Cases reported "Brain Neoplasms"

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1/396. central nervous system atypical teratoid tumor/rhabdoid tumor: response to intensive therapy and review of the literature.

    central nervous system atypical teratoid/rhabdoid tumor (ATT/RT) of infancy and childhood is a unique histologic entity with an extremely aggressive natural history. Standard therapy for infant and childhood medulloblastoma, for which this entity is often mistaken, has been ineffective; most children survive less than 12 months after diagnosis. Intensified therapy has been recently used for children with this disease, with promising results [1,2]. We report four cases of ATT/RT in young children; all had subtotal resections and localized disease at diagnosis. One child treated prior to bone marrow transplant availability died of progressive disease 9 months after diagnosis. Another child, treated with high-dose chemotherapy and radiotherapy in preparation for bone marrow transplant, had a recurrence and died 20 months after diagnosis, without undergoing the transplant. Two children received high-dose chemotherapy and autologous bone-marrow transplant and had a good response to treatment; one survived 19 months, the other child is free of disease 46 months from diagnosis. Intensified therapy has altered the natural history of central nervous system ATT/RT.
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2/396. Middle ear adenocarcinoma with intracranial extension. Case report.

    Middle ear adenocarcinoma is a very rare, locally invasive neoplasm assumed to arise from the middle ear mucosa. Although endolymphatic sac tumor (aggressive papillary middle ear tumor) and jugulotympanic paraganglioma may show brain invasion, intracranial extension of histologically confirmed middle ear adenocarcinoma has not been previously reported. The authors describe a 53-year-old man who suffered from otalgia and tinnitus for more than 10 years and from neurological deficits for 1 year due to a large temporal bone tumor that invaded the temporal lobe. A combined neurosurgical and otolaryngological resection was performed. Pathological analysis revealed a low-grade adenocarcinoma of a mixed epithelial-neuroendocrine phenotype, which showed a close histological similarity to, and topographical relationship with, middle ear epithelium. The authors conclude that middle ear adenocarcinoma belongs to the spectrum of extracranial tumors that have possible local extension to the brain.
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3/396. Intramedullary cavernous angioma. Resection by oblique corpectomy.

    BACKGROUND: Intramedullary cavernomas are rare lesions usually operated on via a posterior approach and myelotomy. CASE REPORT: A 42-year-old woman progressively developed a tetraplegia with sphincter disturbances over a period of 26 years. magnetic resonance imaging showed a cervical intramedullary cavernoma with an extramedullary anterolateral exophytic portion. To avoid myelotomy, this lesion was approached directly via its anterior exophytic portion. Through a cervical anterolateral approach, the vertebral body of C4 and the intervertebral discs were obliquely drilled out. The posterior longitudinal ligament and the dura mater were opened. The exophytic portion was coagulated and the intramedullary portion was completely excised. The dura mater was closed and a bone graft was inserted between C3 and C5 and secured with a plate. RESULTS: After transient worsening, upper limb weakness improved from its preoperative status but paraparesis persisted after a follow-up of 12 months. The sphincter disturbances disappeared. CONCLUSIONS: The anterolateral approach combined with oblique corpectomy may be an appropriate technique in case of anterior intramedullary cavernomas. It provides direct access to the lesion, avoiding additional myelotomy.
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4/396. Three AML patients with existing or pre-existing intracerebral granulocytic sarcomas who were successfully treated with allogeneic bone marrow transplantations.

    We report three acute myelogenous leukemia (AML) patients who developed intracerebral granulocytic sarcomas (GS) and were successfully treated with allogeneic BMT (allo-BMT). The diagnosis of one patient was AML M2 with myelofibrosis, and the other two patients were AML M4 with eosinophilia (AML M4 Eo), according to the FAB classification. Two patients first experienced a relapse in the brain that resulted in the formation of GS, followed by a relapse in the bone marrow. The remaining patient developed an optic nerve GS after suffering a bone marrow relapse. All three patients received irradiation for the GS and systemic chemotherapy before the allo-BMT. TBI was used for conditioning, and GVHD prophylaxis was with cyclosporine (CsA) and short-term MTX in all three cases. These patients are currently 9 to 37 months post-BMT without relapse. Thus, our experience suggests that allo-BMT is an effective treatment for AML patients with existing or pre-existing intracerebral GS.
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5/396. Generalized osteoblastic bony metastases from medulloblastoma.

    Osteoblastic bony metastases were observed in a case of medulloblastoma three years after surgery and radiation treatment. There was clinical response to COP therapy (cytoxin, oncovin, prednisone). Radiographic and isotopic bone scan study showed uniform increase in bone density. serum calcium and phosphorous and acid phosphatase levels were normal. alkaline phosphatase was elevated and declined with therapy without change in the bone appearance. This suggested an increase in bone deposition activity. However, no increase in calcitonin level was detected either during active disease or following a chemotherapy-induced remission.
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6/396. Extramedullary tumor of monoblasts in the central nervous system: presenting feature of simultaneous bone marrow involvement by acute monocytic leukemia.

    An extramedullary tumor of monoblasts in the central nervous system has been described in the literature as a single case report, preceding the development of acute monocytic leukemia by 1 year. We report a previously undescribed presentation of acute monocytic leukemia as a left temporal lobe extra-axial mass with concomitant bone marrow involvement. We describe our findings in order to heighten awareness of this entity, which may be encountered in morphologic evaluation of central nervous system masses.
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7/396. Isolated recurrence of granulocytic sarcoma of the brain: successful treatment with surgical resection, intrathecal injection, irradiation and prophylactic systemic chemotherapy.

    We describe a 40-year-old male who developed an isolated recurrence of granulocytic sarcoma (GS) of the brain 2 years following successful treatment of acute myeloblastic leukemia (AML; M2). Computed tomography (CT) scans and magnetic resonance (MR) images demonstrated a homogeneously enhanced tumor mass in the left temporal lobe and massive peritumoral edema. There was no evidence of relapse in the bone marrow. The patient underwent an emergency surgical resection of the tumor. Five courses of injection with cytarabine and prednisolone through an Ommaya reservoir and whole brain irradiation (total 40 Gy) were performed. Furthermore, prophylactic systemic chemotherapy with cytarabine and etoposide was added. He has been in complete remission for 21 months. Our results, together with other reported cases, indicate that a favorable outcome could be obtained by intensive and combined treatment for an isolated recurrence of GS of the brain if the bone marrow remained in complete remission.
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8/396. Squamous cell carcinoma arising in an intradiploic epidermoid cyst.

    A 71-year-old woman presented with the symptoms of a posterior cranial fossa mass. CT and MRI revealed a lytic lesion in the occipital bone and a tumour infiltrating the dura mater, venous sinuses and cerebellum. Histopathology demonstrated a moderately differentiated squamous cell carcinoma arising from a primarily intradiploic epidermoid cyst. Despite surgery and radiotherapy, the tumour progressed and the patient died 1 year later.
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9/396. A case of Ollier's disease associated with two intracerebral low-grade gliomas.

    Ollier's disease, or multiple enchondromatosis, is a deforming dysplastic disease of cartilage, characterized by multiple, asymmetrically distributed intra-osseous cartilaginous masses in the metaphyses and diaphyses of bones. When associated with soft tissue hemangiomas it is referred to as Maffucci's syndrome, in which the enchondromatosis has no unilateral distribution. The emergence of malignant neoplasms, including gliomas, is a well-recognized complication in Maffucci's syndrome. We report a 28-year-old patient with a history of Ollier's disease, who developed two low-grade cerebral gliomas as well as an intracranial chondroma. This case history questions the distinction between the two forms of enchondromatosis and supports a continuum between these disease entities.
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10/396. Mycobacterial spindle cell pseudotumor of the brain: a case report and review of the literature.

    Spindle cell pseudotumors found in the skin, lymph nodes, bone marrow, spleen, lungs, and retroperitoneum have been reported recently in immunosuppressed patients, including those with acquired immunodeficiency syndrome. The authors report a similar lesion limited to the brain in a 38-year-old human immunodeficiency virus-negative man receiving steroid therapy for treatment of sarcoidosis. Histopathologically the lesions were composed of spindle and epithelioid histiocytes, small foci of necrosis, and numerous acid-fast bacilli. The acid-fast bacilli were determined by culture and polymerase chain reaction to be mycobacterium avium intracellulare. Because of the uncommon histologic appearance of this lesion and the potential for treatment if recognized, mycobacterial spindle cell pseudotumors should be included in the differential diagnosis of spindle cell lesions in the brain in immunosuppressed patients.
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