Cases reported "Skull Neoplasms"

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1/23. Primary Ewing's sarcoma/peripheral primitive neuroectodermal tumor at the vertex of the skull with elevated serum carcinoembryonic antigen: case report.

    A primary Ewing's sarcoma arising in the skull is relatively rare. Although a small number of case reports noted elevated carcinoembryonic antigen (CEA) in patients with primary central nervous system (CNS) neoplasms, there is no report of Ewing's sarcoma/peripheral primitive neuroectodermal tumor (PNET) with elevated serum levels of CEA. A 7-year-old boy who had episodes of headache and vomiting had noticed a solid mass in the vertex of the head. Imaging studies revealed a large intra- and extracranial tumor at the vertex of the skull. Hematological examination demonstrated high serum levels of CEA: 91.09 ng/ml. The patient initially underwent an embolization of the bilateral middle meningeal arteries with Gelfoam particles. One week later, the patient was operated on and a subtotal resection of the tumor was performed. On histopathological and molecular genetic examination, the tumor was diagnosed as a Ewing's sarcoma/peripheral PNET. Immunohistochemical study showed strongly positive staining for CEA in the tumor cells. The serum level of CEA was normalized at 0.83 ng/ml after the tumor was removed and the boy underwent radiotherapy and 3 courses of chemotherapy. This is the first reported case of a primary Ewing's sarcoma/peripheral PNET at the vertex of the skull with elevated serum CEA.
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keywords = central nervous system, nervous system
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2/23. Imaging features of primary malignant rhabdoid tumour of the brain.

    Primary malignant rhabdoid tumour of the central nervous system is a rare neoplasm affecting children. We present a pathologically proven case, which was initially referred to the paediatric surgeons as a sebaceous cyst, and highlights the importance of imaging prior to surgery of potentially innocuous scalp lesions. Imaging features on CT and MRI are presented, which show bony involvement not previously reported in the literature.
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keywords = central nervous system, nervous system
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3/23. Unique presentation of pleomorphic xanthoastrocytoma as a lytic skull lesion in an eight-year-old girl.

    Pleomorphic xanthoastrocytomas (PXAs) are rare primary central nervous system neoplasms of childhood and early adulthood. While PXAs are intimately associated with the leptomeninges, there has never been a description of an extradural-extracranial manifestation of PXA until now. We describe a case of PXA where the patient presented with a calvarial lesion. We also review the differential diagnoses of lytic skull lesions in addition to PXA.
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keywords = central nervous system, nervous system
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4/23. skull base metastasis from gastric cancer--case report.

    A 48-year-old male presented with a metastatic skull base tumor located on the right of the clivus and the sphenoid sinus, which originated from adenocarcinoma of the stomach. The initial symptom was abducens nerve paralysis and there were no symptoms of upper gastrointestinal tract throughout the course. This gastric cancer, which rarely metastasizes to the central nervous system or osseous system, caused multiple bone metastases which produced the neurological symptoms.
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keywords = central nervous system, nervous system
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5/23. Encouraging result of tamoxifen in a retinoblastoma patient with central nervous system metastasis.

    Extraocular retinoblastoma occurs more frequently in developing countries as a delayed diagnosis and prognosis of patients with conventional therapy is very poor. Metastatic retinoblastoma, especially in the central nervous system (CNS), is a highly lethal disease. tamoxifen has been used in some previous studies with variety of responses to therapy in patients with unresectable recurrent brain tumors. A 7-year-old girl with recurrent metastatic retinoblastoma received 60 mg/m2 tamoxifen in addition to chemotherapy and CNS radiotherapy. She was in remission until she has died in a traffic accident at week 114. The authors think that tamoxifen can be added to treatment protocols of metastatic retinoblastoma to provide longer and at least higher quality of life for these patients.
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keywords = central nervous system, nervous system
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6/23. Calvarial malignant fibrous histiocytoma.

    Primary malignant fibrous histiocytoma (MFH) of the central nervous system (CNS) is uncommon. We report cases of two young patients of MFH arising from the cranial meninges and involving the adjacent skull and scalp. There was infiltration of the brain in one case. Both the lesions were excised and primary scalp repair was performed.
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keywords = central nervous system, nervous system
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7/23. Giant cranial extradural primary fibroxanthoma: a case report.

    Primary fibroxanthoma of the cranium is an extremely rare condition. Xanthomatous tumors of the central nervous system are occasionally associated with diseases such as hand-Schuler-Christian disease, malignant fibrous histiocytoma, hyperlipidemia, and a complication of metabolic or storage disorders. However, until 2003, only a few cases of primary fibroxanthoma have been reported. We report a giant fibroxanthoma destroying the lateral skull base unaccompanied by a systemic or metabolic disease.
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keywords = central nervous system, nervous system
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8/23. Amyloidoma of the skull base.

    Localized amyloid tumors of the central nervous system are rare. We present the case of a 59-year-old white man with a large expansile amyloidoma at the base of the skull. To our knowledge this is the second reported case of localized amyloid tumor involving the skull.
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keywords = central nervous system, nervous system
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9/23. Parasellar syndrome caused by plasma cell leukemia.

    A 71-year-old man had incomplete third cranial nerve palsy caused by plasma cell leukemia and a parasellar plasmacytoma. This is the first reported case of plasma cell leukemia in which a central nervous system sign was the initial manifestation. Only one previous case of intracranial plasmacytoma associated with plasma cell leukemia has been reported. The characteristics of intracranial plasmacytomas and of plasma cell leukemia are discussed.
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keywords = central nervous system, nervous system
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10/23. temporal bone pathology of metastatic T-cell lymphoma.

    Metastatic lymphoma involving the temporal bone is a rare tumour. A review of the English literature revealed only 13 reported cases. The common metastatic sites within the temporal bone are bone marrow and the internal auditory canal (IAC). Non-Hodgkin lymphoma has a tendency to infiltrate to the inner ear and middle ear, with bone marrow involvement. Recent advances in immunology have redefined the malignant lymphoma as a neoplasm of the immune system that has involved T and B cells. We present a case of non-Hodgkin T-cell lymphoma that metastasized to the temporal bone and central nervous system.
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keywords = central nervous system, nervous system
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