Primary pituitary neuroendocrine tumor: Case report and literature review.

BACKGROUND: Neuroendocrine tumors (NET) originate from the diffuse neuroendocrine system. These can arise in almost every organ of the body, although they are most commonly found in the gastrointestinal tract and respiratory system. The skull base and sellar region are extremely rare sites for neuroendocrine carcinoma. Consequently, in this case, both diagnosis and definition of surgical goals, as well as further treatment strategies were challenging. CASE DESCRIPTION: A 65-year-old woman was admitted to our Neurosurgery Department with a rapidly progressive visus reduction, drowsiness, polyuria, and polydipsia. Neuroimaging showed a sellar/suprasellar mass (diameter of 2 cm) with a heterogeneous signal compressing the optic chiasm and extending laterally toward the cavernous sinus. Differential diagnosis based on imaging included pituitary macroadenoma or metastasis. The patient underwent endoscopic endonasal transsphenoidal surgery. A total resection of the mass was impossible because of the infiltration of the optic chiasm and the intraoperative histological diagnosis of malignant epithelial neoplasm. Further histological evaluation revealed that the lesion was a NET with no other primary or metastatic sites detectable. Subsequently, the patient was successfully treated with fractioned stereotactic radiotherapy and polychemotherapy. Four years after the surgery, follow-up magnetic resonance imaging showed stability of the residual disease. Neurologic examination revealed a complete visual recovery. CONCLUSIONS: Primary pituitary NET, though rare, should be included in the differential diagnosis of sellar lesions. A multimodality treatment approach is needed. Finally, the present case highlights, that in the case of a pituitary lesion infiltrating the optic chiasm, including NET, the endoscopic endonasal transsphenoidal subtotal resection followed by fractioned stereotactic radiotherapy and chemotherapy may represent an effective and safe choice of treatment.

Analysis of MGMT promoter methylation status on intraoperative fresh tissue section from frameless neuronavigation needle biopsy of 25 patients with brain tumor.

Formalin fixation under conditions that adversely affected the quality of the DNA, or indeterminant assay, or extensive tumor necrosis can compromise the genetic analysis of a brain bioptic sample. The success of DNA extraction and Methyl Guanine Methyl Transferase (MGMT) promoter methylation testing could be improved by freezing of fresh tumor tissue at the moment of biopsy. To ensure an increased concentration of the DNA samples the withdrawal should be performed in an area with high probability of neoplastic cells. From May 2007 to January 2011 fifty-two frameless neuronavigation brain needle biopsy were performed at the Neurosurgery Unit of the "Arcispedale Santa Maria Nuova" City Hospital of Reggio Emilia. The "image-guided" neuronavigated protocol sampling provided withdrawal specimens highly correlated with neuroimaging characteristics of the lesions. In this study the Authors report the genetic analysis on 24 cases of freezing fresh tissue from brain needle bioptic sample starting from July 2008. The molecular determination of MGMT promoter was assessed with the Nested-Methylation Specific-Polymerase Chain Reaction on fresh or cryopreserved needle bioptic tissue. The genetic characterization was feasible in all the bioptic samples. The MGMT promoter was methylated in eleven patients, including a brain infection. The diagnostic yield of brain biopsy could be increased by the neuronavigated trajectories and the intraoperative frozen sections. In the future the availability of the molecular-genetic characterization of a brain tumor before open surgery will provide important information for the optimal treatment. The MGMT promoter status analysis on needle bioptic fresh tissue could be available also for that patient not eligible for surgical remotion of the tumor.

Detection of IDH1 mutations and the status of MGMT promoter on intraoperative fresh tissue section from frameless neuronavigation needle biopsy. Analysis on 17 patients with brain glial tumor ineligible for craniotomy and tumor resection.

It is well known that primary and secondary glioblastomas are histologically largely indistinguishable. Therefore, the detection of IDH1 mutations or the status of the MGMT promoter on a simple bioptic sample could be one of major diagnostic and prognostic importance for glial patients that complements clinical criteria for distinguishing secondary from primary glioblastomas and to predict a more favourable prognosis. Currently, biopsy is the method of choice to obtain tissue from intracranial lesions with uncertain neurodiagnostic findings or in deep locations, with a minimal invasive approach. The needle biopsy with frameless neuronavigation could provide a sampling with elevated diagnostic yield and high concentration of DNA, due to the "image-guided" computer assisted technique of needle insertion through the most neurodiagnostic representative tumor area. The freezing of fresh tumor tissue at biopsy could greatly improve the success of DNA extraction. The concentration of the DNA samples can also improved from a withdrawal in an area with high probability of neoplastic cells. The present study reports the results of 17 patients who had undergone frameless image-guided intracranial needle biopsy from April 2008 until July 2010 at Neurosurgery Unit of the "Arcispedale Santa Maria Nuova" of Reggio Emilia. For these patients the molecular determination of MGMT promoter was assessed with the Nested-Methylation Specific-Polymerase Chain Reaction and the screening of mutations in IDH1 e IDH2 genes was performer by polymerase chain reaction (PCR) and direct sequencing on fresh or cryopreserved needle bioptic tissue.

Spinal dural arteriovenous fistulas: the use of intraoperative microvascular Doppler monitoring.

Spinal dural arteriovenous fistulas are characterized by an abnormal communication between the dural branch of the radiculomedullary artery and an intradural medullary vein. Although the optimal treatment strategy is still debated, a complete interruption of the flow in the fistulas should be obtained. The authors report four cases operated on with intraoperative microvascular Doppler monitoring assistance. In all cases, microDoppler confirmed the location of the fistula and revealed an arterial spectrum on the redundant dorsal medullary veins. After the clipping of the feeder of the arteriovenous shunt, the intraoperative monitoring documented a complete disappearance of the arterial spectrum and the reappearance of the venous pattern. The ultrasonographic changes suggested the complete interruption of the fistulas. Postoperative angiography showed no residual abnormality in all patients. Doppler monitoring during surgery confirms satisfactory interruption of the arterial feeder and may prove useful where initial identification of the feeding vessel is difficult.

Intrinsic III ventricle craniopharyngioma.

A case of intrinsic III ventricle craniopharyngioma in an 11-year-old girl is presented. Only five cases in children have previously been reported in detail. The intraventricular location of a craniopharyngioma originates from ectopic remnants and causes intracranial hypertension without hormonal or neurological disorders. The tumor was safely removed by the transcallosal approach, and we confirmed that the floor of the III ventricle was intact.

Asymptomatic extradural haematomas. Results of a multicenter study of 158 cases in minor head injury.

The authors report a study conducted in three Italian neurosurgical centres on 158 patients admitted after a minor head injury and with CT findings of a hitherto asymptomatic significant extradural haematoma. All patients were examined both prospectively by means of a computerized record containing 18 clinical and radiological parameters, and retrospectively by logistical regression analysis, in order to ascertain which factors influenced most the choice of surgical vs. conservative management. The size of the haematoma, rather than its location, and the degree of midline shift were the factors most influential in deciding in favour of surgical treatment, with a specificity of 0.83 and a sensitivity of 0.92. Conservative management of haematomas having a maximum thickness of less than 10 mm with a midline shift of less than 5 mm appears as safe. Outcome was "good recovery" in both the surgical and the nonsurgical patients, with only one death in the whole series, unrelated to the extradural lesion. This study focuses attention on a group of patients who are seldom examined by CT scan, but who can harbour potentially lethal lesions. Extension of CT scan examination to all adult patients with a minor head injury and a skull fracture can be recommended in order to identify significant haematomas in an asymptomatic phase.