Clinical and Pathological Features of Pit1/SF1 Multilineage Pituitary Neuroendocrine Tumor.

BACKGROUND AND OBJECTIVES: Lineage-based classification has critical clinical implications in pituitary neuroendocrine tumor (PitNET). As the most prevalent subtype of multilineage PitNET, PitNET originating from both pituitary-specific positive transcription factor 1 (Pit1) and steroidogenic factor-1 (SF1) lineages (Pit1/SF1-adenoma) is expected to exhibit rich and varied clinical behaviors. A comprehensive understanding of the clinical and pathological characteristics of Pit1/SF1-adenoma will provide mechanistic insight and influence the prognosis and treatment of PitNET. METHODS: A retrospective study was conducted by reviewing 57 cases of Pit1/SF1-adenoma between 2018 and 2022. We also included 88 cases of PitNET arising from Pit1 cell lineage (Pit1-adenoma) and 70 cases of PitNET arising from SF1 cell lineage (SF1-adenoma) as controls. Comprehensive data, including demographic, symptom, endocrinal, radiological, surgical, pathological, and prognostic information, were systematically collected. All specimens were immunostained for pituitary transcription factors (PTFs) and pituitary hormones. RESULTS: The detection rate was 8.0% for Pit1/SF1-adenoma within PitNET surgical specimens. Pit1/SF1-adenoma displayed a male predominance, with the mean diagnosis age falling between Pit1-adenoma and SF1-adenoma. The endocrine activity of Pit1/SF1-adenoma was lower than Pit1-adenoma but higher than SF1-adenoma. Pit1/SF1-adenoma had a higher incidence of cavernous sinus invasion (56.1%) than both Pit1-adenoma (38.6%, P = .039) and SF1-adenoma (27.1%, P = .001). Furthermore, Pit1/SF1-adenoma showed more postoperative complications than Pit1-adenoma (29.8% vs 8.0%, P = .001). Nonfunctional Pit1/SF1-adenoma had a higher radiological tumor recurrence rate than nonfunctional SF1-adenoma (34.8% vs 10.9%, P = .021). Notably, the immunostaining pattern was diverse in Pit1/SF1-adenoma, with various combinations of staining intensity for PTFs and 15 combinations for 6 pituitary hormones. Intriguingly, various PTFs combinations had no different impact on the outcome of Pit1/SF1-adenoma. CONCLUSION: Pit1/SF1-adenoma represents a unique pathological subtype of PitNET, characterized by distinctive clinical behaviors. Identifying Pit1/SF1-adenoma can facilitate more precise management of PitNET by the practical use of Pit1/SF1 immunostaining.

Internal carotid artery injury during endoscopic transsphenoidal pituitary surgery: risk factors, management.

BACKGROUND: Transsphenoidal surgeons should try to avoid internal carotid artery (ICA) injury but also be prepared to manage it. We analyzed our experience with ICA injury during endoscopic transsphenoidal pituitary surgery and present associated risk factors and a management protocol. METHODS: We retrospectively reviewed and analyzed the medical records of 1596 patients who underwent endoscopic transsphenoidal surgery for pituitary tumor resection in our institution from January 2009 to October 2022. RESULTS: Six patients experienced an ICA injury. All received timely and effective hemostasis with immediate direct tamponade followed by endovascular treatment. No serious postoperative complications occurred. CONCLUSIONS: We proposed a treatment plan for ICA injuries encountered during endoscopic transsphenoidal surgery and described our hemostasis process, methods of endovascular treatment, and means of postoperative follow-up in detail.

An endoscopic transnasal prelacrimal recess transmaxillary approach to the pterygopalatine fossa and infratemporal fossa.

Objective: In this paper, the goal of the authors is to present the anatomic nuances and their clinical experience with lesions of the pterygopalatine fossa and infratemporal fossa using an endoscopic transnasal prelacrimal recess transmaxillary approach (PLRMA). Methods: An endoscopic anatomical dissection of three fresh cadaveric heads was performed bilaterally to evaluate the feasibility of the PLRMA. Prior to dissection, stereotactic computed tomography scans were obtained for each head to obtain anatomical measurements. The area of exposure on the posterior wall of the maxillary sinus was determined using stereotaxis. The cases of six patients with schwannomas or epidermoid cysts who underwent the transnasal PLRMA were illustrated. Results: The mean area of exposure on the posterior wall of the maxillary sinus was 9.55 cm2. Total resection was achieved in all six patients. The mean follow-up time was 16 months, and one patient complained of postoperative facial numbness, which resolved gradually. No cases of chronic sinusitis were reported. Conclusions: The endoscopic transnasal PLRMA provides efficient operative exposure to the pterygopalatine fossa and infratemporal fossa. Preserving the integrity of the mucosa on the nasal lateral wall is an advantage of this approach.

Cerebrospinal fluid dynamics along the optic nerve in patients with spontaneous cerebrospinal fluid rhinorrhea: a retrospective computed tomographic cisternography study.

PURPOSE: Spontaneous cerebrospinal fluid (CSF) rhinorrhea is related to intracranial pressure (ICP) and dynamic changes. This study investigated CSF dynamics along the optic nerve (ON) in patients with spontaneous CSF rhinorrhea. METHODS: The computed tomographic (CT) cisternographies of 66 patients (132 eyes) with spontaneous CSF rhinorrhea were analysed. The contrast-loaded CSF (CLCSF) density was measured in Hounsfield units (HU) at three regions of interest (ROIs) along the ON and adjusted by the basal cistern density. The CLCSF density and ON sheath diameter (ONSD) were analysed between both sides in the different ICP groups. RESULTS: When comparing the density of CLCSF along the ON, no significant differences were found between the ipsilateral and contralateral sides of the leakage. The distribution of CLCSF along the ON showed a highly significant density reduction from the canalicular segment to the bulbar segment on both sides. The CLCSF density significantly decreased on the ipsilateral ON in the canalicular segment and tended to decrease on the ipsilateral ON in the bulbar and canal segments compared with that on the contralateral ON in the low-ICP group. The ONSD tended to decrease on the ipsilateral side of leakage. CONCLUSIONS: According to the CLCSF density on CT cisternography, CSF dynamics along the ON may bilaterally decrease from the optic canal to the retrobulbar segment. Cerebrospinal fluid (CSF) dynamics are possibly influenced by differences in ICP, and a lower ICP may cause more obvious differences or impairments in CSF dynamics along the ipsilateral ON.

Continuous dural suturing for closure of grade 3 leaks after tumor removal via an endoscopic endonasal approach.

Cerebrospinal fluid (CSF) leakage is a major complication after extended endonasal transsphenoidal surgery (EETSS), which is commonly used in the treatment of anterior skull base tumors. Dural suturing and graded reconstruction are promising techniques to further decrease the incidence of postoperative CSF (poCSF) leakage. The effect of continuous dural suturing in endoscopic surgery was investigated in this retrospective study. A total of 79 EETSS patients were included; the procedures were performed for subdural tumor removal by a single endoscopic neurosurgical team. Comparisons were applied between patients who did and did not undergo endoscopic dural suturing after tumor removal. Multivariate logistic regression analysis was performed to identify variables that significantly influenced the incidence of poCSF leakage. In all, 79 adult patients developed Esposito's grade 3 intraoperative high-flow CSF leakage. Ten patients (12.7%) experienced poCSF leakage. One of the 36 patients who underwent intraoperative dural suturing developed poCSF leakage, compared with nine of 43 patients who did not undergo dural suturing (p = 0.016). Regression analysis showed that dural suturing could significantly decrease the incidence of poCSF leakage (p = 0.049, OR 0.108, 95% CI 0.013-0.899). Prophylactic lumbar drainage could also help decrease the CSF leakage rate. Dural suturing under endoscopy is a promising and effective method for application in skull base reconstruction after subdural skull base tumor removal. With future progress, lumbar drainage and even nasoseptal flap placement could be replaced in certain groups of patients undergoing EETSS.

Risk factors and outcomes of cerebrospinal fluid leak related to endoscopic pituitary adenoma surgery.

Objective: The incidence of and risk factors for intra- and postoperative cerebrospinal fluid (poCSF) leak in patients who underwent endoscopic surgery for pituitary adenoma were investigated in this retrospective study.Methods: A total of 216 consecutive patients operated on by a single neurosurgical team were included. Logistic regression was applied to identify risk factors for intraoperative CSF (ioCSF) and poCSF leaks, and the outcome and management of ioCSF and poCSF leaks were analyzed.Results: Sixty-five patients (30.1%) experienced ioCSF leak, of whom 10 developed poCSF leak. Three of 151 patients developed poCSF leak without obvious ioCSF leak. Multiple regression analysis revealed that tumor with lobular or irregular contour and gonadotrophic-positive staining increased the risk of ioCSF leak; additionally, nonfunctional tumor (p = 0.058) and preoperative gonadotrophic hormone abnormalities (p = 0.08) tended to increase this risk. The presence of ioCSF leak and preoperative visual impairment were predictors for poCSF leak. Tumor with Knosp grades 3-4 and a higher grade of ioCSF leak could increase the risk of poCSF leak. Tailored pedicled vascularized nasoseptal flaps (NSFs) preparation before tumor removal greatly decreased the rate of poCSF leak with ioCSF leak and the overall leak rate. Three prophylactic lumbar drains (LDs) were performed in patients with grade 3 ioCSF leak, none of whom developed poCSF leak.Conclusions: Lobular or irregular tumor contour and gonadotrophic-positive staining were associated with a high risk of ioCSF leak, while ioCSF leak and preoperative visual impairment were associated with poCSF leak. NSF preparation, preventive sellar reconstruction and LD use could likely decrease the rate of poCSF leak.

Evolution of transmaxillary approach to tumors in pterygopalatine fossa and infratemporal fossa: anatomic simulation and clinical practice.

BACKGROUND: The endoscopic transnasal approach has been proven to have advantages on the removal of the tumors in pterygopalatine fossa (PPF) and infratemporal fossa (ITF). Herein, this study aimed to describe a modified approach for resection of the tumors in these areas, both in cadaveric specimen and clinical patients. METHODS: The 20 adult cadaveric specimens and five patients with tumors in PPF and ITF were enrolled in this study. For the cadaveric specimens, ten were simulated anterior transmaxillary approach and ten were performed modified endoscopic transnasal transmaxillary approach. The exposure areas were compared between two groups and main anatomic structure were measured. Surgery was operated in the five patients with tumors of PPF and ITF to verify the experience from the anatomy. Perioperative management, intraoperative findings and postoperative complications were recorded and analyzed. RESULTS: The modified endoscopic transnasal transmaxillary approach provided as enough surgical exposure and high operability to the PPF and ITF as the anterior transmaxillary approach did. The diameter of maxillary artery in the PPF was 3.77 ±â€Š0.78 mm (range: 2.06-4.82 mm), the diameter of middle meningeal artery in the ITF was 2.79 ±â€Š0.61 mm (range: 1.54-3.78 mm). Four patients who suffered schwannoma got total removal and one of adenocystic carcinoma got subtotal removal. The main complications were facial numbness and pericoronitis of the wisdom tooth. No permanent complication was found. CONCLUSIONS: With the widespread use of neuroendoscopy, the modified endoscopic transnasal transmaxillary approach is feasible and effective for the resection of tumors located in PPF and ITF, which has significant advantages on less trauma and complications to the patients.

Methylation of Werner syndrome protein is associated with the occurrence and development of invasive meningioma via the regulation of Myc and p53 expression.

The aim of the present study was to investigate the positive rate of Werner syndrome protein (WRN) methylation in meningioma patients, and further assess the association between WRN methylation and the occurrence of meningioma. A total of 56 consecutive meningioma patients and 26 healthy individuals were enrolled in the study. A methylation-specific polymerase chain reaction assay was performed to detect the positive rate of WRN methylation in the peripheral blood and tissue samples collected from the recruited subjects. In addition, western blot analysis was performed to determine the protein expression levels of WRN, Myc and p53 in the peripheral blood and tissue samples. The positive rate of WRN methylation in the peripheral blood of the meningioma group was increased when compared with the control group (P<0.05). In addition, the protein expression levels of WRN were significantly decreased in the peripheral blood and tissue samples collected from the individuals with a positive WRN methylation status (P<0.05), as compared with the samples without WRN methylation. Furthermore, the protein expression levels of Myc and p53 were increased in the peripheral blood and tissue samples that exhibited positive WRN methylation when compared with those without WRN methylation (P<0.05). Therefore, WRN methylation was demonstrated to be associated with the occurrence and development of invasive meningioma, possibly through the regulation of Myc and p53 expression.

Third ventricular meningiomas.

We report 13 patients with third ventricular meningiomas (TVM) and discuss the clinical, radiological, pathological and surgical features, as well as follow-up of these tumors. TVM are rare intracranial tumors, and because of this, there are few reports in the literature. Of 11,600 intracranial meningiomas that were surgically treated and pathologically confirmed at Beijing Tian Tan Hospital over a period of 10 years (2003-2013), 13 TVM were selected for a retrospective review. We recorded the clinical, radiological, pathological, and surgical data and statistically analyzed the preoperative, postoperative and 6 month postoperative Karnofsky performance scale (KPS) scores. TVM represented 0.11% of intracranial meningiomas. Radiologically, TVM were divided into three groups: anterior (n=3), posterior (n=3), and entire third ventricle (n=7). Three patients (23.1%) were misdiagnosed preoperatively. Total removal was achieved in 61.5% (8/13) of patients, and subtotal resection was achieved in 38.5% (5/13). Pathologically, the tumors were World Health Organization (WHO) Grade I in 11 patients (84.6%) and WHO Grade II in two (15.6%). There were no statistically significant differences in the preoperative, postoperative, or 6 month postoperative KPS scores (F=0.814; p=0.401). TVM without dural attachments are rare neoplasms that should be differentiated from choroid plexus papilloma, craniopharyngioma, and pineocytoma. Surgery is the optimal treatment and may result in a favorable prognosis, and understanding of the radiological subtype can help with the choice of surgical approach.

Multicystic vestibular schwannomas with fluid-fluid levels: A report of three cases.

The present study reported three rare cases of multicystic vestibular schwannoma with fluid-fluid levels. These three tumors exhibited apparent fluid-fluid levels that demonstrated a radiological appearance of two fluid levels in the cystic area. Two cases had an acute history, whereas one case presented with a chronic progression. A suboccipital retrosigmoid approach was used for access to the tumors and gross total resection was achieved in all the cases. During the surgical procedures, unclotted blood was observed, and the tumors were adhered to the facial nerve, other cranial nerves or the brain stem. In each case, the facial nerve function of the patients was impaired following surgery. The present study discussed the possible mechanism of the formation of the fluid-fluid levels, the effect of the fluid-fluid levels and the therapeutic strategy employed.

Clinical, radiological, and pathological features in 43 cases of intracranial subependymoma.

OBJECT: Intracranial subependymomas are rarely reported due to their extremely low incidence. Knowledge about subependymomas is therefore poor. This study aimed to analyze the incidence and clinical, radiological, and pathological features of intracranial subependymomas. METHODS: Approximately 60,000 intracranial tumors were surgically treated at Beijing Tiantan Hospital between 2003 and 2013. The authors identified all cases in which patients underwent resection of an intracranial tumor that was found to be pathological examination demonstrated to be subependymoma and analyzed the data from these cases. RESULTS: Forty-three cases of pathologically confirmed, surgically treated intracranial subependymoma were identified. Thus in this patient population, subependymomas accounted for approximately 0.07% of intracranial tumors (43 of an estimated 60,000). Radiologically, 79.1% (34/43) of intracranial subependymomas were misdiagnosed as other diseases. Pathologically, 34 were confirmed as pure subependymomas, 8 were mixed with ependymoma, and 1 was mixed with astrocytoma. Thirty-five patients were followed up for 3.0 to 120 months after surgery. Three of these patients experienced tumor recurrence, and one died of tumor recurrence. Univariate analysis revealed that shorter progression-free survival (PFS) was significantly associated with poorly defined borders. The association between shorter PFS and age < 14 years was almost significant (p = 0.51), and this variable was also included in the multivariate analysis. However, multivariate analysis showed showed only poorly defined borders to be an independent prognostic factor for shorter PFS (RR 18.655, 95% CI 1.141-304.884, p = 0.040). In patients 14 years of age or older, the lesions tended to be pure subependymomas located in the unilateral supratentorial area, total removal tended to be easier, and PFS tended to be longer. In comparison, in younger patients subependymomas tended to be mixed tumors involving the bilateral infratentorial area, with a lower total removal rate and shorter PFS. CONCLUSIONS: Intracranial subependymoma is a rare benign intracranial tumor with definite radiological features. Long-term survival can be expected, although poorly defined borders are an independent predictor of shorter PFS. All the features that differ between tumors in younger and older patients suggest that they might have different origins, biological behaviors, and prognoses.

[Eight cases of cavernous hemangioma of the optic chiasm].

OBJECTIVE: To report the clinical characteristics, neuto-imaging and prognosis of cavernous hemangioma of the optic chiasm. METHODS: Retrospective data analysis was conducted in 8 patients from Beijing Tongren Hospital and Beijing Tiantan Hospital. Patients' clinical characteristics, neuroimaging and prognosis were assessed. RESULTS: There were eight patients aging from 16-58 years old including 3 females and 5 males. The median age was 39.5 years. From the time of onset to diagnosis, the shortest time was 25 days and the longest was 2 years and 4 months. Clinical characteristics of 8 cases were analyzed. Among 8 cases, 3 patients had acute course, 3 patient had chronic course with acute exacerbation and 2 patients had chronic course. All patients presented with decreased visual acuity and/or visual field defect and 3 cases had headache. The most common initial manifestation was decreased visual acuity and/or visual field defect (4 cases). Only 4 cases were diagnosed cavernous hemangioma of the optic chiasm before surgery. The hemangioma was total resected in 6 patients and other two got partly removed. Visual function was improved in 4 cases after surgery. MRI showed mixed signal. CONCLUSIONS: All patients presented decreased visual acuity and/or visual field defect, often accompanied with headache. It can be misdiagnosed at early stage. MRI may be helpful to the diagnosis. Complete resection at early stage may improve the visual function.

[Perspective research of the influence of caloric restriction combined with psychotherapy and chemotherapy associated by hybaroxia on the prognosis of patients suffered by glioblastoma multiforme].

OBJECTIVE: To explore the influence of caloric restriction combined with psychotherapy and chemotherapy associated by hybaroxia on the prognosis of patients with intracranial glioblastoma multiforme. METHODS: This was a perspective, nonrandom, no-double-blinded controlled study. All patients underwent total resection during November 2007 to April 2009 at Beijing Tiantan Hospital and Beijing Tiantan Puhua Hospital. All diagnoses were confirmed by molecule pathology. While 23 patients in control group underwent resections and radiochemotherapy, 11 patients in experimental group were further treated by caloric restriction plus psychotherapy and chemotherapy-associated hybaroxia. The life spans were compared between two groups. RESULTS: The mean survival time of patients in experimental group was (38 ± 13) months versus (20 ± 12) months in control group. The survival time of patients in experimental group was significantly longer than that in control group (P < 0.05). CONCLUSION: Caloric restriction plus psychotherapy and chemotherapy-associated hybaroxia can apparently prolong the life span of patients with glioblastoma multiforme.

Clinical, radiological, and pathological features of 24 atypical intracranial epidermoid cysts.

OBJECT: Most intracranial epidermoid cysts typically present with long T1 and T2 signals on MR images. Other epidermoid cysts with atypical MR images are often misdiagnosed as other diseases. In this study the authors aimed to analyze the incidence and the clinical, radiological, and pathological features of atypical epidermoid cysts. METHODS: Among 428 cases of intracranial epidermoid cysts that were surgically treated between 2002 and 2008 at Beijing Tiantan Hospital, cases with an atypical MR imaging appearance were chosen for analysis. Clinical and pathological parameters were recorded and compared in patients with lesions demonstrating typical and atypical MR appearance. RESULTS: An atypical epidermoid cyst accounts for 5.6% of the whole series. Radiologically, 58.3% of atypical epidermoids were misdiagnosed as other diseases. Compared with a typical epidermoid cyst, atypical epidermoid lesions were significantly larger (p = 0.016, chi-square test). Pathologically, hemorrhage was found in 21 patients with atypical epidermoid cyst and is significantly correlated with granulation (p = 0.010, Fisher exact test). Old hemorrhage was found in 13 cases and was significantly correlated with cholesterol crystals. Twenty-one patients were followed up for 1.3-8.6 years after surgery. The 5- and 8-year survival rates were both 100%. Three patients experienced cyst recurrence. The 5- and 8-year recurrence-free rates were 95% and 81.4%, respectively. CONCLUSIONS: Radiologically, an atypical epidermoid cyst should be differentiated from dermoid cyst, teratoma, schwannoma, glioma, craniopharyngioma, and cavernous angioma. A tendency toward spontaneous hemorrhage is confirmed in atypical epidermoid cysts, and a hypothesis was proposed for spontaneously intracystic hemorrhage in atypical epidermoid cysts. Follow-up confirmed long-term survival of patients with atypical epidermoid cysts.