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

The consultants agreed that the differential diagnosis should focus on congenital masses, including an encephalocele, glioma, dermoid, hamartoma, hemangioma, rhabdomyosarcoma, neurofibroma, and nasolacrimal duct cyst. There was some disagreement as to which is the best way to evaluate the mass, ranging from an MRI (Dr. Reilly), to CT scan (Dr. Cotton), to both MRI and CT (Dr. Koopman). Blood tests to evaluate pituitary function could be indicated if there was a sphenoid defect (Dr. Reilly). None of the experts would biopsy this lesion. All would proceed with a definitive resection. One surgeon would defer surgery for several months and then perform the resection via a biocoronal craniotomy (Dr. Reilly). A combined anterior craniotomy and external ethmoidectomy would be planned by another (Dr. Koopman). The third consultant would combine an anterior craniotomy with a mid-face degloving, external rhinoplasty, or lateral rhinotomy approach (Dr. Cotton). Routine perioperative antibiotics would only be used by two of the surgeons (Drs. Reilly and Koopman). If a CSF leak were encountered there are several options. A small lesion could be allowed to close on its own (Dr. Reilly). If the leak occurred while the bicoronal incision was still open or if the leak were large, it could be repaired from above (Drs. Reilly and Koopman). One surgeon would proceed with a repair from above even if the leak were encountered during the intranasal approach (Dr. Cotton). Only one surgeon would restrict postoperative activity with intubation and sedation or paralysis (Dr. Koopman). Regarding follow-up, no one was concerned about the final pathology report.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Nasal mass in a pediatric patient. 139 78

A 56-year-old man was admitted to our hospital with right chest pain. Chest X-ray, CT scan and MRI revealed a chest wall tumor and enlarged mediastinal lymph nodes. Percutaneous lung biopsy was performed, and the pathological diagnosis of pleomorphic rhabdomyosarcoma was obtained. The only significant abnormal laboratory finding was elevation of serum NSE (24.5 ng/ml). Although chemotherapy (VAC-ADM) and radiation therapy were performed, the patient died about 7 months after admission. To our knowledge, only 17 cases of chest wall rhabdomyosarcoma have been reported in Japan.
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PMID:[A case of chest wall rhabdomyosarcoma]. 175 9

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

There has been a continuous acceleration of medical/scientific inquiry and of actual improvements in management of patients with neoplasms of the mesenchymal tissues over the last four decades. The number of publications in this field has increased from 1140 in 1970 and then to 1700 in 1990. Important advances discussed over this period include: establishment of sarcoma teams in major oncology centers; staging systems for both soft tissue and osseous sarcomas; demonstration of genetic determinants in the development of, at least, some of the sarcomas; the revolutionary change in quality of diagnostic imaging by the introduction of CT and MRI; use of immunohistochemistry in diagnostic pathology; the drastic gains in survival of patients with osteogenic sarcoma, Ewing's sarcoma and rhabdomyosarcoma due to the efficacy of multi-drug and multi-cycle chemotherapy protocols; major advances in surgical techniques which have made limb salvage practical; cell lines derived from human sarcomas have been shown to have in vitro radiation sensitivity comparable to that of cell lines from epithelial tumors; the combination of conservative surgery and moderate doses of radiation yields local control and survival results equivalent to that of radical surgery with a much improved functional and cosmetic outcome; intra-operative electron beam radiation therapy improves the outcome of patients with retroperitoneal sarcomas when given after grossly complete resection combined with external beam radiation therapy (pre- or postoperatively); radiation is a highly effective alternative to extensive surgery for desmoid tumors; local control of giant cell tumors by modern radiation techniques is approximately 80% and the incidence of radiation induced tumors at 10 years is approximately 3%; to decrease the incidence of radiation induced sarcoma, resection has replaced radiation in the management of selected patients with primary Ewing's sarcoma when the response to chemotherapy has been excellent and the morbidity/functional decrement consequent upon the surgery judged reasonable; proton beam radiation therapy has been accepted as being superior to conventional external beam radiation therapy for chondrosarcoma and chordoma of the skull base; and attempts to utilize brachytherapy for sarcomas of the spine/sacrum appear to offer promise. Projected advances in the coming two decades includes:Designation of sarcoma type on genetic characterization; molecular genetics will provide prognostic information as to probability of distant metastasis, response to chemotherapeutic agents and radiation; important further reductions in the radiation treatment volume due to the many technical developments entering, or soon to enter the clinic; non-invasive assessment of the response to chemotherapy; much increased appreciation of the late sequella of treatment, both radiation and chemotherapy.
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PMID:Regaud Lecture, Granada 1994. Tumors of the connective and supporting tissues. 759 17

From 1975 to 1993, 11 of 375 patients treated for soft tissue sarcoma presented with post-irradiation sarcoma. The mean time interval between irradiation therapy and onset of the second neoplasm was 15.8 years (4-31 years). The total radiation dosage ranged from 12 to 60 Gy with a mean of 40 Gy. All patients had complete staging including CT or MRI of the tumour site, and CT of the lung. Surgical resection was the treatment of choice. Wide margins could be achieved in 10 patients. One had a marginal resection. Tumours included malignant fibrous histiocytoma, haemangiosarcoma, rhabdomyosarcoma, malignant schwannoma, fibrosarcoma and undifferentiated sarcoma. All patients were reassessed in our outpatient clinic. After a mean follow-up of 4.7 years (1.0-11.5 years), only 1 patient had died because of the tumour. Although post-irradiation sarcomas are rather infrequently observed, these tumours must be suspected when alterations or symptoms occur in a previously irradiated region. Early detection provides the chance of curative, wide margin resection.
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PMID:Post-irradiation soft tissue sarcoma. 769 75

Thallium-201 scintigraphy was used to evaluate chemotherapeutic response in a case of rhabdomyosarcoma treated with epirubicin. A scintigraphic semiquantitative index of tumor viability was correlated with MRI and clinical assessment. Both imaging studies were performed before therapy and after treatment involving a cumulative dose of 640 mg/m2 of epirubicin. A very good inverse correlation was observed between Tl-201 uptake and tumor necrosis evaluated by MRI, leading to the conclusion that sequential Tl-201 scintigraphy may have a role not only in delineating the extent of the tumor, but in objectively assessing tumor response to therapeutic interventions. The proposed scintigraphic method is much simpler to perform and less expensive than MRI-derived semiquantitative measurement of tumor necrosis.
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PMID:Thallium-201 scintigraphy and chemotherapeutic response in rhabdomyosarcoma. 792 2

Non-squamous malignancies of the larynx are extremely rare, usually subsurface in origin and mostly diagnosed only in an advanced stage. Two cases representative of this group are described, namely, an adenoid cystic carcinoma and a pleomorphic rhabdomyosarcoma. The clinicopathological data of these unusual tumours are presented and the current therapeutic modalities discussed. The diagnostic value of MRI with contrast medium is emphasised by demonstrating an otherwise not recognised far advanced tumour stage. The pleomorphic rhabdomyosarcoma was also examined via electron microscopy.
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PMID:[Non-squamous epithelial malignancies of the larynx with special reference to MRT diagnosis]. 814 51

Twenty-one children with predominantly solid tumours detected by US were also examined by MRI (7 neuroblastomas, 2 Wilms tumours, 3 hepatoblastomas, 2 germinal cell tumours, 1 ganglioneuroblastoma, 1 gangliocytoma, 1 Cushing's adenoma, 1 phaeochromocytoma, 1 retroperitoneal rhabdomyosarcoma, 1 diffuse lymphangiectasia of a kidney, 1 splenunculus). The findings from both methods were compared with respect to the identification of the organ involved, extent of the tumour, effect on neighbouring structures and tissue characteristics. US and MRI were of equal value in defining the origin of the lesion and in demonstrating pathological enlargement of lymph nodes. Exact tumour extent could be better demonstrated with MRI because of the ability to perform multiplanar sections and to demonstrate intrathoracic and intraspinal spread. MRI was superior in 9 cases in demonstrating tumour structure and in 6 cases in the evaluation of vascular involvement and vascular anatomy. MRI is therefore recommended as an additional method to US for diagnosis and for treatment planning.
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PMID:[Abdominal tumors in children. A comparison between magnetic resonance tomography (MRT) and ultrasonography (US)]. 842 85

Today, patients with alveolar rhabdomyosarcoma have a favorable prognosis with a survival rate of more than 80%, due to modern radiotherapy. However, those patients with secondary meningeal involvement are still afflicted with a mortality rate of 90% unless a different therapeutic approach is taken. We report a case of a young adult with invasion of the tumor into the anterior cranial fossa. MRI aided in the assessment of the intracranial tumor spread and allowed for a direct visualization of the meningeal membranes. The signal characteristics of the tumor and the adjacent tissue in unenhanced and postcontrast MRI is discussed. MRI was clearly superior to CT and indicative for the choice of treatment.
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PMID:MR imaging of late onset orbital rhabdomyosarcoma with intracranial extension. 845 40

A case of rhabdomyosarcoma in a 14-year-female is reported herein. The patient developed left inguinal pain and tumor. She was initially seen by her local medical doctor, who biopsied her left inguinal tumor with a pathological report of malignancy. She was referred to the Ryukyu University Hospital in July, 1990. CT and MRI demonstrated a large tumor 10 cm in diameter arising from the left iliac region. The tumor was extirpated and the pathological report indicated rhabdomyosarcoma (alveolar type). The patient was treated with VAC chemotherapy and local irradiation. Her course has been uneventful for the past 2 years without any clinical manifestation of recurrence since surgery.
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PMID:[A infant case of rhabdomyosarcoma arising from the left iliac region with complete remission for 2 years after combination therapy]. 847 Sep 26


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