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Carotid-cavernous sinus fistulas (CCF) are abnormal communications between the carotid artery and the cavernous sinus. Most spontaneous CCFs are low-flow fistulas, supplied by branches of the omolateral internal carotid artery or the external carotid artery. A 64-year-old man, with increasing diplopia, mild exophthalmos on the left side, blurred left vision and omolateral red eye, was admitted to our institution. The patient underwent a bilateral cerebral angiography that showed a left CCF fed by meningo-hypophyseal branches of the right internal carotid artery and draining from the cavernous sinus into a parahippocampal vein. A transarterial embolization of the carotid-cavernous fistula was performed, with complete obliteration of the fistula. Although anecdotal reports exist, there is a scarcity of well-documented cases of exclusively contralateral flow in the carotid-cavernous fistula. Most of the reported cases referred to contralateral flow into the carotid-cavernous fistula by the external carotid artery branches. To the best of our knowledge, there are no previous cases of a spontaneous CCF supplied by contralateral meningo-hypophyseal branches. A discussion of treatment options and a literature review are also performed.

Objective: To evaluate structural variations of the jugular tubercles (JTs) and their relationships with the vertebral artery, the posterior inferior cerebellar artery (PICA), and the vertebrobasilar junction (VBJ)., Methods: The depth, height, and width of the JTs were measured using 30 cadaveric basicranial specimens and 50 three-dimensional angiography computed tomographic (angio-CT) scans evaluating morphological variations between the 2 sides. Angio-CT analysis evaluated the relationships of the JTs with the vertebral arteries and the PICAs. The location of the VBJ with respect to the JT level in the coronal plane was evaluated., Results: In the cadaveric specimens, the mean JT depth ranged from 0.9 to 3.1 cm, the mean height ranged from 0.6 to 1.5 cm, and the mean width ranged from 0.4 to 1.2 cm. According to the 3-dimensional angio-CT scans, JT measurements ranged as follows: depth, 0.7 to 2.6 cm; height, 0.6 to 1.4 cm; and width, 0.3 to 1.2 cm. The vertebral artery was in close contact with the JT on the left side in 30% of cases and on the right side in 24% of the cases. On axial scans, the PICA origin was classified as anterior to the JT in 20.5% of patients on the left side and 17.4% on the right, at the JT level in 50% of patients on the left side and 45.7% on the right, and posterior to the JT in 29.5% of patients on the left side and 36.9% on the right. On coronal scans, the PICA origin was classified as superior to the JT in 13.6% of patients on the left side and 8.7% on the right, at the JT level in 54.6% of patients on the left side and 50% on the right, and inferior to the JT in 31.8% of patients on the left side and 41.3% on the right. In the coronal plane, the VBJ was located above the tubercles in 16 patients (32%), at the JT level in 28 patients (56%), and below the tubercles in 6 patients (12%)., Conclusion: A precise morphometric analysis of the JTs on 3-dimensional CT scans was quick and safe and showed significant variations in their size and shape. The relationship of the JT with vertebral artery and the PICA varied significantly, as well as with the VBJ location. Knowledge of these morphological variations can contribute to optimal preoperative surgical planning, minimizing retraction and reducing morbidity during extreme lateral infrajugular-transtubercular exposure surgery.

Background: Primary leptomeningeal astrocytomas are rare intracranial tumors. These tumors are believed to originate from cellular nests which migrate by means of aberration, ultimately settling in the leptomeningeal structure. They may occur in both solitary and diffuse forms. The literature reports only fifteen cases of solitary primary intracranial leptomeningeal astrocytomas., Case Presentation: The authors report the case of a seventy-eight year-old woman with a polycystic variant of a solitary primary intracranial leptomeningeal astrocytoma. The first neurological signs were seizures and aphasia. CT and MRI scans demonstrated a fronto-parietal polycystic tumor adherent to the sub arachnoid space. A left fronto-temporo-parietal craniotomy revealed a tight coalescence between the tumor and the arachnoid layer which appeared to wrap the mass entirely. Removal of the deeper solid part of the tumor resulted difficult due to the presence of both a high vascularity and a tight adherence between the tumor and the ventricular wall., Conclusion: A new case of a solitary primitive intracranial leptomeningeal astrocytoma of a rare polycystic variant is reported. Clinical, surgical, pathologic and therapeutic aspects of this tumor are discussed.

Hemangioblastomas of the filum terminale are particularly rare tumors. The authors present the case of a 62-year-old woman with a 6 month history of low-back-pain, who underwent surgery for the removal of an hemangioblastoma affecting the cauda at L2-L3 level. This highly vascularized tumour is tightly adherent to the filum terminale and hence is a very challenging pathology to remove. Histologically it consisted in vascular structures interposed to a network of capillary-like vessels, surrounded by stromal cells. MRI, angiography with pre-surgical embolization, and radical surgery represent the focal points in the diagnosis and treatment of these tumours. The most relevant literature has been carefully reviewed.

Central nervous system mesenchymal chondrosarcomas are rare malignant tumors that constitute a separate entity from the classical chondrosarcoma and myxoid variant. Clinical behaviour of central nervous system chondrosarcomas is still unknown. We describe two rare examples of intracranial mesenchymal chondrosarcoma with a review of the literature, in an attempt to clarify the clinical characteristics, prognosis and treatment of choice of these unusual tumors. Among the 55 reported cases, 23 had postoperative radiotherapy. Although there is no statistical significance according to the Log-Rank test (p=0.7), the patients treated with radiation therapy seem to have a better chance of survival. Patients who had adjuvant chemotherapy (only 5) showed survival times similar to those patients who had none. Although clinical behaviour of central nervous system chondrosarcomas remains to be defined, data from our series as well as literature show that radical removal is the best therapeutic choice. In addition, patients treated with postoperative radiotherapy seem to show a trend toward increased survival.

Brain metastasis from prostate carcinoma occurs very rarely. We describe 13 patients with single brain metastasis from prostatic cancer. Total removal of the lesions was performed in ten patients. Three patients underwent stereotactic biopsy. All patients were treated with postoperative whole brain radiotherapy (WBRT). Eight patients died for systemic disease after a mean time of 9.2 months with a diagnosis of metastasis. Five patients are still alive at 20, 14, 11, 7 and 6 months, respectively. Even if brain metastasis from prostate cancer is often a terminal event with death occurring within few months from diagnosis, we suggest the same protocol (surgery and/or radiosurgery plus postoperative WBRT) usually adopted to treat brain metastasis from other primitive tumours. A non specific neurological symptomatology and a possible normal dosage of serum specific antigen may contribute to a delay in diagnosis. However, considering the rarity of brain metastasis from prostate carcinoma, standard brain MRI follow-up in men with prostatic cancer does not seem to be necessary yet.

Interstitial infusion of carmustine (BCNU) into the rat brain stem in conjunction with systemic administration of O6-benzylguanine (O6-BG) was performed in an effort to assess clinical tolerance. A total of 12 rats underwent stereotactic cannula placement into the pontine segment of the brain stem. Six of the rats underwent a 24-h infusion of BCNU (Volume of infusion [Vi] 200 microl) at its maximal concentrated dose (3.3 mg/ml) in 5% dextrose water. Six additional rats underwent 24-h infusion of BCNU preceded by an intraperitoneal injection of O6-BG at a dose of 50 mg/kg. Serial neurological examinations were performed on all animals. Histologic analyses were performed immediately or 2 weeks following sacrifice. Postoperatively, there were no neurological changes in any of the animals. Postmortem histological examination of the brains showed small pontine cavitary lesions (ranging from 20 to 250 microm) containing variable numbers of macrophages or neutrophils consistent with an inflammatory response. No changes beyond these findings indicated any histological evidence of injury. These finding were limited only to the cannula site and no changes beyond region of the cannula tract were found. These findings indicate that interstitial infusion of BCNU into the brain stem in conjunction with systemic administration of O6-BG is safe in a small animal and may serve as a potential investigative strategy for children with diffuse pontine gliomas.

Interstitial infusion of IL13-PE38QQR, a tumor specific, chimeric cytotoxin, into the rat brain stem was performed in an effort to assess safety. Six rats underwent stereotaxic cannula placement into the pontine segment of the brain stem followed by a 24-h infusion of IL13-PE38QQR (volume of infusion (Vi) 200 microl) at a concentration of 10 microg/ml. The animals were assessed neurologically and then sacrificed either immediately or after 2 weeks. All animals tolerated the infusions without exhibiting any neurological changes. Postmortem examination of the brains revealed no significant histological changes beyond the site of the cannula tract. These findings indicate that supratherapeutic concentrations of IL13-PE38QQR administered by interstitial infusion into the rat brain stem is well tolerated and may serve as a potential therapeutic strategy for children with diffuse pontine gliomas.

Objective: Prolonged convection-enhanced delivery was used in an attempt to achieve large volumes of distribution (V(d)) in the rat brainstem. Clinical assessment and histological analysis were performed to establish the safety of this approach., Methods: For evaluation of V(d,), 10 rats underwent stereotactic cannula placement into the brainstem. Five rats underwent a 24-hour infusion (volume of infusion [V(i)], 200 microl), and 5 rats underwent a 7-day infusion (V(i), 2 ml) of fluorescein isothiocyanate-dextran. Serial brainstem sections were imaged with ultraviolet illumination, and V(d) was assessed. For assessment of clinical tolerance, 30 additional rats underwent chronic infusions of an isotonic saline solution into the brainstem. Serial neurological examinations were performed, followed by histological analysis after the animals' death., Results: No animal demonstrated clinically recognized neurological deficits. Foci of organizing necrosis were limited to the site of infusion and cannula tract. V(d) increased linearly with increasing V(i) (range, 24.8-130.6 mm(3)). Maximal cross sectional area of fluorescence and craniocaudal extent of fluorescence increased with increasing V(i). Fluorescence was detected throughout the entire brainstem beyond the compact area of highly concentrated tracer., Conclusion: Prolonged convection-enhanced delivery can be applied safely in the rat brainstem with no recognized limitations of V(d) and minimal histological changes beyond the site of infusion. Chronic brainstem infusions may enhance the potential application of convection-enhanced delivery for therapeutic purposes in treating diffuse pontine gliomas.

GH deficiency is common in survivors of childhood cancer, especially in those treated with radiation to the brain. The impact of GH therapy on disease recurrence has been studied in survivors of pediatric brain tumors, but few data are available on the risk of disease recurrence in survivors of other tumor types who are treated with GH. Likewise, the risk of second neoplasms (SN) associated with GH use has not been systematically evaluated. We studied 361 GH-treated cancer survivors (including 172 brain tumor survivors) from among 13,539 survivors enrolled in the Childhood Cancer Survivor Study, a cohort of 5-yr survivors of childhood cancer. Using a time-dependent Cox model, we compared risk of recurrence, risk of SN, and risk of death between survivors who did and did not receive treatment with GH. The relative risk of disease recurrence was 0.83 (95% confidence interval, 0.37-1.86; P = 0.65) for GH-treated survivors. The relative risk of recurrence was not increased for any of the major cancer diagnoses. GH-treated subjects were diagnosed with 15 SN, all solid tumors and no secondary leukemias, for an overall relative risk of 3.21 (95% confidence interval, 1.88-5.46; P < 0.0001). This was mainly due to a small excess number of SN observed in GH-treated survivors of acute leukemia. The risk of death was not associated with GH use (P = 0.43). We conclude that GH therapy does not appear to increase the risk of disease recurrence or death in survivors of childhood cancer. The increased number of SN, particularly in survivors of acute leukemia, is of concern, but the data need to be interpreted with caution given the small number of events.

Paraneoplastic nephrotic syndrome (NS) in the setting of membranous nephropathy (MN) is a well-known and often prodromal complication of renal and extrarenal malignancy. Primary cerebral lymphoma is a rare neoplasm uncommonly investigated in the setting of NS. We describe for the first time a case of primary cerebral lymphoma in association with MN. An elderly woman presented for renal biopsy because an NS had been diagnosed previously and treated by steroid therapy. Primary membranous glomerulonephritis was diagnosed and immunosuppressive therapy was started. Twenty days later, she was readmitted because of left hemiplegia. Computed tomography and subsequent nuclear magnetic resonance evaluation of the brain documented an extensive cerebral space-occupying lesion. A cerebral malignant lymphoma was diagnosed at intraoperative histologic evaluation, and subsequent light microscopic and immunohistochemical studies allowed the diagnosis of peripheral T-cell lymphoma. Extensive clinical workup, including bone marrow biopsy, documented the lack of any neoplastic involvement outside the brain. Primary cerebral non-Hodgkin's lymphoma of peripheral T-cell derivation consequently was diagnosed. This case suggests that patients with MN, particularly elderly patients, should undergo complete screening for neoplasms. The absence of specific guidelines on this topic may be due partly to the lack of extensive description of all newly identified cases. All cases should be reported to evaluate whether this association is causal or fortuitous., (Copyright 2002 by the National Kidney Foundation, Inc.)