Cases reported "Rhabdomyosarcoma"

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1/30. Small cell neuroendocrine carcinoma with skeletal muscle differentiation: report of three cases.

    Three cases of neuroendocrine carcinoma showing skeletal muscle differentiation are presented. The tumors were located in the skin and subcutaneous tissue, the urinary bladder, and the nasal cavity respectively, and were composed by two cell types admixed intimately with each other. One cell type had features identical to those seen in conventional small cell neuroendocrine carcinoma, including scanty cytoplasm, round nuclei with fine granular chromatin, immunohistochemical reactivity for neuron-specific enolase, chromogranin and cytokeratins, and electron-dense granules on ultrastructural examination. The second cell type was either plasmacytoid or elongated and straplike, with abundant eosinophilic cytoplasm and irregular nuclei with prominent nucleoli. These cells showed immunohistochemical positivity for desmin, sarcomeric actin, myoglobin, and myogenin. They also exhibited ultrastructural evidence of rhabdomyoblastic differentiation in the form of contractile filaments with abortive Z-band formation. An origin from a cell capable of dual differentiation toward neuroendocrine and rhabdomyoblastic elements is postulated for these tumors.
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keywords = cavity
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2/30. Congenital rhabdomyosarcoma--a case report.

    A term female newborn was noted to have a tumor mass in the oral cavity soon after birth. Oral computer tomography revealed a well-enhanced soft tissue mass about 4 x 4 x 3 cm in size in the left buccal area. Group III embryonal type congenital rhabdomyosarcoma was diagnosed after biopsy (gross removal was not feasible). Respiratory distress exacerbated due to rapid tumor growth compressing airway with the result that endotracheal tube had to be intubated. Chemotherapy was done and complicated by two episodes of neutropenic fever and sepsis. Radiotherapy was suggested but refused by the family. Tumor size was slightly reduced and endotracheal tube could be removed four months later. She was taken home under regular chemotherapy. Radiotherapy, was, however, clearly indicated.
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ranking = 4.1185271807907
keywords = oral cavity, cavity
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3/30. Rhabdomyosarcoma of the orbit in the newborn.

    A full-term black boy had a 2- to 3-cm, round, bluish mass on his right lower eye-lid at birth, later diagnosed as rhabdomyosarcoma. It was cystic in nature and extended into the nasal cavity. The tumor was initially classified as neuroblastoma. The child died eitht months later and necropsy report confirmed an original ophthalmologic pathology diagnosis of embryonal rhabdomyosarcoma.
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4/30. Pediatric sinonasal rhabdomyosarcoma: three cases and a review of the literature.

    OBJECTIVE: To understand the clinical presentation, management, and natural history of paranasal rhabdomyosarcoma. DESIGN: Retrospective case series review. methods: Retrospective medical record review of patients less than 20 years of age who presented to our facility with rhabdomyosarcoma of the nasal cavity or paranasal sinuses. RESULTS: medical records of all pediatric patients seen in our pediatric otolaryngology clinic were reviewed from January 1, 1995, through December 31, 2000. Three patients were identified with sinonasal rhabdomyosarcoma. Their presentation, evaluation, and treatment were evaluated. Relevant literature 1966 to the present was reviewed with the assistance of medline. CONCLUSIONS: Rhabdomyosarcoma is an aggressive pediatric malignancy, requiring a high index of suspicion to detect it in its earliest stages. patients with suggestive symptoms should undergo a full evaluation including nasal endoscopy and imaging. Because the current chemotherapy protocols are more effective on localized disease, early diagnosis is crucial to patient survival.
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5/30. Visual evoked potentials during the early phase of optic nerve compression in the orbital cavity.

    We obtained case histories and electrophysiologic recordings from four patients with transient vision impairment due to acute orbital compression. The visual evoked potentials (VEPs) displayed alterations that depended on the size and consistency of the compressing pathology and also on the duration of the compression. This study provides evidence of the utility of the VEP in the assessment of the severity and reversibility of optic nerve lesions. The case histories also emphasized the necessity to elucidate the pathologic process of compressive lesions of the optic nerve.
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6/30. adult rhabdomyosarcoma in the nasal and paranasal sinuses showing complete local response to a combination of chemotherapy and radiotherapy using 3D-CRT and IMRT.

    We report on a 69-year-old woman with rhabdomyosarcoma arising from the nasal and paranasal sinuses. She was referred to our hospital with a reduced ability to smell and impaired bilateral vision, narrowing of the visual field, and left facial pain. Computed tomography (CT) revealed a large tumor in the nasal and left paranasal sinuses invading the left orbital cavity and anterior skull base, and lymph node swellings in the submental and left accessory nerve areas. A biopsy specimen from the nasal tumor was diagnosed histologically as a rhabdomyosarcoma, alveolar type. Because the intracranial direct invasion and distant metastases to the thoracic spine were suspected by pretreatment examination, our case was determined to be inoperable by a head and neck surgeon. Radiotherapy with a total dose of 60 Gy was carried out to control the primary disease. The three-dimensional conformal radiotherapy (3D-CRT) and intensity-modulated radiotherapy (IMRT) techniques were used in order to reduce the doses to risk organs. Combined with radiotherapy, chemotherapy was also performed for the treatment of lymph-node metastases and distant diseases. After the treatment was completed, the primary tumor and lymph-node metastases disappeared completely; there was no sign of re-growth during the follow-up period. chemoradiotherapy may be an effective treatment also for inoperable adult rhabdomyosarcoma in the head and neck region. Furthermore, the 3D-CRT and IMRT techniques are both useful methods of radiotherapy for this disease.
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ranking = 1
keywords = cavity
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7/30. Primitive neuroectodermal tumour in the oral cavity. Case report.

    Primitive neuroectodermal tumour is regarded as a rarely seen lesion as it occurs mainly within the central nervous system. However, this neoplasm does occasionally occur elsewhere in the body. One such case which occurred in the posterior part of the palate in a child is reported. Following a combined therapeutic approach comprising surgery, radiotherapy and chemotherapy, the patient has been well for nine years since treatment.
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ranking = 16.474108723163
keywords = oral cavity, cavity
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8/30. Vascularized omentum graft for the reconstruction of the skull base after removal of a nasoethmoidal tumor with intracranial extension: case report.

    A 16-year-old boy with rhabdomyosarcoma occupying the nasal cavities and the ethmoid sinus with intracranial extension underwent transcranial surgery. The intradural tumor was resected first with the affected dura of the anterior skull base, and the dural defect was repaired with fascia harvested from the sheath of the rectus abdominis muscle. The remaining tumor contiguous to the nasal cavities was completely extirpated. The cranial cavity was then exposed to the opened nasal cavities, where a revascularized omental graft was used to separate these compartments. Lyophilized dura was placed beforehand beneath the omental graft, as a roof to the nasal cavity, and was removed 3 weeks later through the nostril. A bony skull base repair was performed over the omentum using the inner table of the bone flap. subcutaneous fat from the abdomen was placed on the bone graft for fixation and as an additional seal for the dural defect. Reconstruction of the anterior skull base with a vascularized omental transfer provides an efficient barrier to the nasal cavity. It also serves as an excellent supporting structure for regeneration of the mucosal epithelium of the nasal cavities.
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ranking = 3
keywords = cavity
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9/30. Rhabdomyosarcoma of the alveolar ridge in an adult.

    Rhabdomyosarcoma of the head and neck is rare in adults, and past reports indicate that it is a more aggressive disease than that which is found in children. We report the case of a 63-year-old woman with an alveolar type of rhabdomyosarcoma located on the mandibular alveolar ridge. Rhabdomyosarcoma of the oral cavity appears to carry a particularly poor prognosis, especially when bone is involved. Multimodal treatment with surgery, radiation therapy, and chemotherapy is indicated in all patients.
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ranking = 4.1185271807907
keywords = oral cavity, cavity
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10/30. Clear cell rhabdomyosarcoma of the nasal cavity and paranasal sinuses.

    This report describes two cases of alveolar rhabdomyosarcoma of the nasal cavity with unusual histological appearances mimicking clear cell carcinoma. The closely packed tumour cells were polygonal and arranged in sheets and packets. They had an appreciable amount of clear cytoplasm due to accumulation of glycogen. The diagnosis of rhabdomyosarcoma was confirmed by positive staining for desmin and myoglobin. Rhabdomyosarcoma should be included in the differential diagnosis of nasal clear cell tumours, particularly in young adults. A correct diagnosis is important, because chemotherapy is indicated even for apparently localized disease.
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