Cases reported "Orbital Neoplasms"

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1/72. Prenatal sonographic features of embryonal rhabdomyosarcoma.

    We describe a case of fetal rhabdomyosarcoma detected during the third trimester of pregnancy by prenatal sonography. At 33 weeks' gestation, sonography performed because of suspected polyhydramnios showed a solid mass of 120 x 54 mm arising from the anterior wall of the fetal thoracic cage. Another mass within the left maxillary area which originated from the left orbital floor was also detected. In the abdomen, there were multiple round masses in and around the liver. As the previous scan at 28 weeks had appeared normal, the multiple masses which became visible and enlarged rapidly in different locations led us to believe that there was fetal cancer. The most likely diagnosis was rhabdomyosarcoma (which was later confirmed), because it is the most prevalent soft-tissue tumor in children and may develop within or outside muscle anywhere in the body and at any age. Two other reported cases which were detected by prenatal ultrasound examination are also discussed.
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2/72. Immunohistochemical examination of an orbital alveolar soft part sarcoma.

    BACKGROUND: A 32 year-old male patient had a 6-week history of left-sided proptosis. Computer tomography revealed a 16x15x15 mm smooth and well-defined mass between the optic nerve and the medial and superior rectus muscles in the left orbit. methods: The tumour was excised via a cranio-medial orbitotomy approach. RESULTS: Histopathological examination, immunohistochemistry and electron microscopy findings were consistent with an alveolar soft part sarcoma. Immunohistochemical staining showed positive immunoreactivity for neuronespecific enolase, vimentin, p53 (30%), p21 (10%) and cyclin d1 (20%), and negative immunoreactivity for CD45, cytokeratins, S-100 protein, glial fibrillary acidic protein, synaptophysin, chromogranin, calcitonin, serotonin, thyreoglobulin, desmin, myosin, actin, HMB-45, pRB, p16 and BCL-2. The growth fraction of the tumour cells was 3%. At examination 4 years after surgical excision, there was no evidence of local recurrence or for metastases. CONCLUSION: Alveolar soft part sarcoma of the orbit is a rare malignant tumour best controlled by surgery. The unpredictable behaviour of these neoplasms, however, indicates the need for long-term follow-up.
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keywords = alveolar
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3/72. Implant brachytherapy: a novel treatment for recurrent orbital rhabdomyosarcoma.

    BACKGROUND: Orbital rhabdomyosarcoma is the most common primary malignancy of the orbit in childhood. Tumor resection and exenteration were the preferred treatment modalities for rhabdomyosarcoma. In the past 20 years, however, combined local radiation and systemic chemotherapy have shown excellent survival results. Tumor recurrence after any of the aforementioned therapies is almost always fatal. We have developed a novel treatment for recurrent disease that has resulted in long-term survival for three patients. methods: Three patients with recurrent orbital rhabdomyosarcoma were previously treated with primary radiation and chemotherapy. At the time of recurrence, exenteration and localized brachytherapy were performed. An individually molded poly(methylmethacrylate) (Lucite; E. I. du Pont de Nemours & Co., Wilmington, Del.) device loaded with radioactive iodine seeds delivered localized high-dose radiation, 6000 cGy over 6 days, to the orbit without irradiating the brain. RESULTS: All patients are alive and free of disease with follow-up ranging from 4 years and 4 months to 8 years and 4 months. CONCLUSION: A novel technique of delivering localized radiation to the orbit of three children with recurrent orbital rhabdomyosarcoma appears curative.
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keywords = rhabdomyosarcoma
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4/72. Membranous nephropathy and orbital malignant tumor.

    A 7-year-old girl with membranous nephropathy is reported who suffered 16 months later from an orbital rhabdomyosarcoma. proteinuria disappeared at the time of the remission of the tumor. Membranous nephropathy as paraneoplastic syndrome is exceptional in children, since only two other cases associated with a solid tumor have been reported in the literature.
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keywords = rhabdomyosarcoma
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5/72. Orbital rhabdomyosarcoma presenting as an apparent orbital subperiosteal abscess.

    We present a patient with acute sinusitis whose CT scan showed a ring-enhancing lesion within the orbit typical of an orbital subperiosteal abscess. On exploration of the orbit, there was no pus present but a tumour was found, which on histological examination was found to be a rhabdomyosarcoma. The clinical signs and CT characteristics of orbital subperiosteal abscesses and rhabdomyosarcomas are discussed. A ring-enhancing lesion within the orbit on CT scan should not be regarded as being pathognomonic for a subperiosteal abscess and the possibility of other diagnoses should be considered.
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keywords = rhabdomyosarcoma
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6/72. corneal perforation due to late radiation therapy-induced corneal necrosis--clinicopathological correlation.

    A clinicopathological correlation is made between corneal perforation and late radiation therapy-induced corneal necrosis in a male adolescent treated for orbital rhabdomyosarcoma.
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ranking = 0.16666666666667
keywords = rhabdomyosarcoma
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7/72. 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.
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keywords = rhabdomyosarcoma
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8/72. Orbital abscess masquerading as a rhabdomyosarcoma.

    Although orbital cellulitis is the most common cause of acute-onset proptosis with inflammatory signs in a child, the clinician should always be alert to the possibility of rhabdomyosarcoma. We describe an unusual presentation of acute-onset nonaxial proptosis of the left orbit without sinus disease or systemic toxicity in a 6-year-old boy. Our clinical differential diagnosis included orbital cellulitis, metastatic disease, capillary haemangioma, lymphangioma with cyst, ruptured dermoid cyst, and orbital rhabdomyosarcoma. Only after orbital biopsy and subsequent microbiologic confirmation were obtained was a diagnosis of chronic orbital abscess tenable. Features in our patient included paucity of symptoms and signs of inflammation. This case illustrates the difficulty in differentiating a chronic orbital infection from orbital rhabdomyosarcoma on the basis of clinical, laboratory, and orbital imaging findings. Possible causes of this unusual presentation are discussed.
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ranking = 1.1666666666667
keywords = rhabdomyosarcoma
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9/72. 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 = 0.16666666666667
keywords = rhabdomyosarcoma
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10/72. Embryonal rhabdomyosarcoma of the orbit in a 38-year-old woman.

    Primary embryonal rhabdomyosarcoma of the orbit is an extremely rare tumor in adults. A 38 year old female patient with immunohistochemically proved embryonal rhabdomyosarcoma of the right orbit is reported. The histologic types, diagnosis, management and out come of rhabdomyosarcoma is discussed.
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ranking = 1.1666666666667
keywords = rhabdomyosarcoma
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