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 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

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

A phase I study with recombinant human tumor necrosis factor alpha (rhuTNF-alpha; Knoll AG, Ludwigshafen, FRG) in patients with advanced malignant disease was undertaken to evaluate drug toxicity (organ specificity, time course, predictability, reversibility, maximal tolerated dose), effectiveness, antigenicity and pharmacokinetics. TNF was administered as a test dose followed by daily i.v. infusions for 5 days, every 3 weeks (single i.v. infusion lasting 10 min, TNF dissolved in 50 ml 5% human albumin). Dosage was increased in groups of 3 or 4 patients from 0.04 mg/m2 to 0.28 mg/m2. A total of 19 patients with different cancers, including seven large-bowel carcinomas, three chronic myelogenous leukemias, three hypernephromas, two small-cell lung cancers, one malignant melanoma, one malignant lymphoma, one rhabdomyosarcoma and one fibrosarcoma were treated. Major side-effects were chills and fever (maximum 40.4 degrees C, median 38.7 degrees C, 19/19), headache (12/19), nausea and vomiting (12/19) and pronounced (greater than 20%) hypotension (4/19). Acute side-effects could be diminished by paracetamol or indomethacin pretreatment, and with one possible exception no tachyphylaxis to TNF was noted. Mild renal toxicity was seen during TNF treatment. Pharmacokinetic studies showed a serum half-life (t1/2) ranging from 11 min to 17 min for doses from 0.04 mg/m2 to 0.16 mg/m2 and prolonged clearance with t1/2 ranging from 54 min to 70 min in the 0.20-0.28 mg/m2 dose range. No objective antitumor effects were observed in this phase I study.
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PMID:Phase I study of recombinant human tumor necrosis factor alpha in advanced malignant disease. 272 Jul 7

Intracranial extension of rhabdomyosarcoma from the face, nasopharynx or middle ear is rare. A 16-year-old boy presented with deterioration of vision and headache. CT scan revealed a soft tissue mass occupying the sphenoid and ethmoid sinuses, extending to the suprasellar fossa and impinging on the optic chiasm. The tumour, mimicking pituitary carcinoma, was removed by transsphenoidal craniotomy. Morphologic studies, including immunohistochemistry and electron microscopy, revealed that the tumour was a rhabdomyosarcoma. This case stresses the value of immunohistochemical and ultrastructural studies in the diagnosis of tumours occurring in the region of the sella turcica. The origin of this tumour was thought to be the sphenoid or ethmoid sinus. The pituitary gland appeared intact.
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PMID:Rhabdomyosarcoma in the region of the sella turcica. 368 1

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

A 56-year-old male suffered from primary rhabdomyosarcoma on the left internal thoracic wall, which was treated by chemotherapy, and local irradiation following biopsy. Four months after the diagnosis, he suddenly complained of headache and left paresthesia occurred followed by generalized convulsion and left hemiplegia. CT scan revealed a high density mass in the right parietal lobe. The patient was referred to our department and underwent emergency evacuation of the hematoma together with tumor removal. The pathological specimen showed spindle or oval-like cells with hypercellularity and some mitotic figures. Immunohistochemical study demonstrated that many cells were positive for desmin and myoglobin, which is specific to myogenic tumor. These characteristics were compatible with those of the primary thoracic lesion, and a diagnosis of metastatic rhabdomyosarcoma was made. Five weeks after the craniotomy, the metastatic brain tumor recurred in the same site and also in the bilateral occipital lobes. Although radiotherapy to the brain decreased the tumor size, the patient died of respiratory failure eleven and a half months after the initial diagnosis. Sarcomas metastasizing to the brain are rare and only 16 cases of rhabdomyosarcoma metastasizing to the brain have been reported so far. Recent advances in chemotherapy, however, have been able to show the increased incidence of sarcoma metastasis to the brain. Therefore, the necessity of follow-up CT scan for sarcoma patients of long survival is to be stressed, even if the patient shows no neurological symptoms.
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PMID:[Brain metastasis of rhabdomyosarcoma with intratumorous hemorrhage: a case report and literature review]. 825 24

The frequency and severity of neurologic symptoms in children with systemic cancer is unknown. The authors reviewed the records of children with systemic cancer for whom a neurologic consultation was requested between 1993 and 1996. The 157 patients had 161 malignancies and 205 consultations. Leukemia (59) and lymphoma (34) were the most common malignancies. The 68 solid tumors included neuroblastoma (13), Ewing's sarcoma, and rhabdomyosarcoma (10 each). In contrast to adults, in whom back pain and altered mental status are the most common reasons for neurologic consultation, headache (33) and seizures (29) were the most common symptoms in children. Structural lesions were present in 84% of patients with headache and focal deficit and in 14% of patients with isolated headache. Structural disease was identified in 37% of children with seizures. Neurologic signs were caused by complications of cancer therapy in 70 instances and to direct tumor invasion of the nervous system in 60. In 71 consultations, neurologic symptoms could not be attributed to cancer or its treatment. The spectrum of neurologic symptoms in children with cancer differs from adults and requires the consulting neurologist to have a thorough knowledge of childhood cancer and its effects on the nervous system.
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PMID:Neurologic consultations in children with systemic cancer. 1008 40

A 6-year-old male presented with headache, vomiting, visual obscuration, and papilledema. Clinical presentation, initial laboratory data, and radiologic evaluation suggested a diagnosis of pseudotumor cerebri. The development of lower cranial nerve palsies after transient resolution of symptoms prompted reassessment of the diagnosis, which revealed retropharyngeal rhabdomyosarcoma involving the right jugular vein. Cerebral angiography demonstrated the complete venous occlusion at the jugular foramen. Careful neurologic follow-up is essential in a patient with pseudotumor cerebri or idiopathic intracranial hypertension.
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PMID:Retropharyngeal rhabdomyosarcoma mimicking pseudotumor cerebri. 1042 38

A 44 year-old man presented with a three month history of increasing headache and evolving left sided hemiparesis that culminated in an haemorrhage into an intracerebral tumour which was partially resected. Histologic, immunohistochemical, electron microscopic and molecular studies are supportive of a diagnosis of primary embryonal rhabdomyosarcoma. While primary rhabdomyosarcoma of the central nervous system is rare, and 72% of previously reported cases are in the paediatric population, there appears to be subset of these tumours occurring supratentorially in the adult.
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PMID:April 1999--44 year old man with a bleeding intracerebral tumor. 1051 12

A 64 year-old patient, complained of headache and neurological disorders. CT scan found a voluminous solitary tumor of the posterior part of the left cavernous sinus. Removal of tumor was followed by a rapid recurrence and by the patient's death. Histologic study found a malignant undifferentiated tumoral proliferation, with strap-like cells. Immunohistochemical stains were positive for conjunctival and muscular differentiation. Ultrastructural study revealed intracytoplasmic filamentous striated structure. The primary meningeal rhabdomyosarcoma is an exceptional tumor, generally affecting young patients. Its prognosis is poor and its histogenesis remains unclear.
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PMID:[A case of primitive meningeal rhabdomyosarcoma. Histological, immunohistochemical and ultrastructural study]. 1101 54


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