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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

With modern techniques it is usually possible to deliver a high dose of radiation to an orbital tumour without destroying vision. Local cure of lymphoma is possible with no risk of complications. Rhabdomyosarcoma now has a very good prognosis if treated energetically by radiotherapy and chemotherapy. Radiotherapy also has a part to play in the management of lacrimal gland tumours, melanoma, and metastases. A recent series of cases provides evidence that radiotherapy may be the best treatment for some cases of pseudotumour.
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PMID:Role of the radiotherapist in orbital disease. 29 17

Orbital abnormalities encountered in the pediatric population differ substantially from those found in adult patients. Retinoblastoma, the most serious intraocular tumor, is often difficult to diagnose, but use of computed tomography (CT) (which reveals the characteristic focal calcification) and magnetic resonance (MR) imaging allows this tumor to be differentiated from pseudogliomas, such as Coats disease, and retrolental fibroplasia. CT and MR imaging help in the differentiation of orbital cellulitis from preseptal, lacrimal, and eyelid infectious processes and of orbital pseudotumor from Graves disease. In cases of orbital trauma, CT is excellent for detecting orbital fractures and metallic foreign bodies; MR imaging is better for depicting ocular and optic nerve injuries. Both modalities allow the differentiation of rhabdomyosarcoma from dermoid, cavernous hemangioma, and lymphangioma and provide helpful information for the diagnosis of many other tumors. Since CT and MR imaging have widely expanded the capabilities of orbital imaging, it is more important than ever before for radiologists to understand pediatric orbital disease.
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PMID:CT and MR imaging of the pediatric orbit. 160 40

We reviewed patient records of 99 consecutive orbital exenterations performed between 1969 and 1988. Patients ranged in age from 2 to 86 years (mean, 55.9 years). Classification of cases on histopathologic criteria showed 32 exenterations were performed for squamous cell carcinoma originating in the paranasal sinus (13), skin (12), conjunctiva (six), and lacrimal sac (one). Orbital exenteration was performed for treatment of other epithelial malignancy in basal cell carcinoma (eight), sebaceous carcinoma (six), adenoid cystic carcinoma (five), undifferentiated carcinoma (four), adenocarcinoma (two), intraepithelial carcinoma of the conjunctiva (two), benign mixed tumor (one), and transitional cell carcinoma (one). Exenterations were performed for melanoma of the conjunctiva (ten), nasosinus (three), skin (two), orbit (two), and choroid (one). Exenterations were also performed as treatment for mucormycosis (five), meningioma (three), fibrosarcoma (two), rhabdomyosarcoma (two), hemangiopericytoma (two), orbital cellulitis (one), fibrous histiocytoma (one), schwannoma (one), lymphangioma (one), benign lymphoepithelial lesion (one), and undifferentiated malignancy (one).
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PMID:A 20-year series of orbital exenteration. 195 84

A retrospective review of 376 pediatric orbital tumor cases seen at the Ankara University Eye Clinic between 1963 and 1993 was undertaken. The diagnosis was made histologically in every case. Secondary tumors accounted for 127 cases (33.8%), cystic lesions for 82 cases (21.8%), rhabdomyosarcomas for 23 cases (6.1%), vascular lesions for 21 cases (5.6%), inflammatory lesions for 21 cases (5.6%), lymphoma and leukemias for 18 cases (4.8%), other mesenchymal tumors for 11 cases (2.9%), metastatic tumors for 5 cases (1.3%), traumatic foreign bodies for 2 cases (0.5%), and lacrimal fossa lesions for 1 case (0.3%). The most common benign orbital tumors were the cystic lesions. The most common primary malignant tumor was rhabdomyosarcoma. Overall, the most frequent orbital lesion was the secondary orbital invasion of retinoblastoma.
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PMID:Pediatric orbital tumors in Turkey. 854 Dec 61

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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PMID:Visual electrophysiology in children with tumours affecting the visual pathway. Case reports. 1120 May 46

Four patients with relatively large orbital tumors are described. These patients had superomedial rhabdomyosarcoma, inferomedial cavernous hemangioma, lateral primitive neuroectodermal tumor, and superior neurofibroma, respectively. Continuing enlargement and encroachment upon vital ocular structures and suspicion of malignancy warranted surgical intervention. Following exposure of the anterior portions of these tumors, a cryoprobe was used for the complete removal of the mass. Cryoextraction greatly facilitates the excision of well-defined, solid, encapsulated, benign or malignant tumors excluding the lacrimal gland and thus minimizes trauma to adjacent tissues. More importantly, the tumor can be removed intact without capsular rupture and risk of tumor spill-over or hemorrhage. At the histopathological level, the application of a cryoprobe did not alter the microscopic structures of the lesions and did not cause any difficulty for histopathologic interpretation.
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PMID:Cryoextraction in the management of orbital tumors. An old technique revisited. 1204 27

Orbital diseases are not common, but they are closely related with various systems of the body. A significant progress in orbital diseases has been achieved both in China and abroad in the last several years. The study of etiology of thyroid-associated ophthalmopathy and the establishment of an experimental animal model have provided the hope for a novel treatment of this disease; the studies of rhabdomyosarcoma and tumors of lacrimal gland at the molecular level have provided the basis for the development of a novel treatment; new plaque radiotherapy and application of various kinds of seed have enriched the treatment and improved the prognosis of orbital tumors. Some significant progresses, for example, a new viewpoint of meningioma of the optic nerve sheath and glioma, improved approach for entering the orbit and complex treatment of malignant tumors have revealed a new path for the diagnosis and treatment of orbital disease.
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PMID:[Recent advances in the studies of orbital diseases]. 1619 32

An 11-year-old boy presented with right medial canthal swelling. He was initially diagnosed with dacryocystitis and treated with oral antibiotics, followed by incision and drainage of a presumed lacrimal sac abscess. Rapid recurrence of the swelling led to further clinical evaluation, including a maxillofacial CT, which revealed an extensive nasal and orbital mass that was consistent with embryonal rhabdomyosarcoma on histopathologic analysis. This represents an unusual case of rhabdomyosarcoma manifesting as acute dacryocystitis. Rhabdomyosarcoma should be considered in the differential diagnosis for acquired nasolacrimal obstruction and dacryocystitis in the pediatric population.
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PMID:Rhabdomyosarcoma masquerading as acute dacryocystitis. 1630 28

The authors describe a case of orbital rhabdomyosarcoma masquerading as a lacrimal mucocele in a newborn infant. Rhabdomyosarcoma is one of the few life-threatening diseases that an ophthalmologist may be the first to diagnose. This case highlights the importance of close monitoring of such presentations in the newborn.
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PMID:Orbital rhabdomyosarcoma masquerading as a mucocele. 1882 4

Exophthalmus is the leading sign of space-occupying lesions of the orbit. Patients may further present with lid swelling, impaired ocular motility and optic neuropathy including a relative afferent pupillary defect, compressive optic disc edema or optic atrophy. Orbital tumors can be classified into various categories depending on the etiology, as lymphoproliferative lesions (in particular non-Hodgkin's lymphoma as the most common malignant orbital tumor of adulthood), optic nerve and meningeal lesions, lacrimal gland lesions, secondary orbital tumors which extend to the orbit from neighboring structures and metastases. Slightly less common are vasculogenic and cystic lesions including cavernous hemangioma as the most common benign orbital tumor of adulthood and dermoid cysts as the most common benign orbital tumor of childhood. Rhabdomyosarcoma is the most common malignant orbital tumor of childhood but has a low total incidence. Orbital tumors might not only cause symptoms like pain, diplopia and loss of visual acuity but may also lead to esthetically disfiguring changes. Particular attention should be paid to underlying systemic diseases and generalized tumor diseases. This article illustrates the approach to a detailed clinical and neuroradiological assessment which is mandatory for the care of orbital tumor patients.
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PMID:[Clinical and neuroradiological diagnostics of orbital tumors]. 2169 5


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