithelial-mesenchymal transition biomarkers were highly expressed in the ImB group,mostly consisting of children.The degree of astrogliosis was significantly elevated in the ImA group,with severe finger-like protrusions at the invasive front of the tumor.The molecular subgrouping was an independent prognostic factor,with the WNT group having longer event-free survival than ImB(Cox,P=0.04).ImA/ImB cases were more likely to respond to immune checkpoint blockade(ICB)therapy than the WNT group(P<0.01).In the preliminary screening of subtyping markers,CD38 was significantly downregulated in WNT compared with ImA and ImB(P=0.01).Conclusions:ACP comprises three molecular subtypes with distinct imaging and histological features.The prognosis of the WNT type is better than that of the ImB group,which is more likely to benefit from the ICB treatment.展开更多
Background Craniopharyngioma is a common intracranial tumor located in the sellar-suprasellar region.Due to the involvement of adjacent structures,it can lead to increased intracranial pressure,visual impairment,and e...Background Craniopharyngioma is a common intracranial tumor located in the sellar-suprasellar region.Due to the involvement of adjacent structures,it can lead to increased intracranial pressure,visual impairment,and endocrine deficiencies.Surgical resection is the primary treatment,but it is a tough challenge to achieve total resection,which will led to the frequency of recurrences and progressions.Among them,distant spread is extremely rare,but important complication,identifying and providing proper therapy,is crucial.Methods We report two cases of ectopic recurrence craniopharyngioma and make a literature review for the published similar case reports.Results Our literature review revealed 63 cases(including our patient).The onset age in children group and adult group ranges from 2-14 years old(6.70±3.33)to 17-73 years old(40.63±15.58),while the interval year between tumor initiation and ectopic recurrence ranges from 0.17-20(7.28±6.76)years to 0.3-34(6.85±7.29).Achieving gross total resection seems not to prevent the ectopic recurrence.The major pathology of ectopic recurrence craniopharyngioma is adamantinomatous type.The most common site of ectopic recurrence is frontal lobe.According to the pathogenesis,35 cases were seeding along the surgical approach,and 28 cases were seeding via the CSF pathway.Conclusion Ectopic recurrence craniopharyngioma is rare,but it can lead to serious symptoms.Delicate surgical procedure can help to reduce the risk of ectopic recurrence,and standardized follow-up can provide valuable information for treatment.展开更多
Background Generally, total surgical removal of craniopharyngioma results in satisfactory outcome with a low recurrence rate, however, the location of the tumor and its adherence to the hypothalamic structures can mak...Background Generally, total surgical removal of craniopharyngioma results in satisfactory outcome with a low recurrence rate, however, the location of the tumor and its adherence to the hypothalamic structures can make the operation difficult. The goal of the present study was to assess the outcome of craniopharyngiomas in 284 patients treated surgically. Methods A total of 284 patients (151 men and 133 women) with craniopharyngioma were treated surgically by our neurosurgeons from January 1996 to March 2006. Among them, 226 (79.6%) patients were adults (15 years of age or older; mean, 35.8 ± 10.6), 58 (20.4%) were children (14 years of age or younger; mean, 9.1 ± 3.8). The diameter of the tumors were 2.0-9.0 cm (mean, 36.54± 11.4). The tumors were classified into the superior (23 patients) and inferior ventricular (261) types according to the location of the tumor relative to the third ventricular floor. For the patients with craniopharyngioma of inferior ventricular type, pterional approach was used in 191 (67.3%) patients, subfrontal approach in 17 (6.0%), and translamina terminalis through frontobasal interhemispheric approach in 53 08.7%). For those with the tumors of superior ventricular type, transcallosal approach into the anterior third ventricle was done in 10 (3.5%) patients, and the lamina terminalis approach in 13 (4.6%). Of the 284 patients, 204 (71.8%) were followed up for 0.5 to 8 years (mean, 2.1 ± 1.8), including 162 patients received total tumor removal, and 37 underwent subtotal or partial removal. Results Total, subtotal and partial removal of the tumors were achieved in 237 (83.5%), 34 (12.0%) and 13 (4.5%) patients, respectively. The pituitary stalk was preserved in 176 (62.0%) patients, severed in 52 (18.3%), and unidentified in 56 (19.7%). Twelve (4.2%) patients died within one month after the surgery. During the follow-up, 23 (14.1%) patients experienced tumor recurrence 1.0-3.5 years (mean, 1.8± 1.6) after total tumor removal, and 24 (64.9%) had recurrent tumor 0.25- 1.5 years (mean, 0.5 ± 0.4) after subtotal or partial resection. Normal activities of daily living were regained in 63 (80%) patients, independence in 29 (14.2%), and daily life with assistance in 9 (4.4%). Four (2.0%) patients died 0.9-3 years (mean, 1.6± 1.4) after discharge from hospital, 3 of them died of hypothalamic deficiency. Conclusions We can protect the hypothalamic structures and its perforating arteries by choosing surgical approaches according to the location of craniopharygioma relative to the third ventricular floor. The mortality, morbidity, and recurrence rate in patients received total resection are lower than those of patients underwent subtotal or partial resections. In addition, preservation of the pituitary stalk is critical when total tumor resection is feasible.展开更多
文摘BACKGROUND This report describes a case of intracranial multiple inflammatory pseudotumors(IP)after endoscopic resection of a craniopharyngioma,which is relatively rarely reported in the literature,and neurosurgeons should be aware of its existence.CASE SUMMARY Herein,we report the case of a 56-year-old man who developed decreased visual acuity and blurred vision without obvious cause or inducement on April 27,2020.To seek further treatment,he went to the Department of Neurosurgery,Clinical Medical College,Yangzhou University.After falling ill,there was no nausea,vomiting,limb convulsions,obvious disturbance of consciousness,speech disorders,cough,or persistent fever.The neurological examination findings were normal,and pituitary magnetic resonance imaging(MRI)revealed multiple nodules with abnormal signals in the sellar region.The diagnosis was craniopharyngioma.We performed total resection of the tumor via transnasal endoscopy,and the postoperative pathology suggested that the type of tumor was craniopharyngioma.Six months after the operation,the patient experienced sudden hearing loss in the right ear,tinnitus in both ears,and numbness on the right side of the face and head.Meanwhile,cranial MRI showed multiple IP.After steroid hormone and anti-inflammatory therapy,the above symptoms did not significantly improve.Finally,the patient's symptoms were well improved by surgery,and the postoperative pathological diagnosis was multiple IP.CONCLUSION Intracranial inflammatory pseudotumor is a benign disease with slow progression,but the clinical symptoms and imaging findings are not typical,there are no pathological findings,and the diagnosis is relatively difficult.Most of the cases are treated by surgical resection,and the prognosis is good after surgery.
基金supported by the National Natural Science Foundation of China, No. 30872646 and 30973082
文摘Craniopharynigoma samples were collected from 36 patients. Out of the 36 samples, 29 achieved successful sub-culturing, with a success rate of 80.6%. Immunohistochemistry staining showed that cytokeratin-7 was positively expressed in the cytomembrane and cytoplasm of craniopharyngioma cells at 6-8 passages, confirming that all cultured cells were squamous epithelial cells. The doubling time of craniopharyngioma cells was 3 days, as confirmed by the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide assay. In this study, craniopharyngioma cells cultured in vitro were established; however, establishment of immortalized craniopharyngioma cell lines requires further research.
文摘BACKGROUND Cerebral venous sinus thrombosis(CVST)is a rare condition in patients with craniopharyngioma following transsphenoidal surgery.CASE SUMMARY A 56-year-old man who underwent transsphenoidal surgery for craniopharyngioma 26 d ago presented gradual headache and cerebrospinal fluid leakage while vomiting 5 d post-discharge and required readmission to our department of neurosurgery.After admission,head imaging examination showed a hyperdense shadow in the superior sagittal sinus and right transverse sinus,edema at the bilateral parietal lobe,and hemorrhage at the left parietal lobe and right occipital lobe;the venous phase of cerebral angiography revealed CVST.The patient was treated immediately by intravenous thrombolysis,endovascular thrombolysis,and mechanical thrombectomy after the definite diagnosis.However,the neurological status of the patient continued to deteriorate and he died on the fourth day after readmission.CONCLUSION For craniopharyngioma undergoing transsphenoidal surgery,it is vital to take an effective strategy to manage the postoperative complications,such as diabetes insipidus,severe electrolyte imbalance,and cerebrospinal fluid leakage.Additionally,the early differential diagnosis of CVST is essential when it develops clinical symptoms,especially in patients following transsphenoidal surgery with a high risk of CVST.Subsequently,the timely and effective treatment of the CVST is critical for preventing neurological deterioration.
文摘Objective. To explore an effective method of surgical management of craniopharyngioma. Subjects and methods. Fifty patients with craniopharyngioma had total andsubtotal tumor ectomy. There were 29 males and 21 females, ranging in age from 15 to 56 years (mean 34.1 years). MR imaging showed that the tumors were locatedin the superior sellar region in 24 cases, in superior sella region and extended into the third ventricular floor in 19 cases, into parasella in 3 cases and down to intrasella in 4 cases. Complete cystic tumors were found in 5 cases, whilethe partial cystic tumor in 24 cases and complete solid tumors in 21 cases. Pterional approach was used in 48 patients and subfrontal approach in 2 patients. Great attention was paid to the preservation of the perforating arteries from thecarotid, posterior and anterior communicating and anterior choroidal arteries to the hypothalamic structures. The clinical outcome was evaluated according to the GOS scale.Results. Of the 50 patients surgically treated, 47 patients obtained total ectomy of the tumor and 3 patients with the secondary surgery had subtotal ectomy of the tumor. The pituitary stalk was preserved in 29(58%) patients, severed in 14 patients and unidentified in 7 patients. Forty-six patients regained a normal life; one patient needed assist in life. Of the 3 deaths, one patient died of diabetes insipidus, one of inhalation asphyxia, and another one of water and sodium disorders. Conclusion. Avoidance of the injury to the neural structures in the thirdventricular floor and preservation of the perforating arteries to hypothalamus are the key to achieve good surgical results in treating craniopharyngioma.
基金Supported by the grants from the National Clinical Key Specialty Construction Project and National Natural Science Foundation of China(No.81270865)
文摘Objective Typically,the transcranial approach has been used for the treatment of craniopharyngiomas with suprasellar extension,whereas the transsphenoidal approach has been used mostly for infradiaphragmatic craniopharyngioma.Total resection of craniopharyngioma can reduce the recurrence rate,especially in young children,but it may lead to severe complications.Therefore,any benefit of the degree of resection must be weighed against the risk of complications by the surgeons.The purpose of this study was to explore the therapeutic outcome after transsphenoidal microsurgical treatment of infradiaphragmatic craniopharyngioma and share our experiences.Methods Between January 2003 and June 2013,30 patients with infradiaphragmatic craniopharyngioma underwent transsphenoidal microsurgical resection in our hospital.The neurological,visual,and endocrine functions,and extent of resection were analyzed retrospectively.Recurrence or growth of residual tumor tissue during follow-up was assessed using magnetic resonance imaging(MRI).Results Total resection was achieved in 25 patients(83.3%),subtotal resection was achieved in 4 patients(13.3%),and partial resection was achieved in 1 patient(3.4%).There were no perioperative deaths.Cerebrospinal fluid(CSF) leakage occurred in 6 patients,and among them,2 required surgical repair of the sella.New-onset postoperative diabetes insipidus(DI) developed in 8 patients.Vision and visual fields were improved at different levels in 13 out of 16 patients who had sight impediments before treatment.Tumor recurrence and regrowth was observed in 2 patients;1 patient underwent transsphenoidal reoperation,the condition of the other patient who had undergone several craniotomies grew worse over the 6-month follow-up period.Conclusion Transsphenoidal surgery is an ideal choice in treating infradiaphragmatic craniopharyngioma.The transsphenoidal approach,which preserves pituitary function and avoids damage to the hypothalamic structures and optic nerve,is associated with fewer complications than the transcranial approach and a low mortality rate.
文摘Objective Few studies have investigated the differences in outcomes between primary and repeat surgery for a craniopharyngioma in adults.As a result,a treatment concept for adult patients with a craniopharyngioma has not yet been established.The present study aimed to retrospectively analyze adult patients with craniopharyngioma to compare surgical outcomes between primary surgery and surgery for recurrence.Methods The demographic and clinical data of 68 adult patients with craniopharyngioma who had primary surgery(n=50)or surgery for recurrence(n=18)were retrospectively analyzed.In addition,the patients were followed up for an average of 38.6 months(range:1–133 months).Results The cohorts of patients undergoing primary surgery or repeat surgery did not differ preoperatively in terms of demographic data,or radiological tumor features.However,patients with recurrent craniopharyngioma had significantly more pituitary hormone deficits and hypothalamo-pituitary disorders before surgery compared with patients with newly diagnosed craniopharyngioma.The success rate of complete resection in primary surgery was 53.2%.Even after repeat surgery,a satisfactory rate of complete resection of 35.7%was achieved.Operative morbidity was increased neither in patients with repeat surgery compared with those with primary surgery(postoperative bleeding P=0.560;meningitis P=1.000;CSF leak P=0.666;visual disturbance P=0.717)nor in patients with complete resection compared with those with partial resection.We found no difference in recurrence-free survival between initial surgery and repeat surgery(P=0.733).The recurrence rate was significantly lower after complete resection(6.9%)than after partial resection(47.8%;P<0.001).Conclusion Attempting complete resection is justified for not only those with newly diagnosed craniopharyngioma but also for those with recurrent craniopharyngioma.However,the surgeon must settle for less than total resection if postoperative morbidity is anticipated.
文摘Objective.To study the treatment of the patients with blood sodium disorder after craniopharyngioma surgery. Method.The blood sodium in 44 patients with craniopharyngiomas was daily examined from operative to post-operative days. Hypernatremia is defined as [Na]>145 mmol/l, and hyponatremia as [Na]<135 mmol/L. Results.Of the 44 patients, 36 developed sodium disorder. Among them, simple hyponatremia was 16 patients, simple hypernatremia was 9 patients, and alternative sodium disorder was 11 patients. Conclusion.The blood sodium disorder in patients with craniopharyngiomas after surgery appears to present complicated changes. Three types of blood sodium disorder could be determined as simple hyponatremia, simple hypernatremia, and alternative sodium disorder. Treatment of the sodium disorder in patients with craniopharyngiomas after surgery could be carried in accordance with the above three types.
文摘Objective: This review article attempts to examine and provide an overview of the risk factors associated with craniopharyngioma recurrence. Methods: A literature review of articles relating to the recurrences of craniopharyngioma and the clinical, molecular prognostic indicators of recurrence and treatment outcomes was performed retrospectively. Results: A total of 107 studies which described specific risk factors related to craniopharyngioma recurrence were identified which included but not limited to 54 retrospective case series, 7 systematic reviews, 21 laboratory reports, 13 case reports and 12 literature reviews. Conclusion: Based on the evidence identified in this review, the risk factors for recurrence in craniopharyngioma management are interrelated in a complex way, and surgery with or without adjuvant radiotherapy is reported to be of long-term benefit, but a disparity in findings suggests no definitive consensus on the risk factors of craniopharyngioma recurrence. More high-quality research is needed.
文摘<strong>Background: </strong>Craniopharyngioma is a benign tumor which represents 2% - 3% of all intracranial tumors, there are two types: childhood type which affects children between 5 and 10 years and adulthood type which affects patients 50 - 60 years old. The presenting symptoms develop over years and include visual, endocrine, hypothalamic, neurological and neurophysiological manifestations. Surgery is the treatment of choice. Postoperative radiotherapy, gamma-knife and intra tumoral injection of chemotherapeutic drugs have been used as an adjuvant therapy in some cases. <strong>Objectives: </strong>In this study, we evaluated the role of endoscopy in assisting microscopic surgical removal of craniopharyngioma. <strong>Methods: </strong>Eleven patients were operated upon in Cairo University Hospitals, Egypt. All operations were done using microscope through the subfrontal approach. At the end of surgery, the endoscope was used to detect any residual tumor in the subchiasmatic and retrochiasmatic areas and to visualize the posterior part of the tumor which couldn’t be seen by the microscope to check if it was adherent to the hypothalamus and to evaluate whether to be removed or not. <strong>Results:</strong> The study included eleven cases, four of which were childhood type and seven adult type craniopharyngiomas. Total removal was achieved in six cases (five cases of adulthood type). Oumaya reservoir was inserted in five cases;ventriculoperitoneal shunt was needed in five cases. All cases suffered from temporary diabetes insipidus postoperatively, while only two cases developed permanent diabetes insipidus. Three cases presented preoperatively with pituitary hypofunction and two cases developed postoperative pituitary hypofunction, which necessitated hormone replacement therapy. <strong>Conclusion: </strong>Craniopharyngioma is one of the most difficult and challenging tumors for neurosurgeons due to its relation to optic nerve, hypothalamus and vascular system formed by Willis circle and its perforating branches. Endoscopy has a role in decision making after microscopic removal of craniopharyngioma.
文摘<strong>Objective:</strong> This retrospective study aims to survey the clinical outcomes of 341 consecutive patients surgically treated for and diagnosed with craniopharyngioma (CP) treated in a 10-year period in a single institution. <strong>Methods:</strong> The clinical reports of three hundred forty-one patients CP patients treated surgically between January 2006 and December 2016 were reviewed and analyzed retrospectively. <strong>Results:</strong> Our cohort consisted of 341 patients (202 male, 139 female) with a mean age of 34.9 years (range 1 - 74 years);Tumor Features: 129 patients (37.8%) had cystic tumors, 88 (23.8%) had solid tumors whereas 126 (36.4%) had heterogeneous lesions with a solid and cystic portion;calcifications were present in 139 (40.8%);Tumor Topography: Suprasellar 198 (58.1%), Intrasellar 40 (11.7%), Intra-third ventricular 103 (30.2%);Surgical approaches used among the patients included: Pterional 262 (76.8%), Transsphenoidal (TS) 42 (12.3%) Transcallosal 20 (5.9%), Transcortical 16 (4.7%) suboccipital 1 and combined approach 1;Gross total removal (GTR) was achieved in 247 patients (72.4%), Subtotal removal (STR) in 94 patients (27.6%). Good postoperative outcome at discharge was achieved in 324 (95%) patients while 17 (5%) patients had poor outcome including 5 (1.5%) perioperative deaths. Mean hospital length of stay was 21.87 (8 - 129). There were 42 (12.9%) recurrences with a mean time to recurrence of 28.36 (3 - 84) months, among which 37 (88.1%) underwent surgery for recurrence treatment. Follow-up time ranged from 3 months to 10 years. There was a statistical significance between open transcranial surgery and suprasellar tumors (p < 0.0001), TS and intrasellar tumors (p < 0.0001);postoperative diabetes inspidus and gross total resection (p < 0.0001);GTR and cystic tumors (p = 0.034) calcification and GTR (p = 0.0008). <strong>Conclusion:</strong> Good surgical outcome and long-term tumor control can be achieved through individual-based selective resection, whether total or subtotal resection. Whereas surgical prehistory of CP was found to be a significant risk factor to recurrence (p < 0.0001), the clinical risk factors of CP of recurrence are still arguable, including the presence residual tumor due to subtotal resection. More future studies are necessary.
基金Fujian Medical University(No.XRCZX2017001 to XW)Natural Science Foundation of Fujian Province(No.2019J01294 to XW)+1 种基金Sanbo Brain Hospital Management Group(No.SBJT-KY-2020-002 to ZL)Capital Health Research and Development Special Fund(No.2022-2-8013 to ZL)
文摘Background:Adamantinomatous craniopharyngioma(ACP)is the commonest pediatric sellar tumor.No effective drug is available and interpatient heterogeneity is prominent.This study aimed to identify distinct molecular subgroups of ACP based on the multi-omics profiles,imaging findings,and histological features,in order to predict the response to anti-inflammatory treatment and immunotherapies.Methods:Totally 142 Chinese cases diagnosed with craniopharyngiomas were profiled,including 119 ACPs and 23 papillary craniopharyngiomas.Whole-exome sequencing(151 tumors,including recurrent ones),RNA sequencing(84 tumors),and DNA methylome profiling(95 tumors)were performed.Consensus clustering and non-negative matrix factorization were used for subgrouping,and Cox regression were utilized for prognostic evaluation,respectively.Results:Three distinct molecular subgroups were identified:WNT,ImA,and ImB.The WNT subgroup showed higher Wnt/β-catenin pathway activity,with a greater number of epithelial cells and more predominantly solid tumors.The ImA and ImB subgroups had activated inflammatory and interferon response pathways,with enhanced immune cell infiltration and more predominantly cystic tumors.Mitogen-activated protein kinases(MEK/MAPK)signaling was activated only in ImA samples,while IL-6 and epithelial-mesenchymal transition biomarkers were highly expressed in the ImB group,mostly consisting of children.The degree of astrogliosis was significantly elevated in the ImA group,with severe finger-like protrusions at the invasive front of the tumor.The molecular subgrouping was an independent prognostic factor,with the WNT group having longer event-free survival than ImB(Cox,P=0.04).ImA/ImB cases were more likely to respond to immune checkpoint blockade(ICB)therapy than the WNT group(P<0.01).In the preliminary screening of subtyping markers,CD38 was significantly downregulated in WNT compared with ImA and ImB(P=0.01).Conclusions:ACP comprises three molecular subtypes with distinct imaging and histological features.The prognosis of the WNT type is better than that of the ImB group,which is more likely to benefit from the ICB treatment.
基金supported by the Clinical Research Plan of SHDC(SHDC-2020CR2004A)the National Project in Promoting the Diagnosis and Treatment of Major Diseases by MDT.
文摘Background Craniopharyngioma is a common intracranial tumor located in the sellar-suprasellar region.Due to the involvement of adjacent structures,it can lead to increased intracranial pressure,visual impairment,and endocrine deficiencies.Surgical resection is the primary treatment,but it is a tough challenge to achieve total resection,which will led to the frequency of recurrences and progressions.Among them,distant spread is extremely rare,but important complication,identifying and providing proper therapy,is crucial.Methods We report two cases of ectopic recurrence craniopharyngioma and make a literature review for the published similar case reports.Results Our literature review revealed 63 cases(including our patient).The onset age in children group and adult group ranges from 2-14 years old(6.70±3.33)to 17-73 years old(40.63±15.58),while the interval year between tumor initiation and ectopic recurrence ranges from 0.17-20(7.28±6.76)years to 0.3-34(6.85±7.29).Achieving gross total resection seems not to prevent the ectopic recurrence.The major pathology of ectopic recurrence craniopharyngioma is adamantinomatous type.The most common site of ectopic recurrence is frontal lobe.According to the pathogenesis,35 cases were seeding along the surgical approach,and 28 cases were seeding via the CSF pathway.Conclusion Ectopic recurrence craniopharyngioma is rare,but it can lead to serious symptoms.Delicate surgical procedure can help to reduce the risk of ectopic recurrence,and standardized follow-up can provide valuable information for treatment.
文摘Background Generally, total surgical removal of craniopharyngioma results in satisfactory outcome with a low recurrence rate, however, the location of the tumor and its adherence to the hypothalamic structures can make the operation difficult. The goal of the present study was to assess the outcome of craniopharyngiomas in 284 patients treated surgically. Methods A total of 284 patients (151 men and 133 women) with craniopharyngioma were treated surgically by our neurosurgeons from January 1996 to March 2006. Among them, 226 (79.6%) patients were adults (15 years of age or older; mean, 35.8 ± 10.6), 58 (20.4%) were children (14 years of age or younger; mean, 9.1 ± 3.8). The diameter of the tumors were 2.0-9.0 cm (mean, 36.54± 11.4). The tumors were classified into the superior (23 patients) and inferior ventricular (261) types according to the location of the tumor relative to the third ventricular floor. For the patients with craniopharyngioma of inferior ventricular type, pterional approach was used in 191 (67.3%) patients, subfrontal approach in 17 (6.0%), and translamina terminalis through frontobasal interhemispheric approach in 53 08.7%). For those with the tumors of superior ventricular type, transcallosal approach into the anterior third ventricle was done in 10 (3.5%) patients, and the lamina terminalis approach in 13 (4.6%). Of the 284 patients, 204 (71.8%) were followed up for 0.5 to 8 years (mean, 2.1 ± 1.8), including 162 patients received total tumor removal, and 37 underwent subtotal or partial removal. Results Total, subtotal and partial removal of the tumors were achieved in 237 (83.5%), 34 (12.0%) and 13 (4.5%) patients, respectively. The pituitary stalk was preserved in 176 (62.0%) patients, severed in 52 (18.3%), and unidentified in 56 (19.7%). Twelve (4.2%) patients died within one month after the surgery. During the follow-up, 23 (14.1%) patients experienced tumor recurrence 1.0-3.5 years (mean, 1.8± 1.6) after total tumor removal, and 24 (64.9%) had recurrent tumor 0.25- 1.5 years (mean, 0.5 ± 0.4) after subtotal or partial resection. Normal activities of daily living were regained in 63 (80%) patients, independence in 29 (14.2%), and daily life with assistance in 9 (4.4%). Four (2.0%) patients died 0.9-3 years (mean, 1.6± 1.4) after discharge from hospital, 3 of them died of hypothalamic deficiency. Conclusions We can protect the hypothalamic structures and its perforating arteries by choosing surgical approaches according to the location of craniopharygioma relative to the third ventricular floor. The mortality, morbidity, and recurrence rate in patients received total resection are lower than those of patients underwent subtotal or partial resections. In addition, preservation of the pituitary stalk is critical when total tumor resection is feasible.