Cases reported "Orbital Neoplasms"

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1/82. color Doppler image of central retinal artery of eyes with an intraconal mass.

    PURPOSE: Retinal ischemia secondary to hypoperfusion of the central retinal artery is recognized as one factor that may contribute to the development of loss of vision in eyes with intraorbital tumors. We study intraorbital tumors which produce motility disturbances and visual problems by color Doppler imaging to evaluate this factor. methods: We examined the central retinal artery velocities of 3 patients with disc edema caused by intraconal masses (2 cavernous hemangiomas and 1 presumed optic nerve glioma) via color Doppler imaging. RESULTS: The time-velocity waveform demonstrated abnormally high vascular resistance in the central retinal artery of all affected eyes in the primary position compared with the normal waveform seen in the other eyes. We compared the pulsatility index of eyes with an intraconal mass and contralateral, control eyes using Student's t test for paired samples and significant differences were noted between both groups (p< 0.01). CONCLUSIONS: Intraconal tumors could produce increased pressure in the optic nerve sheath and the optic nerve tissue which could be associated with impaired retinal and optic nerve blood flow and the subsequent amaurosis encountered with intraorbital tumors.
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ranking = 1
keywords = glioma
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2/82. hypnosis instead of general anaesthesia in paediatric radiotherapy: report of three cases.

    PURPOSE: This report proposes hypnosis as a valid alternative to general anaesthesia for immobilisation and set-up in certain cases in paediatric radiotherapy. methods: We report three cases of children who underwent radiotherapy in 1994 and were treated using hypnosis for set-up during irradiation. The first and the second were two cases of macroscopic resection of cerebellar medulloblastoma in which craniospinal irradiation was necessary, while the third patient suffered of an endorbitary relapse of retinoblastoma previously treated with bilateral enucleation, radiotherapy and chemotherapy; in this last situation the child needed radiation as palliative therapy. hypnosis was used during treatment to obtain the indispensable immobility. Hypnotic conditioning was obtained by our expert psychotherapist while the induction during every single treatment was made by the clinician, whose voice was presented to the children during the conditioning. RESULTS: Every single fraction of the radiation therapy was delivered in hypnosis and without the need for narcosis. CONCLUSIONS: hypnosis may be useful in particular situations to prepare paediatric cancer patients during irradiation, when lack of child collaboration might necessitate the use of general anaesthesia and when anaesthesia itself is not possible.
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ranking = 59.816813407306
keywords = retinoblastoma
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3/82. Fine structure of a radiation-induced osteogenic sarcoma.

    An osteogenic sarcoma arose in the right orbit of a 7-year-old boy some 5 years after the right orbit had been treated by four courses of radiotherapy (total dose approximately 13,000 rads) for a multicentric retinoblastoma. death occurred 6 months after the orbital tumor was first detected. Study of the orbital tumor by electron microscopy revealed a cell population of varied morphology in which two main types were identified. In one group, the cells were large with radiolucent cytoplasm, which contained long branching segments of rough endoplasmic reticulum. In the second group, the cells were smaller with irregular nuclei and an electron-dense cytoplasm, which contained short segments of dilated rough endoplasmic reticulum and numerous mitochondria. The first group of cells closely resembled osteoblasts, while the second group had some features of osteoclasts or their percursors. The branching processes of the tumor cells were separated by an amorphous ground substance, which contained collagen-like fibrils and hydroxyapatite crystals. Crystal deposition was in some instances in close relation to extracellular membrane-bound vesicles.
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ranking = 59.816813407306
keywords = retinoblastoma
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4/82. Orbital ganglioglioma arising from ectopic neural tissue.

    PURPOSE: To report a case of neonatal orbital ganglioglioma originating from ectopic neural tissue. METHOD: Case report. RESULTS: An African-American male presented at birth with proptosis and expansion of the left orbit. A discrete soft-tissue mass was located inferolateral to the globe, which proved to be a ganglioglioma. CONCLUSION: The tumor is presumed to have originated from ectopic neural tissue in the orbit. Although infrequent, this tumor should be included in the differential diagnosis of neonatal orbital neoplasms.
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ranking = 6
keywords = glioma
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5/82. Nonocular cancer in retinoblastoma survivors.

    From a review of the records of 2,302 patients with retinoblastoma collated from CPMC and AFIP, it was found that retinoblastoma patients who survived their original eye cancer have a high incidence of second nonocular malignancies. The second neoplasms occur almost exclusively (97.5%) in patients who have had bilateral retinoblastoma, although retinoblastoma is much more commonly unilateral. The second neoplasms have appeared between 1 and 42 years after the successful treatment of retinoblastoma and have been fatal in approximately 85% of cases. Seventy-one percent of patients develop tumors in the field of the radiation beam; many of these were following treatment with low doses of radiation and after short latent periods. Nineteen percent of patients develop tumors clearly out of the field of radiation (eg, osteogenic sarcoma of the femur). retinoblastoma patients appear to be unusually radio-sensitive to low doses of radiation and develop tumors in the field of radiation following treatment of 3,500 rads. They have a high incidence of nonocular tumors clearly distant from the radiation beam develop nonocular malignancies even if no radiation has been given. The incidence of multiple malignancies in patients successfully treated for retinoblastoma appears to be higher than for any other primary malignancy whether they receive radiation or not.
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ranking = 598.16813407306
keywords = retinoblastoma
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6/82. Right temporal lobe glioblastoma presenting in the left orbit. Case report.

    Dissemination of gliomas outside the central nervous system without preceding neurosurgery is a rare phenomenon. Glial neoplasms presenting as bone lesions are even more rare. A case of glioblastoma multiforme (GBM) with initial presentation in the orbit following a single generalized seizure is described. Signs of intracranial hypertension resulted from subarachnoid tumor invasion. The patient was treated with whole-dose radiation therapy but survived for only 6 months following the initial presentation. An autopsy revealed a right temporal GBM with extensive subarachnoid spread and invasion in the left orbit and skull base. The literature on dissemination of primary tumors of the brain is reviewed.
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ranking = 37.354951203212
keywords = glioblastoma, glioma
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7/82. Orbital leiomyosarcoma after retinoblastoma.

    patients with the inherited, bilateral form of retinoblastoma have an increased incidence of osteogenic sarcoma such that the mortality from the secondary tumor exceeds that of the initial bilateral retinoblastoma. We report a 29-year-old male survivor of bilateral retinoblastomas originally diagnosed at 8 months of age, whose treatment eventually included bilateral enucleation, bilateral orbital radiation, and systemic chemotherapy. At age 26, a tumor removed from his right maxillary sinus was diagnosed as fibroma. At age 29, he developed an inferior orbital mass that extended into the right maxillary sinus. A biopsy and comparison with the previous maxillary sinus mass revealed both lesions to be leiomyosarcoma. Both light and electron microscopy supported the diagnosis. The patient has survived treatment with orbital exenteration and maxillectomy combined with postoperative radiation to the right orbital-maxillary area. This appears to be the fourth case of leiomyosarcoma in the third decade of life in a male patient with a previously irradiated orbit after enucleation for bilateral retinoblastoma. leiomyosarcoma appears to be another orbital tumor associated with bilateral retinoblastoma.
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ranking = 538.35132066575
keywords = retinoblastoma
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8/82. Post-transplant lymphoproliferative disorder after autologous peripheral stem cell transplantation in a pediatric patient.

    Post-transplant lymphoproliferative disorder (PTLD) is a complication of allogeneic bone marrow transplantation (BMT). Rare cases of PTLD after autologous BMT have been reported only in adults. This case report is the first to describe PTLD in a pediatric patient after autologous peripheral stem cell transplantation (PSCT). This 2-year-old male with stage IV neuroblastoma underwent autologous PSCT. The post-PSCT course was complicated by fever with hematochezia and a lung mass. On day 94 post PSCT, colonoscopy revealed an ulcer due to a PTLD, monomorphic type, B cell phenotype, associated with Epstein-Barr virus. Fine needle aspiration identified the lung mass as neuroblastoma. PTLD can occur in pediatric autologous PSCT recipients, and may occur more frequently in autologous grafts manipulated by T cell depletion or CD34 cell selection.
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ranking = 26.419584457511
keywords = neuroblastoma
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9/82. Visual electrophysiology in children with tumours affecting the visual pathway. case reports.

    In 9 children (8-14 years of age) with orbital, suprasellar or postchiasmal tumours, visual loss was studied by visual electrophysiology in relation to ophthalmologic and neuroimaging findings. Pattern electroretinography (PERG) and pattern visual evoked potentials (PVEP) to full and half-field pattern-reversal stimulation were recorded and PERG and PVEP changes were related to the tumour location. PERG wave P50 attenuation was found associated with the central retinal dysfunction in the child with orbital rhabdomyosarcoma; PVEP wave P100 delay was associated with the optic nerve dysfunction in a child with retrobulbar chondrosarcoma and in a child with optic nerve glioma; PVEP wave P100 asymmetry was associated with the crossed fibers dysfunction in a child with hypothalamic germinoma, and PVEP wave P100 uncrossed asymmetry was associated with postchiasmal dysfunction in children with postchiasmal tumours (one with pilocytic astrocytoma and two with angioma). On the other hand, normal PERG suggested that there was no central retinal dysfunction in a child with pleomorphic adenoma of the lacrimal gland, and normal PVEP to full and half-field stimulation excluded visual pathway dysfunction at the chiasm in a child with suprasellar arachnoidal cyst. Follow-up was useful in indicating whether visual dysfunction was progressive or not. We conclude that PERG and PVEP findings contributed to understanding whether the dysfunction originated was at the retina, in the optic nerve, chiasm or postchiasmal pathway.
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ranking = 1
keywords = glioma
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10/82. Orbital malignant fibrous histiocytoma following irradiation for retinoblastoma.

    PURPOSE: An unusual case is reported of orbital malignant fibrous histiocytoma that developed after irradiation for retinoblastoma. methods: Case report. RESULTS: A 5-month-old girl underwent enucleation of the left eye, external beam irradiation of the right eye, and systemic chemotherapy for bilateral sporadic retinoblastoma. At age 17 years, a malignant fibrous histiocytoma developed in the medial aspect of the orbit and nasal cavity. After attempted excisional biopsy, she underwent craniofacial resection, orbital exenteration, maxillectomy, and tracheostomy. The patient was alive 12 months postoperatively without local or metastatic disease. CONCLUSIONS: Although orbital fibrous histiocytoma occurs usually as a primary tumor of adulthood, it can also develop as a secondary tumor after irradiation for retinoblastoma.
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ranking = 418.71769385114
keywords = retinoblastoma
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