Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 20-month-old child with systemic features of neurofibromatosis type 1 presented with sudden-onset proptosis. Biopsy and histopathology confirmed the diagnosis of an embryonal rhabdomyosarcoma. The tumor regressed completely with chemotherapy and external beam radiotherapy. This case highlights the association of rhabdomyosarcoma with neurofibromatosis type 1.
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PMID:Orbital embryonal rhabdomyosarcoma in association with neurofibromatosis type 1. 1741 33

Rhabdomyosarcoma is the most common pediatric primary neoplasm in the orbit, often presenting with rapid proptosis and orbital symptoms. We describe a 15-year-old girl who presented with an acute mass in her medial rectus muscle that was subsequently diagnosed as widely disseminated alveolar rhabdomyosarcoma. To our knowledge, this represents the first reported case in which an enlarged extraocular muscle was the initial manifestation of disseminated alveolar rhabdomyosarcoma.
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PMID:Alveolar rhabdomyosarcoma presenting as an acute orbital mass in the medial rectus muscle. 1741 34

The histologic spectrum of nonosseous tumors and tumorlike lesions of the extraocular orbit in children differs from that in adults, and the appearance of these lesions at imaging reflects their pathologic features. Rhabdomyosarcoma is the most common extraocular orbital tumor in children. This neoplasm usually manifests in young children, grows quite rapidly, and is fairly vascular. Vasculogenic lesions are common orbital lesions in newborns and young infants. The most prevalent of these are infantile hemangioma, a true neoplasm, and venous-lymphatic malformation, a developmental anomaly. Hemangioma is quite vascular, has a predictable course of proliferation followed by slow involution, and is distinguished on magnetic resonance images by the finding of flow voids within the mass and at its periphery. Venous-lymphatic malformation in the orbit is an anomaly of venous and lymphatic development that is characterized by unenhancing, cystic lymphatic and enhancing, solid venous components. Intralesional hemorrhage is common and frequently produces distinctive fluid-fluid levels within the cystic portions. Unlike hemangiomas, venous-lymphatic malformations grow with the patient and never involute spontaneously. Infantile fibromatosis is one of the fibromatoses and affects newborns and young infants. The tumor is nodular and composed of a zonal architecture, with frequent hemorrhage or necrosis in the central portion, characteristics that confer a target appearance at imaging. These lesions usually stop growing or spontaneously regress. All of these extraocular masses typically manifest with proptosis, and imaging differentiation is desirable because the treatments and prognoses vary greatly.
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PMID:From the archives of the AFIP: Pediatric orbit tumors and tumorlike lesions: nonosseous lesions of the extraocular orbit. 1802 17

Orbital rhabdomyosarcoma is the most common orbital malignancy of childhood with the common presentation of rapidly evolving unilateral proptosis. We studied six patients who were diagnosed and treated for rhabdomyosarcoma between January 1999 and June 2004. The age of the patients ranged from 4 to 29 years. Four patients presented with acute onset proptosis associated with signs of inflammation, mimicking orbital cellulitis. One patient presented with lid mass. Another patient presented with a soft, blind eye that was pushed superotemporally by a large inflammed, vascularised mass. Embryonal rhabdomyosarcoma was the commonest histopathological type in our series found in five patients. One patient was completely cured with chemotherapy alone whereas two patients were treated with a combination of chemotherapy and radiotherapy. Three patients in our series required exenteration.
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PMID:Orbital rhabdomyosarcoma: a case series. 1825 May 31

A 68-year-old female who had undergone treatment several years previously for breast cancer presented with diplopia and unilateral proptosis and exposure keratopathy related to biopsy-proven rhabdomyosarcoma of the sinus and orbit. Further evaluation revealed multiple metastatic lesions felt to have originated from the primary sinus and orbital tumor. Histopathologic examination showed primitive-appearing rhabdomyosarcoma with some features suggestive of the alveolar subtype. Orbital or sinus rhabdomyosarcoma is seen almost exclusively in the pediatric population, but may very rarely occur in adults. There are several genetic mutations that appear to play a role in both rhabdomyosarcoma and certain breast tumors. There is also increasing evidence that even low doses of radiation may contribute to the future development of cancer, particularly in susceptible individuals. In our patient with atypical demographics for rhabdomyosarcoma, the previous neoplasm and treatment thereof may have predisposed to the development of this rare tumor.
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PMID:Stage IV primitive-appearing sinus and orbital rhabdomyosarcoma presenting in a 68-year-old female previously treated for breast cancer. 1830 53

A 24-year-old woman developed acute bilateral proptosis. She had a history of rhabdomyosarcoma of the left orbit treated 2 years previously with chemotherapy and radiation. Computed tomography demonstrated enlargement of each of the extraocular muscles in both orbits. Extraocular muscle biopsy confirmed rhabdomyosarcoma. She was treated with radiation but died 2 months after presentation.
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PMID:Rhabdomyosarcoma metastases to all extraocular muscles. 1864 54

Idiopathic inflammatory orbital myosites represent 10% of the orbital pseudotumors and 8% of the orbital pathologic processes in children. They are rarely described although they raise real diagnostic problems. The authors report the case of a 10-year old little girl who presented sudden proptosis. The clinical and radiological work up and the therapeutic test with corticosteroids were in favor of an inflammatory orbital myositis. The authors remind the clinical, radiological, and therapeutical aspects of this entity that remains very unfrequent in children and needs to be differenciated from rhabdomyosarcoma.
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PMID:[Orbital idiopathic inflammatory orbital myositis in a child: case report ]. 1870 Apr 52

Emergencies in childhood orbital tumorals are rare. The absolute emergency involves malignant primary orbital tumors, such as rhabdomyosarcoma or secondary malignant tumors (metastatic neuroblastoma, leukemia), involving a vital prognosis requiring prompt diagnosis. Delayed emergencies are usually vascular lesions. Among these lesions, immature orbital hemangioma, with a good prognosis, must be distinguished from orbital adnexal lymphangiomas, which are less frequent but can lead to dramatic cosmetic and functional disorders. In rare cases, they can be responsible for sudden, painful proptosis, due to orbital hemorrhage, with a risk of optic nerve compression, requiring emergency surgical treatment. Neurogenous lesions, either isolated, such as in gliomas, or associated with a systemic disease, such as Recklinghausen neurofibromatosis, threaten the functional prognosis. Diagnosis of pediatric orbital tumors is based on a good clinical examination, precise imaging investigations, and evaluation of the locoregional extension of the tumor. Biopsy is required in emergency situations, when rhabdomyosarcoma is suspected, in order to start the chemotherapy. However, the biopsy can be superfluous, and even useless or dangerous, when clinical and imaging investigations are sufficient to provide a diagnosis of capillary hemangioma, lymphangioma, or metastatic tumor from an abdominal malignancy. Treatment is closely related to the etiopathogenesis of the tumor. The outcomes are vital, functional and cosmetic. They may require orbital surgery (biopsy, tumoral resection, orbital decompression in case of a compressive hemorrhage), systemic corticotherapy (as in immature adnexal hemangioma), radiation, and chemotherapy (rhabdomyosarcoma, secondary malignant tumor). These diseases require a pediatric ophthalmological medical center specializing in orbital surgery, with close collaboration of multiple specialists such as onco-pediatricians and neurosurgeons.
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PMID:[Orbital tumor emergencies in childhood]. 1976 75

Proptosis due to intraorbital tumors is frequent and secondary to several etiologies. Generally, young people (0-20 years of age) will be affected mainly by benign diseases, such as angiomas or cysts (often dermoid cysts). Yet the ophthalmologist must never forget the possibility of rhabdomyosarcoma occurrence in a child. Adults (20-60 years of age) will for the most part develop benign tumors (cavernous hemangiomas, meningiomas) and inflammatory diseases. Last, malignant tumors, such as lymphomas and metastasized tumors, will mainly be observed in elderly people over 60 years of age.
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PMID:[Tumoral exophthalmitis]. 1985 66

The authors present a rare form of orbital cavitary rhabdomyosarcoma in which lymphangioma was mistakenly diagnosed on the basis of echography and MRI. Rhabdomyosarcoma can usually be differentiated from lymphangioma by echographic and MR imaging, because cavitation is very rare in orbital rhabdomyosarcoma, but rhabdomyosarcoma should be suspected whenever the clinical presentation of a rapidly progressive unilateral exophthalmos is observed in a child.
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PMID:Orbital cavitary rhabdomyosarcoma: a diagnostic dilemma. 2030 11


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