1/38. Solitary fibrous tumor of the meninges: two new cases and review of the literature.BACKGROUND: Solitary fibrous tumor (SFT), a mesenchymal neoplasm originally described in the pleura has been more recently reported to arise in a number of other sites, including the meninges. Nowadays immunohistochemistry facilitates the otherwise problematic differential diagnosis with regard to other benign and malignant spindle cell neoplasms of the central nervous system. methods: Two recently treated cases of meningeal SFT (one craniospinal, one spinal) are presented and discussed in the light of the present knowledge and a review of the literature. RESULTS: Total resection was followed by complete recovery and both patients are presently asymptomatic and without evidence of disease. The microscopic and immunohistochemical profiles (CD 34, vimentin positive; S-100, EMA negative) were consistent with those of previously reported cases. CONCLUSIONS: The majority of SFTs behave in a benign fashion and do not recur unless subtotally resected. Malignant variants may account for up to 37% of SFTs in other locations but have never been reported to occur in the meninges. Meningeal SFTs are to be considered a new pathological entity. Wider use of immunohistochemical screening should enable the determination of their real incidence; larger series and longer follow-up will provide conclusions about their treatment and prognosis.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/38. Craniofacial hemangiopericytoma associated with oncogenic osteomalacia: case report.A craniofacial hemangiopericytoma associated with oncogenic osteomalacia is described and the literature is reviewed. A 46 year-old male with multiple fractures and hypophosphatemia was found to have a craniofacial mass extending from the right ethmoid sinus into the right frontal lobe. Initial detection of the tumor was made with an 111Indium-pentreotide scan (Octreoscan). Gross total resection of the tumor was achieved and the patient received postoperative radiation therapy. One year after surgery, the patient remains free of tumor with significant increase in bone density and normal phosphate levels. This is the first report of a hemangiopericytoma invading the brain that was associated with paraneoplastic hypophosphatemia and osteomalacia. Also, this is the first reported detection of a hemangiopericytoma by an Octreoscan. Primary detection and secondary surveillance of hemangiopericytomas may be possible with serial Octreoscans.- - - - - - - - - - ranking = 0.031026769411858keywords = brain (Clic here for more details about this article) |
3/38. MEPE, a new gene expressed in bone marrow and tumors causing osteomalacia.Oncogenic hypophosphatemic osteomalacia (OHO) is characterized by a renal phosphate leak, hypophosphatemia, low-serum calcitriol (1,25-vitamin-D3), and abnormalities in skeletal mineralization. Resection of OHO tumors results in remission of the symptoms, and there is evidence that a circulating phosphaturic factor plays a role in the bone disease. This paper describes the characterization and cloning of a gene that is a candidate for the tumor-secreted phosphaturic factor. This new gene has been named MEPE (matrix extracellular phosphoglycoprotein) and has major similarities to a group of bone-tooth mineral matrix phospho-glycoproteins (osteopontin (OPN; HGMW-approved symbol SPP1), dentin sialo phosphoprotein (DSPP), dentin matrix protein 1 (DMP1), bone sialoprotein II (IBSP), and bone morphogenetic proteins (BMP). All the proteins including MEPE contain RGD sequence motifs that are proposed to be essential for integrin-receptor interactions. Of further interest is the finding that MEPE, OPN, DSPP, DMP1, IBSP, and BMP3 all map to a defined region in chromosome 4q. Refined mapping localizes MEPE to 4q21.1 between ESTs D4S2785 (WI-6336) and D4S2844 (WI-3770). MEPE is 525 residues in length with a short N-terminal signal peptide. High-level expression of MEPE mRNA occurred in all four OHO tumors screened. Three of 11 non-OHO tumors screened contained trace levels of MEPE expression (detected only after RT-PCR and Southern 32P analysis). Normal tissue expression was found in bone marrow and brain with very-low-level expression found in lung, kidney, and human placenta. Evidence is also presented for the tumor secretion of clusterin (HGMW-approved symbol CLU) and its possible role as a cytotoxic factor in one of the OHO patients described.- - - - - - - - - - ranking = 0.031026769411858keywords = brain (Clic here for more details about this article) |
4/38. Haemangiopericytoma of the trigeminal nerve.A 41-year-old man presented with a 4-year history of progressive right-sided diplopia on lateral gaze and right nasolabial paraesthesia. A CT revealed minor bone erosion of Meckel's cave and of the right petrous apex by a uniformly enhancing lesion at the base of the skull. magnetic resonance imaging on three occasions over 2 years showed tumour, measuring 4 cm in diameter, with features suggestive of a trigeminal neuroma. At surgery the lesion had the macroscopic appearance of a giant schwannoma. Histopathological findings were that of a meningeal haemangiopericytoma (HPC) of the trigeminal nerve. Intracranial HPC are rare and aggressive tumours of the central nervous system. They usually arise from the falx, tentorium and dural sinuses. The present case is unique as it originates from a cranial nerve.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/38. Four cases of meningeal hemangiopericytoma treated with surgery and radiotherapy.We report our experiences of four cases with meningeal hemangiopericytoma treated with surgery and postoperative radiotherapy and survey the literature to elucidate the efficacy of radiotherapy. patients were treated with surgical resection and 46-52 Gy postoperative radiotherapy. Three patients had local control for 30, 54 and 138 months, respectively and one patient had local recurrence after 49 months. Distant metastases were observed in two patients; one had multiple bone, liver and lung metastases and the other multiple bone and brain metastases. For bone and brain metastases, better tumor control was obtained with palliative radiotherapy and stereotactic radiotherapy. literature analyses demonstrated that surgery and postoperative radiotherapy of 50 Gy or more resulted in significantly better local control than surgery alone (p = 0.02). Stereotactic radiosurgery was effective for intracranial recurrence or metastasis, especially when the tumor volume was <8 cm(3) and >15 Gy at the 50% isodose line was used. radiotherapy for bone metastases was also effective for palliation.- - - - - - - - - - ranking = 0.062053538823716keywords = brain (Clic here for more details about this article) |
6/38. Intradural hemangiopericytoma of the lumbar spine: case report.OBJECTIVE AND IMPORTANCE: hemangiopericytoma is a rare tumor of the central nervous system, most often found supratentorially. Thirty-nine cases within the spinal column, of which five were intradural, have been reported. To date, no magnetic resonance imaging descriptions of intradural hemangiopericytomas have been published. This article is the first report of an intradural hemangiopericytoma of the lumbar spine and the first magnetic resonance imaging description of such a lesion. CLINICAL PRESENTATION: A 31-year-old man presented with progressive bilateral leg paresthesia and increased lower extremity cramping and fatigue during a period of several months. This progressed to urinary urgency, frequency, and sexual dysfunction. A neurological examination revealed no motor or sensory deficits. gadolinium-enhanced magnetic resonance imaging of the lumbar spine revealed a centrally located intradural mass posterior to the L4 vertebral body. TECHNIQUE: The patient underwent a laminectomy of L4 and partial laminectomy of L3 with complete en bloc resection of the tumor. A discrete, intradural, red-appearing lesion was found and resected en bloc. Pathological findings were consistent with hemangiopericytoma. CONCLUSION: Intradural hemangiopericytomas, although rare, cannot be differentiated from other, more benign tumors. Spinal hemangiopericytomas ideally should be resected en bloc to reduce operative blood loss and potentially increase disease-free survival time. Despite total surgical resection of these benign-seeming lesions, the high recurrence rate mandates close follow-up and consideration of adjuvant therapy.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/38. Intracranial hemangiopericytoma with extracranial metastasis occurring after 22 years.A 65-year-old man developed pancreatic and pubic tumors 22 years after craniotomy for a left sphenoid ridge tumor. The histological finding of the primary sphenoid ridge tumor was meningotheliomatous meningioma. The histological appearance of the biopsy specimen of the pubic tumor was hemangiopericytoma. The recurrent intracranial tumor was treated by radiosurgery, and the metastatic tumors were treated by conventional irradiation. Intracranial tumors rarely metastasize outside the central nervous system, except for meningeal hemangiopericytomas. This case indicates that meningeal hemangiopericytoma may metastasize many years after the initial onset and requires long-term follow up.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/38. hemangiopericytoma in the setting of acromegaly.acromegaly is associated with an increased incidence of neoplasia thought to be related in part to tonic increases in circulating levels of insulin-like growth factor-1 (IGF-1). Hemangiopericytomas, particularly those occurring in soft tissues, are known to possess IGF receptors. These tumors often behave aggressively and can be recalcitrant to surgery and radiation therapy. A 49-yr-old man presented with an erosive, midline scalp mass and cutis gyrata. Further diagnostic imaging and endocrinologic analysis confirmed two diagnoses: a hemangiopericytoma and acromegaly associated with an intrasellar pituitary tumor. Both the hemangiopericytoma and the pituitary adenoma were surgically resected; Western blot analysis confirmed the presence of IGF-1 receptors in the hemangiopericytoma. Two years later, the patient underwent resection of a right frontal hemangiopericytoma with an identical histologic phenotype and receptor positivity for IGF-1. The occurrence of a central nervous system hemangiopericytoma in patient with acromegaly is rare. growth and recurrence of hemangiopericytoma may have been fostered by the presence of IGF-1 receptors. Paracrine mechanisms related to IGF-1 may have contributed to its pathogenesis and growth. The presence of IGF-1 receptors in hemangiopericytomas may have treatment implications for additional adjuvant therapies.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/38. Primary parenchymal cerebral cystic haemangiopericytoma: a 5-year follow up of disease progression.Haemangiopericytoma (HPC) is a rare tumour of the central nervous system. Previously, HPCs were thought to originate from meninges and ventricular walls. Currently, they are accepted as distinctive mesenchymal neoplasms unrelated to meningiomas. Imaging appearances, clinical progression and haemorrhage into a cystic tumour is documented in an 18-year-old man where the final diagnosis, with histopathological verification, proved to be a cystic HPC. This interesting and illustrative case is discussed with the relevant literature.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/38. Unusual presentations of cerebral solitary fibrous tumors: report of four cases.OBJECTIVE AND IMPORTANCE: Four rare cases of central nervous system solitary fibrous tumors (C-SoFTs) are described. This tumor has not previously been reported in children or in deep cortical structures. Three of these tumors occurred in the posterior fossa. Only four cases in the posterior fossa have been described previously. Nine years after its debulking from the posterior fossa, one tumor disseminated to the spine, lung, and liver. Only one such aggressive C-SoFT has been described previously. CLINICAL PRESENTATION: A 7-year-old child had had 1 year of right-sided weakness at presentation. magnetic resonance imaging scans revealed a left basal ganglia lesion. A 49-year-old woman and a 30-year-old man presented after experiencing headaches for months. magnetic resonance imaging scans in these patients revealed a tumor in the fourth ventricle and right jugular foramen, respectively. A 55-year-old man had spinal, liver, and lung dissemination of a previous posterior fossa tumor at presentation. INTERVENTION AND TECHNIQUE: All four patients underwent craniotomy for resection or subtotal removal of the tumor. Intraoperative observations noted solid well-encapsulated tumors. immunohistochemistry confirmed C-SoFTs in all four cases. CONCLUSION: C-SoFTs are rare central nervous system, typically dural-based, tumors. They frequently are overlooked in the differential diagnosis of solid central nervous system tumors. Our findings suggest that these tumors can occur at any age and in most locations, regardless of proximity to the meninges (basal ganglia and ventricle), suggesting that the cells of origin are not meningothelial, but rather the mesenchyme of the cerebral vasculature. T1- and T2-weighted magnetic resonance imaging was notable for areas of hypointensity and of hyperintensity best described as patchy or a ying-yang appearance in all cases. Delayed extracranial metastasis may be noted.- - - - - - - - - - ranking = 3keywords = central nervous system, nervous system (Clic here for more details about this article) |
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