1/15. 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 = 1keywords = life (Clic here for more details about this article) |
2/15. Fibrous dysplasia of the orbit in an infant.Fibrous dysplasia is an idiopathic fibro-osseous lesion of the skeletal bones. These uncommon osseous lesions usually manifest within the first two decades of life. Its occurrence during infancy is extremely uncommon, and we describe here a case of fibrous dysplasia involving the orbit in a 9-month-old male child.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
3/15. blood stem-cell transplantation for chronic active Epstein-Barr virus with lymphoproliferation.A boy with chronic active Epstein-Barr virus infection (EBV) developed bilateral exophthalmos by infiltrating EBV-infected natural-killer cells during combined chemotherapy. This unusual presentation resolved completely after allogeneic bone-marrow transplantation, and EBV-infected peripheral T and natural-killer cells were also eradicated.- - - - - - - - - - ranking = 4keywords = life (Clic here for more details about this article) |
4/15. Juvenile xanthogranuloma of the orbit in an adult.We present a case of juvenile xanthogranuloma (JXG) with unilateral involvement of the orbit and eyelid and proptosis, histologically confirmed in a 32-year-old man with a 1-year history of a pansinusitis and dacryoadenitis with rhinitis. Nine months later an infiltration of the anterior upper part of the right orbit and right eyelid appeared. Computed tomography scan and magnetic resonance imaging studies confirmed the presence of pansinusitis and infiltration. The patient underwent a blepharoplasty and excision of the infiltrated tissues of the orbit, eyelid, and levator muscle. hematoxylin-eosin and immunohistochemical studies revealed features consistent with a diagnosis of JXG (Touton giant cells). JXG, a non-Langerhans'-type benign proliferation, is a rare condition in adulthood.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
5/15. Angiomatoid melanoma: a novel pattern of differentiation in invasive periocular desmoplastic malignant melanoma.A case of locally invasive, long-standing desmoplastic and amelanotic malignant melanoma is described in an 84-year-old man. Histologic examination of the involved periorbital tissue showed neoplastic foci exhibiting a novel pattern reminiscent of microvascular proliferation. These regions were characterized by malignant, S-100-positive tumor cells lining vessel-like spaces in transverse sections and forming tubuler-like structures in longitudinal sections. Recent data indicate that melanoma cells may express genes and patterns of differentiation in vitro akin to endothelial cells. Because angiosarcoma often involves facial and scalp skin of elderly individuals, awareness of angiomatoid differentiation in melanoma has important diagnostic implications. HUM PATHOL 31:1520-1522.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
6/15. Meningeal solitary fibrous tumor as an unusual cause of expohthalmos: case report and review of the literature.OBJECTIVE AND IMPORTANCE: We report an extremely rare case of a patient with meningeal solitary fibrous tumor with orbital involvement presenting as unilateral exophthalmos. This rare tumor should be considered in the differential diagnosis for aggressive dural-based lesions. CLINICAL PRESENTATION: A 54-year-old man presented with a protruded eyeball on the right side and left hemiparesis. Computed tomography and magnetic resonance imaging demonstrated a huge mass, markedly enhanced in and around the right anterior clinoid process, which extended to the orbit and middle cranial fossa. cerebral angiography revealed a richly vascular tumor fed by branches of both the right external and internal carotid arteries. A presumptive diagnosis of meningioma or hemangiopericytoma was considered. INTERVENTION: The tumor was exposed through an orbitozygomatic approach. At surgery, the tumor was grossly firm to hard and had destroyed the dura, orbital roof, anterior clinoid process, temporal bone, and muscle. Histological analysis revealed that the tumor was composed of spindle-cell proliferation in a collagen-rich background, but it exhibited regional variations. The vascular network demonstrated irregular vascular lumina with a "hemangiopericytoma-like" pattern. Histological features included high cellularity and a high degree of pleomorphism. Immunohistochemical analysis of the tumor demonstrated diffuse positive staining for CD34 and vimentin. The tumor displayed no positive staining for cytokeratin, epithelial membrane antigen, glial fibrillary antigenic protein, S-100 protein, and factor xiii. CONCLUSION: Meningeal solitary fibrous tumor is considered a unique pathological entity. Wider use of immunohistochemical screening should enable analysis of the real incidence of these tumors; larger series and longer follow-up duration will allow conclusions to be drawn regarding treatment and prognosis. Differential diagnosis is discussed and the literature is reviewed.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
7/15. Intermittent visual loss and exophthalmos due to the Blue rubber bleb nevus syndrome.PURPOSE: To describe the occurrence of intermittent visual loss in a patient with orbital hemangioma due to the blue rubber bleb nevus syndrome. methods: Case report. RESULTS: A 70-year-old woman complained of intermittent blurred vision combined with pressure behind her left eye occurring after head bending or Valsalva maneuvers. Examination revealed 2 mm of enophthalmos on the left. After downward bending of the head the eye became exophthalmic by 12 mm and visual acuity decreased to finger counting. Neuro-ophthalmologic examination was otherwise normal. magnetic resonance imaging revealed an intraconal hemangioma. Bluish, soft, rubber-like, compressible and slowly refilling hemangiomas on the inferior lip, sublingually, on the hand, and vagina led to the diagnosis of BRBNS. CONCLUSIONS: Intermittent visual loss may be the presenting symptom of an orbital hemangioma in BRBNS. Recognition of this rare condition is important because of its potentially life-threatening complications.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
8/15. Rosai-Dorfman disease presenting with isolated bilateral orbital masses: report of two cases.Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is a rare benign idiopathic proliferative disease of phagocytic histiocytes. Approximately 80% of patients present with painless massive cervical lymphadenopathy. Isolated extranodal involvement is relatively uncommon. Two cases of Rosai-Dorfman disease are reported: one with isolated bilateral orbital involvement and one with marked cervical lymphadenopathy and multiple dural-based and intraventricular masses.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
9/15. Non-Hodgkin's orbital lymphoma.BACKGROUND: The non-Hodgkin's lymphomas (NHL) are a group of neoplasms characterized by proliferation of malignant lymphocytes. patients with NHL have a wide variety of presenting signs and symptoms, depending largely on the site of involvement and aggressiveness of the disease. Many organs in the body may be affected, including the eye and orbit. CASE REPORT: A 47-year-old male with a 3-year history of stage IV non-Hodgkin's lymphoma who had undergone recent monoclonal antibody therapy presented with a complaint of blur in the left eye with occasional diplopia. Significant ocular findings of the left eye included ptosis, mild proptosis, increased intraocular pressure, and choroidal folds. magnetic resonance imaging of the orbit revealed an orbital lymphoma that completely resolved after 2400 rads of external beam irradiation therapy. Eight months later, the patient developed a secondary radiation retinopathy. CONCLUSION: The prevalence of NHL is on the rise, and orbital involvement may occur at any time during the course of the disease. The standard treatment for non-Hodgkin's orbital lymphoma is external beam irradiation therapy, although the optimal dose for obtaining local tumor control without complications remains to be determined. Non-Hodgkin's lymphoma, orbital lymphoma, and various treatment options are discussed.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
10/15. Ocular pulsation in neurofibromatosis. A clinical/neuroradiologic correlation.A 61-year-old woman presented with early cataract formation in an eye which had been pulsating synchronously with her heart beat all of her life. This report presents the first, to our knowledge, three dimensional reconstruction CT images of this form of neurofibromatosis 1. It also documents by MRI and MRA the association of an asymptomatic carotid-ophthalmic junction aneurysm ipsilateral to the sphenoid dysplasia.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
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