1/79. Primary orbital leiomyoma and leiomyosarcoma.A case of an extremely rare primary orbital leiomyoma in a 25-year-old male patient is presented who had a lifelong history of deviation of the left eye globe with slight enophthalmos and reduced motility. Because of pain and increasing deviation of the eye the tumor was totally resected. On histologic examination the tumor showed ossification which is extremely rare so that a calcifying fibroma had to be ruled out. In immunohistochemistry, however, this tumor stained with smooth muscle antigen. Less than 2% of cells stained positive for Ki-S1, a proliferation marker. The second case is a rare primary orbital leiomyosarcoma in an 84-year-old female patient that showed massive growth. After exenteration histologic examination showed a dedifferentiated highly malignant soft tissue tumor which expressed desmin and smooth muscle actin but was negative for myoglobin, S-100 and HMB-45.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/79. Primary orbital Ewing sarcoma in a middle-aged woman.A 43-year-old woman had unilateral exophthalmos caused by primary orbital Ewing sarcoma. Specialized immunohistochemical stains, primarily MIC-2 (CD99), aided in the diagnosis of Ewing sarcoma. Twenty-two months after radiotherapy and multiagent chemotherapy, the patient remained tumor free. To our knowledge, this is the first reported case of orbital Ewing sarcoma to present in an adult beyond the fourth decade of life.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
3/79. orbital pseudotumor: case report and literature review.The term orbital pseudotumor refers to a broad category of non-specific idiopathic inflammations of the orbit. This disease, which may affect any orbital structure, is one of the commonest causes of exophtalmus, occurring with a similar incidence in both sexes. The diagnosis is based on a combination of clinical, radiological and histopathological findings, after careful exclusion of specific systemic and local diseases. Many classification systems have been proposed and a range of therapeutic modalities, including surgery, steroids, immunosuppressive agents, and radiation therapy, have been employed by various authors in heterogeneous series of patients. This slowly proliferating disease, which usually presents with a long clinical history and high variability in clinical manifestations and prognosis, is difficult to manage with any of the available therapeutic options. The difficulties and controversies regarding the diagnostic and therapeutic management of these patients are addressed in an updated review of the literature and exemplified in our case report.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
4/79. Giant cell angiofibroma of the orbit and eyelid.PURPOSE: To report the clinicopathologic features of a newly recognized tumor, giant cell angiofibroma. DESIGN: Observational case series. MAIN OUTCOME MEASURES: Clinical and histopathologic features of giant cell angiofibroma. methods: light and electron microscopy and immunohistochemistry of five cases of giant cell angiofibroma. RESULTS: A total of five patients (4 women and 1 man) are described: two presented with a painless mass in the eyelid, two with a mass in the orbit, and one presented with a conjunctival lesion. All lesions were well demarcated with no capsule and were composed of blood vessels, a patternless spindle-shaped cell proliferation with a solid and pseudovascular appearance, and multinucleated giant cells. Both spindle-shaped and giant tumor cells were intensely positive for CD34 and vimentin. CONCLUSION: Giant cell angiofibroma resembles solitary fibrous tumor and giant cell fibroblastoma and should be considered in the differential diagnosis of spindle-cell tumors in the eyelid, orbit, and conjunctiva.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
5/79. Follicular large-cell lymphoma of the orbit: a clinicopathologic, immunohistochemical and molecular genetic description of one case.BACKGROUND: Follicular large cell lymphoma of the orbit is a very rare and aggressive lymphoproliferative disease of the ocular adnexa. In this study we analyzed the clinicopathologic characteristics of one patient, including the immunoglobulin gene rearrangement assay by means of polymerase chain reaction. CASE REPORT: A 71-year-old female underwent an incisional biopsy in the superior nasal quadrant of the left orbit following the occurrence of bilateral eyelid edema and ptosis. histology and immunohistochemistry revealed a follicular large cell lymphoma of the orbit. Computerized tomography aimed at staging the disease revealed a supraclavicular lymphoadenopathy approximately 0.5 cm in diameter. bone marrow biopsy was negative for lymphoma. The patient was classified as stage IV. She underwent a 10-week cycle of polychemotherapy (VP16, adriamycin, cyclophosphamide, vincristine, prednisone, bleomycin). Ten months after therapy, disease recurred at the cervical lymph nodes and the patient underwent a radiotherapy cycle of 4320 cGy with disease remission. At the time of writing, the patient presents a left axillary adenopathy on computerized tomography followup. CONCLUSION: Ophthalmologists should be aware of possible rare occurrences of follicular large cell lymphomas of the orbit. In this case the clinical outcome is always more aggressive than the more common MALT-type lymphoma, which usually exhibits benign behavior.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
6/79. Extraorbital giant cell angiofibroma.A number of intriguing fibrovascular mesenchymal proliferations with benign or low grade malignant potential have recently been described. Giant cell angiofibroma was introduced as an entity by Dei Tos et al. in 1995 and initially considered to be a lesion of the orbit. We describe an extraorbital example, indicating that giant cell angiofibroma is not confined to the orbit. Immunologically, giant cell angiofibroma is positive for CD 34, bcl-2 and vimentin, and negative for epithelial and muscle markers, and S-100. The tumor shares several morphological and immunological properties with giant cell fibroblastoma and solitary fibrous tumor, yet it features a histology sufficiently characteristic to allow its categorization as a separate entity. The recommended treatment is complete but conservative excision. Metastases have not been reported.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
7/79. Epithelioid hemangioma of the orbit.OBJECTIVE: To describe the histopathologic features of two cases of epithelioid hemangioma occurring in the orbit and to distinguish this condition from Kimura's disease and from other vascular lesions of proliferated endothelium. DESIGN: Two interventional case reports. INTERVENTION: Treatment consisted of orbitotomy with excision of the tumor. MAIN OUTCOME MEASURES: Histopathologic examination including light microscopy, immunohistochemistry, and electron microscopy and clinical follow-up. RESULTS: In one case, there was no local recurrence after 2 years of follow-up. In the other case, local recurrence required re-excision 2 years after surgery with no apparent recurrence 16 years later. Histopathologic examination of both tumors disclosed an epithelioid hemangioma. A characteristic finding was the presence of peculiar plump vacuolated endothelial cells lining the vascular lumina. CONCLUSIONS: Epithelioid hemangioma is an uncommon benign vascular tumor that can occur in the orbit, and surgical excision is usually required. It is the same condition as angiolymphoid hyperplasia with eosinophilia. There are distinct clinical and histopathologic characteristics to distinguish epithelioid hemangioma from Kimura's disease and from other vascular tumors.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
8/79. Orbital extension of a frontal sinus osteoma in a thirteen-year-old girl.Osteomas are uncommon, slow-growing, benign osteogenic neoplasms that arise most frequently in the craniofacial skeleton. (1,2) osteoma is the most common benign tumor of the nose and paranasal sinuses and the most common neoplasm of the frontal sinus. (3-5) Paranasal sinus osteomas originate in the sinus wall, fill the lumen with well-defined mature osseous tissue, and occasionally extend into the orbit where they give rise to orbital signs and symptoms. Osteomas most commonly become symptomatic in the second to fifth decade in life, but orbital involvement has rarely been reported in patients aged 18 years and younger. (2,6-10) We report a case of a frontal sinus osteoma with orbital extension in a 13-year-old girl.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
9/79. myoepithelioma of the lacrimal gland: report of a case with potentially malignant transformation.myoepithelioma of the lacrimal gland is extremely rare and only four cases, one of which was malignant, have been reported in detail. The present report describes a case of lacrimal gland myoepithelioma in a Japanese male with histological features suggestive of potentially malignant transformation. The excised tumor consisted of two components, a central nodular component and a peripheral component surrounding the former. These components were separated by a fibrous tissue. Microscopically, both components were comprised almost entirely of spindle-shaped cells, but with some epithelioid cells containing glycogen granules. Extracellular spaces in the peripheral component were filled with eosinophilic materials with the occasional crystalloid structures, which were immunoreactive for collagen type i. Neoplastic cells were immunoreactive focally for vimentin and S-100, but negative for cytokeratins, epithelial membrane antigen, muscle actin, smooth muscle actin, desmin, myosin, and glial fibrillary acidic protein. The neoplastic cells in the central component showed nuclear pleomorphism and atypia with a higher frequency of mitotic figures, and higher labelings of proliferation markers than those in the peripheral component. Neither invasion, necrosis, nor hemorrhage was observed in the tumor. From these findings we proposed a diagnosis of potentially malignant myoepithelioma.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
10/79. Fractionated, three-dimensional, planning-assisted proton-radiation therapy for orbital rhabdomyosarcoma: a novel technique.PURPOSE: Most children with orbital rhabdomyosarcoma will survive their disease. However, conventional photon-radiation treatment, as part of multimodality therapy, results in varying degrees of long-term functional and cosmetic side effects. This report introduces external beam proton radiation therapy (PRT) as a conformal, three-dimensional planned radiation technique for this disease, analyzes normal tissue dosimetry, and describes the technique's application in the first 2 patients. MATERIAL AND methods: Between January 1995 and February 1996, 2 patients underwent PRT following biopsy and chemotherapy for orbital rhabdomyosarcoma. Fifty and 55 cobalt Gray Equivalent (CGE) were delivered to the gross tumor volume and 40 CGE to clinical target volumes in both patients. A relative biologic effectiveness (RBE) of 1.1 was utilized to correlate proton dose calculations with CGE. To achieve dose conformity, a "patch technique" was utilized, where target regions were divided into segments, each treated by a separate proton field. Dose-volume histograms were obtained for target and nontarget regions, including lens, bony orbit, pituitary gland, optic chiasm, optic nerves, lacrimal gland, and ipsilateral frontal and temporal lobes. RESULTS: At 3.4 and 2.5 years after PRT, both patients are clinically and radiographically free of disease. visual acuity remains excellent, without signs of cataract formation; pituitary function is normal; cosmetically, only mild enophthalmos is noticeable. Doses to 90%, 50%, and 5% of lens volume were kept at less than 1%, less than 2%, and less than 8%, respectively. Fifty percent of lacrimal gland volume received less than 36% of the prescribed dose and 50% of the volume of the optic chiasm, pituitary gland, and hypothalamus were restricted to less than 2%. Proton conformity to orbital contents resulted in between 9% and 36% of the prescribed dose reaching the ipsilateral temporal and frontal lobes immediately adjacent to bony orbit (5% volume). CONCLUSION: PRT can offer excellent sparing of lens and selected intraorbital and ocular normal structures, while maintaining conformal target-dose coverage. The steep dose gradient beyond the orbit minimizes irradiation of normal brain parenchyma, with almost complete sparing of the pituitary gland. Reduction of integral irradiation exposure of the periorbital region will, hopefully, reduce the risk of second malignancy later in life. Reduced radiation dose to specific organs in close proximity to, but not part of the target region promises improved functional outcome and better cosmesis for childhood cancer survivors.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
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