Diffuse Neurofibroma Presenting with Spontaneous Intra-Tumoral Hemorrhage

This report describes a case of diffuse neurofibroma(DNF) that presented with spontaneous intratumoral hemorrhage. A 31-year-old man with cutaneous manifestations typical of neurofibromatosis type 1(NF1) was referred to hospital with a progressively expanding scalp swelling. Magnetic resonance imaging(MRI) showed an acute hematoma surrounded by a dense mass on the parietooccipital scalp. Plain skull radiographs and a computed tomography(CT) scan revealed bony defects in the occipital bone around the lambdoid suture and the skull base, including the sphenoid and petrous bones. The tumor extended to the upper cervical region and infiltrated through the fascia of the musculature. The tumor and adjacent soft tissue were highly vascular, which made surgical resection difficult. We describe the clinical, radiological and pathological features of this patient and discuss the surgical methods used to avoid a life-threatening hemorrhage during surgery.

Massive Cerebral Infarction Due to Rhinocerebral Mucormycosis

Rhinocerebral mucormycosis is rare, but fatal infection of the nasal cavity and sinuses. It can spread to the orbits and cranium within days, and prognosis is directly associated with length of time before diagnosis and treatment. Rhinocerebral mucormycosis can cause cerebral infarction via carotid artery occlusion. Therefore, neurosurgeon is paramount in making the proper management. We recently encountered a case of rhinocerebral mucormycosis with massive cerebral infarction. The clinical and radiological details of this case are presented here with a brief review of the literature.

Cytotoxicities of Tumor-specific T Lymphocytes Primed by Glioma Apoptotic Body-or Glioma Cell Lysate-pulsed Dendritic Cells

OBJECTIVE: The choice of tumor antigen for dendritic cell(DC)-loading has still been an unresolved problem in the DC-based vaccine strategies against malignant gliomas that has not been found well-characterized tumor specific antigens. In this study, we compare tumor-specific T cell response induced by glioma apoptotic body(GAB)-pulsed DCs to response induced by glioma cell lysate-pulsed ones quantitatively. METHODS: DCs generated in the presence of granulocyte macrophage-colony stimulating factor and interleukin(IL)-4 from peripheral blood mononuclear cells(PBMCs) of HLA-A2 positive healthy donors were cultured. Each GABs and glioma cell lysate generated from HLA-A2 positive T98G glioblastoma cells were co-incubated with DCs. CD8+ T lymphocytes isolated from PBMCs of same donors were cultured in media containing IL-2 and either stimulated by GAB- or lysate-pulsed DCs three times at a weekly interval. The interferon(IFN)-gamma concentrations of each cell culture supernate were measured by enzyme immunoassay technique. Cytolytic activity of the generated cytotoxic CD8+ T cells either stimulated with GAB- or lysate-pulsed DCs was determined by a standard 4-h 51Cr-release assay. RESULTS: IFN-gamma production and cytolytic activity of effector T cells stimulated by GAB-pulsed DCs were significantly higher than those of T cells stimulated by lysate-pulsed ones. CONCLUSION: These results indicate the choice of antigen is a critical determinant in the induction of antitumor immunity against malignant glioma. Antigen preparations from GABs represent a promising alternative to glioma cell lysate in DC-based glioma vaccine strategies.

Two Cases of Multiple Hemangioblastomas in a Von Hippel-Lindau Family

Hemangioblastoma is uncommon, accounting for 1 to 2.5% of all primary neoplasms of the central nervous system. Ten to 20% of hemangioblastomas occur as part of Von Hippel-Lindau disease(VHL). Multiple hemangioblastomas are seen only with VHL and these are seen in up to half of VHL-associated hemangioblastomas. We have treated two cases of multiple hemangioblastomas associated with VHL disease in a family, sister(case I, 48/female) and her brother(case II, 41/male). Both patients had renal and pancreatic cysts in addition to CNS hemangioblastomas. Their hemangioblastomas were removed totally, and then their neurological symptoms had Improved. With a review of the literature, the authors present a family of VHL-associated multiple hemangioblastomas.