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

Rhabdomyosarcoma, the most common primary malignant childhood orbital tumor, is composed of neoplastic striated muscle cells (rhabdomyoblasts) in various stages of differentiation and in patterns suggestive of neoplastic analogs of normal muscle embryogenesis. Orbital rhabdomyosarcoma is most commonly seen in children and adolescents, the average age of onset of symptoms being 7.8 years. The tumor usually presents as a rapidly evolving exophthalmos, often associated with drooping of the upper eyelid. A mass is palapable in only 25% of cases, loss of central vision at the time of presentation is uncommon, and laboratory studies are often of little help in diagnosis. The best diagnostic aid is a high index of suspicion whenever one sees a rapidly progressive exophthalamos in a child. Orbital rhabdomyosarcoma is almost always of the embryonal type, believed to originate in the orbital soft tissues from undifferentiated pluripotential embryonic mesenchyme. In the past, orbital exenteration has been the primary therapy. Review of 162 literature cases of orbital rhabdomyosarcoma, generally treated by unassisted surgery, revealed that only 25% of the patients survived 3 or more years. Recently, it has been shown that radiation therapy, alone or combined with chemotherapy, can be successful. A multidisciplinary approach, utilizing surgery, radiation therapy and chemotherapy has also been advocated. Both approaches appear to offer greater survival than unassisted orbital exenteration. The possibility of primary radiation therapy is extremely promising; if it becomes increasingly effective, a mutilating surgical procedure may become obsolete.
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PMID:Ophthalmic striated muscle neoplasms. 79 29

A case is reported of a primary rhabdomyosarcoma occurring in the right subfrontal region of a 16 year old girl. The patient suffered from dull frontal headache and proptosis for three months before hospitalization. The circumscribed and demarcated neoplasm involved the dura mater, and invaded the frontal sinus and roof of the orbit on the same side. The pathogenesis of the tumour is thought to be related to aberrant differentiation of unstable mesenchyme. A suggestion is made that the "medullomyoblastoma" should be classified as a type of neoplasm of mixed mesenchymal and neuroepithelial origin.
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PMID:Primary intracranial rhabdomyosarcoma producing proptosis. 95 May 63

Fourteen cases of orbital rhabdomyosarcoma have been studied. Clinical presentation was by way of short history, fast growing proptosis, inflammatory signs and epistaxis. Computerised tomography showed only a mass lesion without bony involvement and was very useful in follow up. Ultrasonography revealed pseudo-encapsulation of the tumour which is postulated as possibly specific of Rhabdomyosarcoma due to its fast growth, cellular content and oedema fluid. A survival rate of 21% over a 3 year period has been achieved by combined mode therapy consisting of surgery, radiation and chemotherapy.
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PMID:Clinical, ultrasonographic and CT evaluation of orbital rhabdomyosarcomas with management. 184 88

We report here a specific case of metastatic brain tumor orienting from rhabdomyosarcoma. An 11-year-old boy came to our hospital with complaints including headache, exophthalmos and tumors in the right frontal and left occipital region. 6 months previously subtotal removal of rhabdomyosarcoma in his right foot had been performed. CT scan demonstrated tumors in the right orbit, right frontal region and left occipital region. The tumors existed bilaterally in the skull and markedly enhanced by contrast medium. But the changes of the cranium were mild, only the dipole was slightly dilated. MRI showed that the tumors were extraaxial masses. Right external carotid angiogram revealed numerous tumor vessels fed by the meningeal artery. On April 3rd, biopsy was performed at the right frontal region. The tumor had spread in the epidural space but the cortical surface seemed to be intact. Histologically, the tumor consisted of small round cells and revealed multinuclear giant cells. It involved the skull bone, dura mater and subdural tissues. Accordingly the tumor was diagnosed as rhabdomyosarcoma. Although tumor increased rapidly, the intracranial region did not change remarkably. It was an interesting phenomenon. Eventually, rhabdomyosarcoma metastasized to the lung, and the patient died due to respiratory distress.
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PMID:[A case of brain metastasis of rhabdomyosarcoma in a child]. 189 Oct 55

Embryonal rhabdomyosarcoma of orbit presenting as a case of rapid proptosis of the right eye is reported in a 4 year old male child. There was no evidence of recurrence during a follow up of 6 months. Rhabdomyosarcoma is one of the most common primary malignant orbital neoplasms of child hood. It usually produces a precipitously progressing unilateral proptosis of sudden onset. It is a highly malignant neoplasm of pleuripotential embryonic mesoderm, which commonly differentiates to form cells similar to rhabdomyoblasts of the foetus. Because of the presence of elongated cells that contain abundance of eosinophilic glycogen rich cytoplasm, it is generally referred to as embryonal form of rhabdomyosarcoma.
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PMID:Embryonal rhabdomyosarcoma of orbit--a case report. 208 80

Rhabdomyosarcoma commonly involves the orbit resulting in unilateral proptosis, swelling, and restricted ocular motility. We saw two unusual cases of children with nasopharyngeal rhabdomyosarcoma which caused rapid bilateral visual loss due to simultaneous spread of the tumor to the orbital apices. Initial CT scans revealed the tumor, but were misinterpreted as "normal," causing a significant delay in diagnosis. We recommend high resolution CT scans of the nasopharynx, orbits, and suprasellar region in any child with rapid bilateral visual loss to rule out this common childhood tumor.
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PMID:Rhabdomyosarcoma causing rapid bilateral visual loss in children. 623 56

The endodermal sinus tumor is a malignant germ cell neoplasm that usually arises in gonads, but on rare occasion occurs in extragonadal locations. Five tumors of the orbit, which by light microscopy displayed features characteristic of gonadal endodermal sinus tumor, were tested for the presence of alpha fetoprotein using paraffin-embedded tissue and an immunoperoxidase technique. Each tumor contained intracytoplasmic alpha fetoprotein. Review of the clinical histories revealed that orbital endodermal sinus tumors differ from other extragonadal endodermal sinus tumors in that they occur at a younger age and when treated aggressively can result in long-term survival. In children with rapidly progressive proptosis the distinction between endodermal sinus tumor and rhabdomyosarcoma poses diagnostic difficulties for both the clinician and the pathologist. When considering the diagnosis of endodermal sinus tumor, the use of immunohistochemical testing for alpha fetoprotein can be helpful.
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PMID:Endodermal sinus tumor (yolk sac tumor) of the orbit. 667 41

Although thyrotoxicosis and orbital complications of acute ethmoid or frontal sinusitis are among the most common causes of unilateral exophthalmos, inflammatory pseudotumor is frequently accompanied by progressive acute unilateral proptosis. Because the associated chemosis, scleral erythema, and ophthalmoplegia constitute a spectrum of clinical findings present in numerous inflammatory orbital disorders and systemic diseases, the diagnosis of inflammatory pseudotumor is one of exclusion, often requiring orbital biopsy. Four patients without evidence of sinusitis, endocrinopathy, collagen vascular disease, or Wegener's granulomatosis are described. The diagnosis of orbital pseudotumor was disclosed by computed axial tomography, thus avoiding orbitotomy. The finding of scleral and choroidal thickening with enhancement following intravenous contrast injection represents a select group of patients with orbital pseudotumor and differentiates them from patients with endocrine exophthalmopathy or neoplasms. This noninvasive technique is extremely valuable because early diagnosis is critical for successful treatment. All four patients responded dramatically to high-dose corticosteroid therapy. In the absence of significant clinical response, however, Wegener's granulomatosis, lymphoma, and rhabdomyosarcoma, especially in younger patients, must be carefully excluded. Orbital exploration or decompression or both are used when proptosis, headache, or orbital pain does not resolve promptly, visual acuity deteriorates, or the diagnosis remains unknown.
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PMID:Computerized axial tomography in inflammatory pseudotumor of the orbit. 682 19

Five patients had juvenile fibrosarcoma of the orbit and eyelid. At initial surgical intervention, the patients ranged in age from newborn to 8 years (mean, 3 years 9 months). Three patients were male and two were female. Proptosis and/or painless swelling of the eyelid were the most common signs and symptoms. Four tumors grew within a few weeks or months, and all five were poorly circumscribed. They were composed of immature, spindle-shaped fibroblastic cells in a classic herringbone pattern or in interlacing fascicles. Hypercellularity and mitotic activity were present in all lesions. The differential diagnosis included rhabdomyosarcoma, fibromatosis, and fibrous histiocytoma. Electron microscopy confirmed the fibroblastic nature of the tumor cells in three cases. Follow-up ranged from one to 32 years (median, seven years). Two of five tumors recurred locally but none metastasized. Apparently surgical excision is the treatment of choice and the tumor has a favorable prognosis.
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PMID:Juvenile fibrosarcoma of the orbit and eyelid. A study of five cases. 682 71

Unilateral proptosis, dislocation of the globe and impairment of motility in childhood are known to be typical presenting signs of rhabdomyosarcoma of the orbit. In a retrospective analysis of 17 patients with rhabdomyosarcoma (1974-1993) only 8 patients presented with typical signs. 9 patients did never show the typical signs which initially lead to a wrong diagnosis in many cases. A 5-year-old boy complained of a conjunctival cyst in the lower fornix of the left eye. Two weeks later the cyst was replaced by a solid tumor. The tumor was not completely resectable, histology revealed an embryonal rhabdomyosarcoma. Presenting signs and symptoms of orbital rhabdomyosarcoma show a sometimes misleading variety including conjunctival cyst formation. In any recent onset of unilateral rapidly progressing alterations of the lid, the conjunctiva or the caruncle in childhood the physician should consider a rhabsomyosarcoma of the orbit.
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PMID:[The initial clinical manifestations of rhabdomyosarcoma]. 765 15


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