1/47. Primary orbital angiosarcoma: a case report.PURPOSE: The pathogenesis, natural history, histopathology, and recommended treatment for orbital angiosarcoma are illustrated and reviewed. methods: Case report. RESULTS: A 71-year-old white male presented with bluish discoloration and swelling of the left medial canthal area. A fine needle aspiration and excisional biopsy with histopathologic examination was performed, which showed angiosarcoma. Pattern of growth was demonstrated radiographically and histopathologically, confirming primary orbital angiosarcoma. Subsequent wide surgical resection was carried out, with substantial reconstruction of the left orbital and periorbital area. The patient responded well to the surgery, and was free of tumor after six years of follow-up. CONCLUSION: Angiosarcoma is a rare and highly malignant tumor of epithelial origin. The aggressive nature of this tumor usually results in a high mortality rate despite treatment. However, early diagnosis and wide surgical excision has resulted in successful treatment of these tumors.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/47. Gamma probe localization of cranial bone lesions.PURPOSE: Staging of cancer is essential to formulate appropriate treatment plans and to help predict prognosis. A solitary region of increased radionuclide uptake ("hot spot") on a bone scan may represent a metastasis or a masquerading lesion. biopsy may be required to determine its histologic nature, but localization of the site may be difficult because bone scans provide poor spatial resolution. methods: In two patients with breast carcinoma, radioactive technetium was administered intravenously and a gamma probe was used preoperatively and intraoperatively to identify the site of cranial bone involvement. RESULTS: The lesions were resected; one was a benign fibro-osseous lesion and one was a metastatic breast adenocarcinoma. CONCLUSIONS: A gamma probe may be helpful in localizing the site of radioactive uptake identified by bone scan.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/47. Ongoing hypermutation in the Ig V(D)J gene segments and c-myc proto-oncogene of an AIDS lymphoma segregates with neoplastic B cells at different sites: implications for clonal evolution.To investigate the role of somatic Ig hypermutation in the evolution of AIDS-associated B cell lymphomas, we analyzed the Ig V(D)J and c-myc genes expressed by neoplastic B cells in two extranodal sites, testis and orbit, and clonally related cells in the bone marrow. testis and orbit B cells expressed differentially mutated but collinear V(H)DJ(H), V kappa J kappa and c-myc gene sequences. Shared mutations accounted for 10.2%, 8.4%, and 4.3% of the overall V(H)DJ(H), V kappa J kappa, and c-myc gene sequences. Tumor-site specific V(H)DJ(H), V kappa J kappa, and c-myc mutations were comparable in frequency, and a single point-mutation gave rise to an EcoRI site in the testis c-myc dna. Both shared and tumor site-specific V(H)DJ(H), V kappa J kappa, and c-myc mutations displayed predominance of transitions over transversions. The "neoplastic" V(H)DJ(H) sequence was expressed by about 10(-5) cells in the bone marrow, and contained two of the three orbital, but none of the testicular V(H)DJ(H) mutations. The nature and distribution of the Ig V(D)J mutations found in the kappa chain suggested a selection by antigen in testis and orbit. Our data suggest that, in AIDS-associated B cell lymphomas, the Ig hypermutation machinery targets V(H)DJ(H), V kappa J kappa, and c-myc genes with comparable efficiency and modalities.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/47. The role of ultrasonography in the diagnosis of orbital lymphangiomas.Orbital lymphangiomas are benign hamartomatous tumours usually diagnosed in early childhood. In contrast with the benign histology, these lesions have a locally aggressive nature. They may enlarge slowly, producing mild proptosis; nevertheless intrinsic haemorrhage can occur, producing a mass effect and leading to intractable, painful, progressive proptosis, and compressive optic neuropathy. A conservative approach is advisable for several reasons: complete resection is difficult because these lesions, unlike haemangiomas, are not encapsulated, do not respect anatomic boundaries, tend to penetrate into normal structures, and can bleed profusely during surgery. For these reasons, an early diagnosis is important. On this subject, ultrasonography appears to be the first-choice diagnostic test, because of its sensitivity and simplicity, particularly in childhood; therefore we recommend to always perform this test in the presence of orbital vascular lesion.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/47. Multiple recurrences in malignant peripheral nerve sheath tumor of the orbit: a case report and a review of the literature.PURPOSE: To report the onset of malignant peripheral nerve sheath tumor of the orbit 8 years after irradiation in a patient with neurofibromatosis type-1. methods: Case report of a young man with neurofibromatosis type-1 who received irradiation for presumed bilateral optic nerve and chiasmal gliomas and in whom a malignant peripheral nerve sheath tumor later developed. Exenteration with extirpation of the entire contents of the orbit was performed 6 times. RESULTS: Complete recurrence of the tumor occurred after each surgical procedure until the patient died of malignancy. CONCLUSIONS: Our case underscores the risk of irradiation, especially in children with neurofibromatosis type-1, and emphasizes that radiotherapy should never be given as an empirical therapy. The authors believe that irradiation and neurofibromatosis type-1 may, in combination, pose a significant risk for the development of malignancies. Clear-cut indications and a precise tissue diagnosis are desirable before the initiation of radiotherapy, particularly in the pediatric population. We recommend that if irradiation is necessary in persons with neurofibromatosis type-1, regular follow-up is imperative. In view of the hostile nature of malignant peripheral nerve sheath tumor, early aggressive treatment appears to be the only viable alternative at present.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/47. Orbital lymphangioma with positive immunohistochemistry of lymphatic endothelial markers (vascular endothelial growth factor receptor 3 and podoplanin).BACKGROUND: Existence of true orbital lymphangiomas has been questioned in recent years. Therefore an orbital lymphangioma was analyzed with two new specific markers of lymphatic endothelium. methods: Case-report with clinicopathological, immunohistochemical, and ultrastructural findings. A 25-year-old man presented with recurrent lower lid "hematomas" and a pea-sized tumor palpable in the left lower lid. magnetic resonance imaging showed an inferonasally located orbital tumor which extended to the posterior pole of the eye. The highly vascularized tumor was excised by medial orbitotomy. RESULTS: Histopathologically, the mass consisted of large, erythrocyte-filled cavernous vessels without evidence of smooth muscle cells or pericytes surrounding them. Numerous lymph follicles and small arterioles were scattered between them. Immunohistochemically, endothelial cells lining the lumina of the cavernous vessels were partly positive for podoplanin and vascular endothelial growth factor receptor 3 (flt-4), two markers of lymphatic endothelium. These markers did not react with endothelial cells lining the arterioles. Ultrastructurally, cavernous vessels displayed features characteristic of lymphatic vessels, and the smaller vessels demonstrated signs of arterioles. CONCLUSION: Ultrastructural analysis and immunohistochemistry using two new markers of lymphatic endothelium suggest a lymphatic nature of large vessels in an orbital lymphangioma. A greater series of vascular orbital tumors must be studied with these new lymph endothelial markers to confirm the existence of true orbital lymphangiomas and to analyze different profiles of lymph endothelial marker expression.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/47. radiation-induced meningiomas involving the orbit.PURPOSE: To review the clinical features and outcomes of patients with radiation-induced meningiomas involving the orbit. DESIGN: Retrospective case series. PARTICIPANTS: Eight patients with radiation-induced meningiomas affecting the orbit. methods: Clinical and pathologic data of the patients were reviewed. MAIN OUTCOME MEASURES: Age at diagnosis, mean interval between radiation therapy and meningioma diagnosis, tumor recurrence, histologic atypia, and mean follow-up time after initial diagnosis. RESULTS: The mean age at diagnosis was 42 years (range, 21 years to 70 years). The mean interval between radiation therapy and meningioma diagnosis was 26 years (range, 3 years to 54 years). All patients underwent gross total resection or subtotal resection of the meningioma. Five tumors (62.5%) recurred, based on clinical findings and CT imaging. The mean interval between resection of the meningioma and recurrence was 3 years (range, 9 months to 9 years). Three patients (37.5%) had atypical meningiomas. One patient (12.5%) had multiple tumors. The mean follow-up interval was 7 years after initial diagnosis of the meningioma (range, 15 months to 19 years). CONCLUSIONS: This series of radiation-induced meningiomas, the first in the ophthalmic literature, illustrates the aggressive nature of this tumor.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/47. Solitary fibrous tumor of the orbit. Case report and review of the literature.BACKGROUND: Solitary fibrous tumor (SFT), which usually presents in the pleura and is thought to be mesothelial in nature, has been recently discovered in extrapleural sites, including the orbit. Presently ultrastructural studies show absence of epithelial-mesothelial features, and reactivity of the tumor cells to CD34 antigen on immunohistochemical analysis suggests the mesenchymal origin of such tumors. CASE DESCRIPTION: A 40-year-old woman had a 4-year history of progressive swelling of her right upper lid and a slow-growing palpable mass of the orbit. CT and MR imaging showed a well circumscribed, nonenhanced extraconal mass with mild erosion of the right orbital roof. The tumor was totally excised. Histological examination disclosed a spindle-cell tumor in a dense fibrous tissue. immunohistochemistry showed positive staining for vimentin and CD34. We review the clinical, diagnostic, and surgical features of 22 orbital SFTs including the present case. CONCLUSIONS: Orbital SFT generally pursues a slow, indolent, and nonaggressive course, reaches a size up to 4.5 cm, and can be cured by a single excision. It must be immunohistochemically differentiated from other spindle-cell tumors of the orbit.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/47. Marginal-zone B-cell lymphoma, extranodal-malt-type: report of three cases.A high proportion of extranodal lymphoid infiltrates are diffuse small lymphocytic proliferations [1, 2] and therefore pose a particularly challenging diagnostic problem [1-4]. Their benign or malignant nature cannot be determined using clinical and radiologic criteria. The application of traditional morphologic criteria (i.e., cytologic maturity and polymorphism favor benignancy, while cytologic atypia and monomorphism favor malignancy) [5, 6], has probably improved diagnostic accuracy. However, these criteria generally have not been helpful in evaluating the large number of lymphoid infiltrates composed of monomorphic collections of small cytologically-mature appearing lymphoid cells [1-4] and, therefore, have not always accurately predicted clinical behavior. Extranodal lymphoid proliferations are also of considerable biologic interest since their pathogenesis and natural history have not been fully elucidated. Localized non-Hodgkin's lymphoma, extranodal-MALT-type. was diagnosed in our Department in three cases: Two arose in the orbit and the third one in the tonsil. The tumors had a typical histologic appearance. The microscopical features and immunohistochemical profile are discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/47. Solitary fibrous tumor of the orbit with extraorbital extension: case report.OBJECTIVE AND IMPORTANCE: solitary fibrous tumors (SFTs) are rare tumors of mesenchymal origin that typically arise in the pleura. Only 24 cases of SFTs in the orbit have been reported, all located within the orbit and generally with a benign course. We report the first case of an orbital SFT with extraorbital extension and short-term regrowth. CLINICAL PRESENTATION: A 54-year-old man presented with proptosis and double vision that had persisted for 7 months. The tumor extended from the right extraconal inferolateral orbit to the extradural middle cranial fossa and cavernous sinus, via the superior orbital fissure, on magnetic resonance imaging scans. Positron emission tomography with [(18)F]fluorodeoxyglucose demonstrated faint uptake in the orbital portion. INTERVENTION: Resection of the tumor was performed twice, because of short-term regrowth of the residual tumor in the orbit. The histological diagnosis was a SFT. The MIB-1 labeling index was 7% and the mitotic count was 5 mitotic figures/10 high-power fields at the time of the second operation. These findings indicate the malignant nature of the tumor. CONCLUSION: The natural history of SFTs of the orbit remains unclear, and the importance of careful and continued follow-up monitoring of the tumor should be emphasized.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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