1/23. Sinonasal teratocarcinosarcoma: ultrastructural and immunohistochemical evidence of neuroectodermal origin.The authors report a case of sinonasal teratocarcinosarcoma in a 37-year-old man, which was located in the anterior skull base and extended to the right nasal cavity and paranasal sinuses. The tumor was surgically resected twice, but it could not be removed completely. Microscopically, it was mainly composed of primitive cell nests within a moderately cellular stroma. The components of squamous cell epithelia with focal teratoid appearance and adenocarcinomatous differentiation were observed. There were many rhabdomyoblasts scattered in the nests and stroma. Ultrastructurally, the primitive cells had many neural processes with parallel microtubules, resembling olfactory neuroblastoma. Rhabdomyoblasts showed various degrees of skeletal muscle differentiation. Some of the stromal spindle cells had actin filaments with dense patches and dense core granules. Immunohistochemically, the primitive cells were positive for epithelial markers, neuron-specific enolase, synaptophysin, and myogenic regulatory proteins. The rhabdomyoblasts showed immunoreactivity for myoid markers, cytokeratin, epithelial membrane antigen, and synaptophysin. Most of the stromal spindle cells were positive for smooth muscle actin, neuron-specific enolase and synaptophysin. The immunohistochemical and ultrastructural findings suggest that primitive cells had the most primitive phenotype of placodes, and support the possibility that sinonasal teratocarcinosarcoma is essentially a neuroectodermal tumor with divergent differentiation.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/23. Epithelioid haemangioendothelioma of the sphenoid bone.The authors report a case of cranial EH occurred in the left sphenoid bone that was totally excised without transfusion. A 26-year-old woman presented with a 1-year history of progressing exophthalmos in the left eye. A cranial X-ray showed a mixed osteolytic and sclerotic expansile mass lesion in the left sphenoid bone. Neuroradiologic imaging study revealed a left temporosphenoidal extra-axial expansile mass lesion with heterogeneous enhancement after contrast enhancement associated with destruction and erosion of the temporal and sphenoid bones. The cerebral parenchyma and orbital contents were compressed without any sign of infiltration. Bone scan showed hot uptake in the left orbital region. Angiogram demonstrated marked neoplastic angiogenesis from the middle meningeal artery and other branches of left external carotid artery, for which preoperative embolisation was tried in vain. Cranio-orbito-zygomatic approach was undergone in two stages because of the patient's religious belief (patient was Jehovah's witness) and profuse bleeding during the first surgery. Two weeks after initial operation, second surgery was carried out with total excision of the residual mass in the left orbital ridge. Histopathological examination revealed typical epithelioid cell cords or nests in myxoid stroma with a positive immunoreactivity to factor viii-related antigen. The authors report an unusual case of EH involving sphenoid and temporal bone in a young woman. Preoperative embolisation is thought to be absolutely essential before removal. Because there is no convincing data to advise radiation/chemotherapy, total resection and close follow-up may be reasonable.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/23. Tissue-specific expression of SV40 in tumors associated with the li-fraumeni syndrome.Inactivation of wild-type p53 tumor suppressor function is the primary mechanism of tumor initiation in li-fraumeni syndrome (LFS) individuals with germline p53 mutations. Tumors derived from LFS patients frequently retain the normal p53 allele, suggesting that alternative mechanisms in addition to gene deletion must be involved in inactivating wild-type p53 protein. dna tumor viruses, such as SV40, target p53 for inactivation through the action of viral oncoproteins. We studied the probands from two unrelated LFS families, each of whom presented with multiple malignant neoplasms. Patient 1 developed an embryonal rhabdomyosarcoma (RMS) and a choroid plexus carcinoma (CPC), while patient 2 developed a CPC and subsequently presented with both an osteosarcoma (OS) and renal cell carcinoma (RCC). We utilized DNA sequence analysis and immunohistochemistry to determine p53 gene status in the germline and tumors, as well as evidence for SV40 T-antigen oncoprotein expression. Each patient harbored a heterozygous germline p53 mutation at codons 175 and 273, respectively. In patient 1, the normal p53 gene was lost while the mutant p53 allele was reduced to homozygosity in the RMS. Both normal and mutant genes were maintained in the CPC. In patient 2, normal and mutant p53 alleles were retained in both the CPC and RCC. Both specific PCR and immunostaining detected SV40 T-antigen in both CPCs and the RCC. In addition to chromosomal alterations, epigenetic mechanisms may disrupt p53 function during tumorigenesis. In two LFS patients, we found SV40 DNA sequences and viral T-antigen expression that could account for inactivation of the normal p53 protein. Inactivation of p53 or other tumor suppressors by viral proteins may contribute to tumor formation in specific tissues of genetically susceptible individuals.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
4/23. 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.- - - - - - - - - - ranking = 5keywords = antigen (Clic here for more details about this article) |
5/23. Primary angiosarcoma of the cranial vault: a case report and review of the literature.BACKGROUND: Angiosarcoma is a rare neoplasm. It is most likely to affect the scalp in elderly people and involvement of the cranium is uncommon. We report a case of primary malignant angiosarcoma of the cranial vault. CASE DESCRIPTION: An 82-year-old man presented with right hemiparesis, deformity of the cranium and mildly swollen scalp. Further studies disclosed bilateral parietal lesions, with destruction of the dura matter and infiltration of the brain parenchyma. thrombocytopenia was found. Faint enhancement of the lesions was seen on magnetic resonance imaging (MRI) after i.v. administration of contrast medium with gadolinium. The tumor was excised including the affected portion of the skull and dura matter. Histologic examination revealed typical pictures of angiosarcoma with immunohistochemical evidence of factor viii-related antigen. Postoperative radiotherapy was employed. The patient remained well at 10 months postoperative follow up. CONCLUSION: Primary angiosarcoma of the cranium is sometimes difficult to differentiate from trauma on clinical examination. We review the literature pertaining to the pathogenesis, clinical course and treatment of this condition. Determination of platelet count may be helpful in monitoring the progression of the disease. The tumor may show faint enhancement on MRI.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/23. Collet-Sicard syndrome: a rare presentation of metastatic prostate adenocarcinoma.Metastatic spread of prostate adenocarcinoma to the temporal bone is rare. Involvement the jugular foramen may result in Collet-Sicard syndrome. This extremely uncommon clinical entity is characterized by paralysis of the lower four cranial nerves. A case of metastatic prostate adenocarcinoma involving the temporal bone causing a Collet-Sicard syndrome associated with an ipsilateral lower motor facial palsy and a mixed sensorineural and conductive hearing loss is presented. This case highlights the potential of prostate adenocarcinoma to cause symptoms referable to the temporal bone region and histopathologic analysis of biopsy material should include immunohistochemical staining for prostate specific antigen.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/23. December 2004: one-year-old girl with aggressive skull tumor.December 2004. Twelve-month old girl presented with recurrent subcutaneous lesion in the left parietal region, one year after excision of a "benign" tumor. An MRI demonstrated left temporo-parietal skull tumor infiltrating the soft tissue, surrounding craniotomy flap, and extending to the brain parenchyma. biopsy revealed biphasic neoplasm displaying nests of poorly differentiated neuroblastic cells positive for synaptophysin and pigmented cuboidal epithelioid cell positive for keratins, epithelial membrane antigen and MHB-45. In addition, some neoplastic cells were immunoreactive for synaptophysin as well as HMB-45 and epithelial markers, suggestive of their origin from a common progenitor. Interestingly, cell with the neuroblastic immunophenotype displayed 80% nuclear MIB-1 reactivity indicating that the aggressiveness of the neoplasm was confined mostly to this pattern of differentiation. The overall histological features are consistent with a rare malignant variant of a melanotic neuroectodermal tumor of infancy.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/23. Primary temporal bone angiosarcoma: a case report.We present a rare case of temporal bone angiosarcoma diagnosed in a 26-year-old female patient at 36 week of pregnancy. The patient was referred with a 2 months history of left otalgia and tinnitus with a tender swelling above the mastoid. Cranial imaging studies showed a 7 x 5 x 4 cm hypervascularized mass located in the left middle fossa with lysis of the temporal bone and extension to the subcutis. After the baby was delivered by caesarean section, the patient entered the oncology protocol. Selective embolization of the feeding vessels was followed by gross total surgical resection using a combined supra- and infra-tentorial approach. Pathological findings were those of a poorly differentiated, highly malignant sarcoma with a large epitheloid component and immunohistochemical evidence of endothelial differentiation (CD31, factor viii related antigen, CD34), consistent with an angiosarcoma with epitheloid features. No extra-cranial tumor was found after extensive staging. The patient received adjuvant radiotherapy followed by a course of chemotherapy consisting of 6 cycles of paclitaxel. At 15 months follow-up, she developed multiple distant metastasis to a left postauricular lymph node and to the lungs and ribs. The patient was given a second line chemotherapy using doxorubicine and ifosfamide. Despite an initial good response, she died with metastatic disease 26 months after diagnosis. We present a rare case of primary temporal bone angiosarcoma and report our experience with a multimode therapeutic approach combining surgery, radiotherapy and chemotherapy.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/23. Sarcomatous transformation in cranial chordoma.In a study of 52 patients with cranial chordoma treated at the Mayo Clinic over a 19-year period (1966-1984), two tumors showed anaplastic features, both de novo, i.e., unassociated with prior irradiation. The incidence of anaplastic transformation was thus 4%. immunohistochemistry showed the mixed mesenchymal-epithelial phenotype typical of chordoma in portions of both tumors, but loss of reactivity for keratin and epithelial membrane antigen was noted in anaplastic components. The study indicates that sarcomatous change in chordoma is a rare event that may occur de novo and is associated with the loss of immunophenotypic features of epithelial differentiation.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/23. Unilateral proptosis as a clinical presentation in primary angiosarcoma of skull.A case is reported of unilateral proptosis of the left eyeball as a unique clinical presentation of primary angiosarcoma of the skull involving the sphenoid and frontal bones and roof of the orbit on the left side of a 32-year-old man. The intraosseous, extradural non-encapsulated, and friable tumour contained cytoplasmic factor viii-related antigen. After complete removal of the tumour the patient has been well for 10 months of the follow-up period. This case brings the total number of reported primary angiosarcomas of the cranium to nine. All patients were male and most of them were young. The average age was 24 years. The frontal bone seems to be the site of predilection for cranial angiosarcomas. Surgical extirpation, radiation, or combined surgical removal and radiation have been used for treating angiosarcoma of the skull with variable results. It is suggested that angiosarcoma of the skull has a worse prognosis than angiosarcoma of bones other than the cranium because the patient may die of secondary neoplastic involvement of the brain.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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