Cases reported "Ear Neoplasms"

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1/17. facial paralysis: a presenting feature of rhabdomyosarcoma.

    The purpose of this paper is to present a child with embryonal rhabdomyosarcoma involving the left middle ear, who initially presented with unilateral facial paralysis. A 5-year-old boy presented with a 4-week history of left-sided facial weakness, associated with persistent otitis media on that side. Examination revealed complete left lower motor neuron facial weakness and hearing loss. A myringotomy revealed a soft tissue mass behind the tympanic membrane. biopsy and oncologic assessment confirmed a stage II, group III left middle ear embryonal rhabdomyosarcoma. Despite debulking surgery, local irradiation and multiple chemotherapeutic courses the child deteriorated quickly. He developed carcinomatous meningitis and died 9 months after his initial presentation. In conclusion, middle ear tumors should be considered in the differential diagnosis of unresolving otitis media, particularly when associated with persistent ipsilateral facial paralysis. An ear mass, discharge, facial swelling, or systemic symptoms may be initially absent despite the presence of this aggressive malignancy. Careful examination of the middle ear is recommended in children with facial weakness. A myringotomy incision may be necessary including a complete assessment of the middle ear cavity, particularly when there is no fluid return.
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keywords = rhabdomyosarcoma
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2/17. Tumors presenting as aural polyps: a report of four cases.

    All biopsy material from the ear, received over an 8 yr period by a private practice, was reviewed. Four unusual neoplasms presenting as aural polyps are described (meningioma, embryonal rhabdomyosarcoma, eosinophilic granuloma and adenoid cystic carcinoma). The relevant literature is briefly reviewed and the importance of histopathological examination of aural polyps is discussed.
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keywords = rhabdomyosarcoma
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3/17. rhabdomyosarcoma of the middle ear and mastoid: a case report and review of the literature.

    We report a case of rhabdomyosarcoma of the middle ear and mastoid in a 3-year-old boy. The patient was treated according to Intergroup rhabdomyosarcoma Study IV protocol (chemo- and radiotherapy), and he experienced a complete remission. However 7 months after the completion of treatment, he experienced a recurrence at the primary site that spread to the brain. Despite treatment, the patient died of progressive metastasis to the lung 4 months later.
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keywords = rhabdomyosarcoma
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4/17. rhabdomyosarcoma of the middle ear and mastoid in children.

    Three cases of embryonal rhabdomyosarcoma in the middle ear and mastoid in children are presented. Diagnosis was confirmed by histopathology. A multidisciplinary approach employing surgery, chemotherapy and radiation therapy is the method of choice in the management of this rare and highly lethal condition.
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keywords = rhabdomyosarcoma
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5/17. Embryonal rhabdomyosarcoma of the middle ear.

    An embryonic sarcoma of the middle ear occurring in a child has been described. The mainstay of treatment has been triple therapy. The child is well 4 years and 3 months after diagnosis.
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keywords = rhabdomyosarcoma
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6/17. Embryonal rhabdomyosarcoma of the ear: a review of the literature and case history.

    Approximately 50 per cent of all rhabdomyosarcomas in children occur in the head and neck region with the orbit, nasopharynx and ear in order of descending frequency. Embryonal rhabdomyosarcoma is the commonest malignant tumour of the aural region in childhood and its clinical course is usually rapidly fatal, with extensive local disease and or distant metastases (Dehner and Chen, 1978). Other malignant tumours that can occur in children include melanoma and other mesenchymal tumours, including undifferentiated sarcoma, fibrosarcoma, osteogenic sarcomas and Ewing sarcoma. Secondary extension may occur from a meningioma. Osseous disorders of the temporal bone, such as eosinophilic granuloma and hand-Schuller-Christian disease, should be included as a differential diagnosis (Lewis, 1979).
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keywords = rhabdomyosarcoma
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7/17. Dental root agenesis secondary to irradiation therapy in a case of rhabdomyosarcoma of the middle ear.

    There have been only a few published reports on the dental-facial effects of radiation therapy and the subsequent oral management of these patients. A case involving a 13-year-old black male patient with a history of rhabdomyosarcoma is presented. The patient received 4,050 rads of radiation to the right middle ear when he was 2 years of age. His residual medical and dental difficulties are apparently complications from the initial therapy. Examination of the oral cavity revealed bimaxillary micrognathia and marked loss of vertical dimension. A Class II facial profile with Class I molar relationship was observed. The mandible was thin and hypoplastic, with a small knife-edge alveolar ridge. The remaining eighteen erupted permanent teeth were very mobile, and root development had ceased after only initial formation. All of the teeth except the first permanent molars were extracted, and immediate partial dentures were inserted at the time of surgery. A discussion of therapeutic considerations follows the case report.
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keywords = rhabdomyosarcoma
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8/17. rhabdomyosarcoma of the head and neck.

    rhabdomyosarcoma is an uncommon neoplasm that is seen in the head and neck area. Since the symptoms of rhabdomyosarcoma, such as aural discharge and nasal obstruction, mimic common disease states, the clinical diagnosis is often delayed. case reports and a literature review are presented to emphasize the clinical and roentgenological findings in rhabdomyosarcoma. Misinterpretation of microscopic findings can delay the histological diagnosis. Cross-striations, the "hallmark" of rhabdomyosarcoma, are not always found, while "racquet" and "spindle" cells may be seen. Special stains and electron microscopy can assist the pathologist. The pathological findings of the different variants of rhabdomyosarcoma and a review of cases accessioned by the A.F.I.P. Otolaryngic Registry are presented. early diagnosis of rhabdomyosarcoma is important because the disease, once inevitably fatal, can now be controlled and apparently cured in most cases by a combination of surgery, radiation, and multidrug chemotherapy. This therapeutic approach, its results, complications, and support measures are considered.
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ranking = 0.83333333333333
keywords = rhabdomyosarcoma
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9/17. Embryonal rhabdomyosarcoma of the middle ear presenting as sarcoma botryoides. Favorable outcome in a 12-year-old boy.

    A 12-year-old boy presented with a history of earache, fever and granuloma of the auditory canal. biopsy revealed embryonal rhabdomyosarcoma. The patient was treated with chemotherapy and radiation therapy, which led to complete disappearance of the tumor without recurrence after three years. Diagnosis, staging and timing of the treatment plan is discussed for this rare but highly malignant tumor.
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ranking = 0.83333333333333
keywords = rhabdomyosarcoma
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10/17. temporal bone findings in rhabdomyosarcoma with predominantly petrous involvement.

    temporal bone rhabdomyosarcomas are rare tumors that occur almost exclusively in children. They arise most commonly in the middle ear, from which they extend through the mastoid and petrous portions of the temporal bone, eventually invading the cranium. We report the histopathologic findings of a rhabdomyosarcoma with anterior and medial displacement, predominantly involving the petrous bone. Recognition and separate study of this type of tumor is clinically important. Predominantly petrosal rhabdomyosarcomas are less prevalent than similar lesions with lateral expansion and tend to develop significant ear symptoms late in the course of the disease. When first diagnosed, these lesions usually exhibit signs of intracranial extension, and consequently are less amenable to treatment and are more rapidly fatal.
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ranking = 1.1666666666667
keywords = rhabdomyosarcoma
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