Urgent surgical management of congenital intracranial hemangiopericytoma in a preterm neonate.

Hemangiopericytoma is a rare mesenchymal tumor originating from capillary pericytes, known as Zimmermann pericytes. The adult form is not uncommon and generally malignant but tumor is found rarely in children. Here we describe an intracranial hemangiopericytoma in a preterm newborn whose had the tumor resected successfully shortly after birth.

The effect of polymorphisms in the promoter region of the MMP-1 gene on the occurrence and invasiveness of hypophyseal adenoma.

BACKGROUND: The matrix metalloproteinase-1 enzyme (MMP-1, also called collagenase 1) plays a key role in turnover of collagen fibers in the intercellular matrix. Insertion of a guanine residue was found within the promoter region of the MMP-1 gene. We found that MMP-1 levels increased approximately twofold over normal when this insertion was present, enabling MMP-1 to facilitate tumor invasion and metastasis. MMP-1 is also believed to play a role in tumor development. The aim of our study is to investigate the effect of polymorphisms in the promoter region of the MMP-1 gene on the development of benign and invasive hypophyseal adenomas. PATIENTS AND METHODS: Thirty patients with hypophyseal adenomas diagnosed by radiological examination underwent surgical removal, and the diagnosis was confirmed using immunohistochemical staining of the pathology specimens. We found that ten of these patients had invasive adenomas confirmed by radiological examination and immunohistochemical staining. DNA isolation was performed on all specimens, and 5-cc venous blood samples were obtained from all patients as well as 30 volunteers using the Qiagen QIAquick kit. Promoter regions of MMP-1 genes from the DNA samples were amplified using polymerase chain reaction (PCR) and primers designed for the site-directed mutation method. Following PCR, a guanine residue within the promoter region of the MMP-1 gene was identified using the restriction fragment length polymorphism method and the ALU I restriction enzyme. Three genotypes were detected in a genotyping assay: 2G/2G, 1G/2G, and 1G/1G. RESULTS: Of the surgically treated patients, 36.6% had the 2G/2G genotype, 46.6% had the 1G/2G genotype, and 16.6% had the 1G/1G genotype. The 2G allele frequency was found to be 83.4%. In 90% of cases of invasive adenoma, a homozygous 2G/2G genotype was detected. DISCUSSION: The risk for development of hypophyseal adenoma may be greater in patients with the 2G allele. In cases of existing hypophyseal adenoma, those with the homozygous 2G allele tend to be invasive.

Multimodal Intraoperative Neurophysiological Monitoring in Spinal Cord Surgery.

AIM: Intraoperative neurophysiological monitoring (IONM) monitors the functional integrity of critical neural structures by electrophysiological methods during surgery. Multimodality combines different neurophysiological methods to maximize diagnostic efficacy and provide a safety margin to improve the outcomes of spinal surgery. Our aim was to share our intraoperative monitoring experiences with patients who underwent surgery because of spinal cord pathologies between September 2013 and January 2015. MATERIAL AND METHODS: We had twenty-six cases. Location of the lesions, surgery, neurological findings, and electrophysiological findings intraoperatively and postoperatively were documented. RESULTS: The combination of motor evoked potential (MEP), somatosensorial evoked potential (SSEP), free-run and trigger electromyography (EMG) were performed according to lesion localization. MEPs plus SSEPs were run in 23 patients and MEPs with triggered EMG were performed in 4 patients. In only one patient, optimal recording could not be elicited because of technical problems. MEP and SSEP changes were recorded in 12 and 3 patients respectively. Postoperative neurological deficits were observed in 2 patients. Deficits were transient in one case and permanent in the other. While baseline MEP responses were either absent or low amplitude ( < 50 microvolt) in 7 patients, following resection they were either visible or increased in amplitude. Surgery was ended in one patient with C7-T2 intramedullary tumour after the right distal MEP response disappeared. CONCLUSION: Multimodal IONM is an important method to monitor the neural structures under risk in spine surgery and to keep the surgery within safety limits, especially for intramedullary spinal cord lesion surgery.

Immunohistochemical expression of SPARC is correlated with recurrence, survival and malignant potential in meningiomas.

Meningioma is a common neoplasm that constitutes almost 30% of all primary central nervous system tumors and is associated with inconsistent clinical outcomes. The extracellular matrix proteins play a crucial role in meningioma cell biology and are important in tumor cell invasion and in progression to malignancy. SPARC (secreted protein, acidic and rich in cysteine) (osteonectin) is a matricellular glycoprotein that regulates cell function by interacting with different extracellular matrix proteins. The aim of this study was to evaluate the expression of SPARC with proliferation index, p53 reactivity in WHO grade 1 (benign), grade 2 (atypical) and grade 3 (anaplastic) meningiomas and correlate with clinical features of the patients, including location of the tumor, recurrence of the tumor and survival of patients. We studied 111 meningiomas, 69 being benign, 34 being atypical and eight being anaplastic meningiomas of various histological types. Using immunohistochemical analysis, we evaluated the expression of SPARC, Ki-67 (MIB-1) and p53 in meningiomas. Immunohistochemical scores of SPARC were determined as the sum of frequency (0-3) and intensity (0-3) of immunolabeling of the tumor cells. A high immunohistochemical score (4-6) for SPARC was more frequent in atypical and in anaplastic meningiomas than in benign meningiomas (p < 0.01). MIB-1 proliferation index showed significant association between tumor grades in meningiomas (p < 0.01). At the end of a follow-up period of 47.53 +/- 25.04 months, 30 tumors recurred. A high SPARC expression was significantly associated with tumor recurrence (p = 0.02). The immunoreactivity of p53 protein and MIB-1 score were significantly higher in recurrent meningiomas than in non-recurrent meningiomas. The cumulative survival of patients with high SPARC expression was significantly lower than patients with low SPARC expression. The high SPARC expression scores were predominantly identified in meningothelial, fibrous and chordoid meningiomas; low SPARC expression scores were mostly spotted in secretory and psammomatous meningiomas. Evaluating SPARC expression might help assessing recurrence risk and survival estimation in meningiomas.