Cases reported "Rhabdomyosarcoma"

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1/65. adult rhabdomyoma of the extremity: a case report and review of the literature.

    The adult rhabdomyoma is a rare, benign skeletal muscle neoplasm that usually occurs in the head and neck. A case report of an adult rhabdomyoma arising in the thigh is presented with a review of the literature. This is the first case of an extremity adult rhabdomyoma to be reported. It is also the largest at 13 centimeters. Distinction from a highly differentiated rhabdomyosarcoma is important. Recent chromosomal studies suggest that the adult rhabdomyoma is a true neoplasm. Total resection is curative but the lesion may recur if incompletely excised.
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2/65. A case of head and neck kaposiform hemangioendothelioma simulating a malignancy on imaging.

    Kaposiform hemangioendothelioma (KH) is an endothelial-derived spindle cell neoplasm often associated with kasabach-merritt syndrome. Most cases arise in infancy and childhood and are soft-tissue tumors. The tumor displays an appearance between capillary hemangioma and Kaposi's sarcoma. We report a case of KH in a 1-year-old girl involving a mass that showed abnormal enhancement of soft tissue superficial to the right temporal bone with partial destruction of the temporal bone, the temporomandibular joint, mandibular condyle, and occipital bone. The physical finding of a discolored mass led clinicians to consider a hemangiomatous lesion, whereas the radiological picture suggested a more aggressive diagnosis of rhabdomyosarcoma and aggressive fibromatosis.
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keywords = neck
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3/65. Anticipation of dental anomalies induced by radiation therapy.

    Therapeutic irradiation of the head and neck of children up to the age of 16 years can cause malformation and developmental arrest of the permanent teeth. Dental anomalies occurring after irradiation of the palatal tumor in an 8-year-old are described and compared with a normal 8-year-old's developmental sequence to demonstrate that the immature, noncalcified dental structures are most susceptible to radiation injury. The stage of dental development at the time of therapy may be precisely determined by a routine oral radiographic survey, which provides a reliable estimate of the deformation that may be anticipated following radiotherapy.
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4/65. Spindle cell lesions of the head and neck mimicking rhabdomyosarcoma in children.

    Malignancies of the head and neck are uncommon among children. The most common solid tumors to occur in this region are rhabdomyosarcoma, hodgkin disease, and non-Hodgkin lymphoma. Two children are described who presented with signs and symptoms consistent with rhabdomyosarcoma, but who were found to have benign spindle cell lesions. These rare lesions should be part of the differential of children with a head and neck mass.
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keywords = neck
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5/65. rhabdomyosarcoma of the adult nasopharynx.

    rhabdomyosarcoma of the adult head and neck is rare, particularly beyond 40 years of age. In this region, the nasopharynx is an unusual site. Most nasopharyngeal rhabdomyosarcomas are of an alveolar variety. We report a case of embryonal or spindle cell rhabdomyosarcoma in the nasopharynx of a 47-year-old man. The histology of this tumour revealed clear cells that have not been described in embryonal rhabdomyosarcomas occurring in the head and neck. The pathology is discussed and current literature reviewed.
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ranking = 2
keywords = neck
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6/65. Orofacial rhabdomyosarcoma in neonates and young children: a review of literature and management of four cases.

    rhabdomyosarcoma (RMS) is an aggressive malignant skeletal muscle neoplasm arising from embryonal mesenchyme. It accounts for over 50% of all pediatric soft tissue sarcomas. The head and neck region is the most common site for this tumor in children. Neonatal presentation of this tumor is rare. We present the management of one neonatal case and three additional cases of orofacial RMS in children under the age of 7 years. All four patients were seen in the department of oral and maxillofacial surgery at Children's Hospital and Regional Medical Center (CHRMC) in Seattle between 1992-2000. Three of the four cases were alveolar RMS and one was botryoid sub-type of embryonal RMS. Three patients were treated with a combination of surgery, chemotherapy and radiation, while the patient with botryoid RMS was treated with surgery and chemotherapy only. The patient with congenital RMS died at 2.5 years of age due to recurrent metastatic disease. The other three patients are alive without evidence of recurrent with a mean follow up was 5.5 years (range 2.5-8.5 years). We discuss the current management, diagnosis, biological behavior, histopathology, prognosis and survival of head and neck RMS in neonates and young children.
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7/65. Successful cochlear implantation in a child after recovery from a head and neck malignancy: a case report.

    We present the case of a successful pediatric cochlear implantation that was carried out following bilateral perilingual deafness caused by meningitis during the treatment of a childhood malignant tumor. A rhabdomyosarcoma localized in the frontobasal area was removed from the child at the age of 2 years. He then received 11 months of postoperative cytostatic treatment. A purulent meningitis developed at the end of the chemotherapy, resulting in a major-grade, bilateral sensorineural hearing loss (practically a perilingual deafness). After 6 tumor-free years and a meticulous preoperative assessment, a Nucleus 24 M cochlear implant was successfully implanted in the child's left ear. Two years after the operation, the child shows excellent hearing results and moderate speech development.
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ranking = 4
keywords = neck
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8/65. A rapidly progressing periorbital mass in an infant: fasciitis nodularis.

    Nodular fasciitis is a benign, rapidly growing proliferation of fibroblasts, which is histologically difficult to distinguish from neoplasms. In several reports, as many as half of all cases have been initially misdiagnosed as a fibrosarcoma or some other malignancy. Although the head and neck is a region of predilection in infants and children, only eight periocular lesions have previously been reported in paediatric patients. We present a case of nodular fasciitis which occurred in the periorbital region in a 1 1/2-year-old girl. The process was excised locally in order to perform a biopsy. The mass was initially classified as a sarcoma but subsequently as infantile fibromatosis. Only after a thorough review of the case and four independent pathological consultations was the final diagnosis of nodular fasciitis confirmed. No further treatment was scheduled. Although the primary surgical removal of the tumour was not radical, no recurrence was observed during a 3-year follow-up period.
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ranking = 1
keywords = neck
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9/65. Parameningeal rhabdomyosarcoma in a patient with costello syndrome.

    costello syndrome, a rare entity with multiple congenital anomalies, has been associated with a high incidence of benign and malignant tumors, particularly rhabdomyosarcoma. Although the head and neck is the most common site for rhabdomyosarcomas in children, only one other case in the literature has been reported of a head and neck rhabdomyosarcoma in a Costello patient. The authors describe a 3-year-old with a parameningeal infratemporal fossa rhabdomyosarcoma. Management is discussed with particular attention to the difficulties encountered due to the congenital heart abnormalities associated with the syndrome.
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keywords = neck
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10/65. Sclerosing rhabdomyosarcoma: a rare variant with predilection for the head and neck.

    OBJECTIVES/HYPOTHESIS: Sclerosing rhabdomyosarcoma is a newly described variant of rhabdomyosarcoma with a predilection for the head and neck. Little has been written on the topic, because of the scarcity of the disease and its recent recognition as a distinct entity. The present report describes the fifth confirmed case of sclerosing rhabdomyosarcoma and is the first report in the otolaryngology literature. STUDY DESIGN: Case report. methods: The authors have reported the case of a 66-year-old woman with a 35-year history of heavy cigarette smoking and daily alcohol consumption and a 2-month history of progressive dysphagia and dysarthria secondary to an enlarging tongue mass. Urgent tracheotomy was performed for impending respiratory embarrassment. Direct laryngoscopy revealed a bulky, exophytic mass involving the base of tongue. Specimens were obtained and submitted for analysis. RESULTS: Initial frozen-section analysis of the specimens favored carcinoma, although subsequent immunohistochemical analysis disproved this. The diagnosis of sclerosing rhabdomyosarcoma was based on microscopic appearance and patterns of gene expression, including the expression of desmin and myogenin. A search of the literature revealed only four confirmed cases of sclerosing rhabdomyosarcoma. With the inclusion of the oropharyngeal tumor in the present report, three of the five confirmed cases have occurred in the head and neck. CONCLUSION: Sclerosing rhabdomyosarcoma is a rare variant of rhabdomyosarcoma that has a predilection for the head and neck. The clinical presentation may mimic carcinoma. The otolaryngologist-head and neck surgeon must be familiar with this disease entity.
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ranking = 8
keywords = neck
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