Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Rhabdomyosarcoma of the larynx is a malignant disease rarely seen in children. We present five cases (3 males, 2 females) observed among 126 patients with rhabdomyosarcoma of the head and neck (excluding the orbit) treated in the Institut Gustave-Roussy between 1955 and 1981. The patients were aged between 5 1/2 years and 13 1/2 years at the time of diagnosis. The presenting symptoms were dysphonia and/or dyspnea; tracheotomy was required in two cases. All five patients received chemotherapy consisting of vincristine, cyclophosphamide, actinomycin D + procarbazine or doxorubicin. Four patients also received radiotherapy (45 Gy during 5 weeks). Laryngectomy was not performed. Treatment sequelae included regional hypoplasia, huskiness, and subclinical thyroid insufficiency; two patients developed a benign thyroid adenoma. One patient presented a bilateral carotid stenosis subsequent to radiotherapy performed on "wide fields" portal designed to provided prophylactic treatment on uninvolved lymph nodes. All the patients are alive and relapse-free with a follow-up of between 13 and 17 years. This small series of RMS of the larynx shows that patients can be cured without laryngectomy, although long-term sequelae associated with radiotherapy are observed.
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PMID:Rhabdomyosarcoma of the larynx in children: a series of five patients treated in the Institut Gustave Roussy (Villejuif, France). 201 Oct 95

An exhaustive literature review of the last two decades discloses 47 laryngeal malignant neoplasms in children and adolescents. The most frequent malignant neoplasm is the embryonal variant of rhabdomyosarcoma. The timely diagnosis of a laryngeal neoplasm depends on maintaining a high index of suspicion in a patient with progressive airway obstruction, dysphagia or dysphonia, and conducting an efficient work-up-including magnetic resonance imaging and direct laryngoscopy under general anesthesia in association with bronchoscopy-in order to define the extent of the lesion, rule out multiple lesions, establish and maintain an airway, and perform a biopsy of the tumor. The authors observed that several risk factors, such as previous radiation therapy for juvenile laryngeal papillomatosis, intrauterine exposure to ionizing radiation, chemical carcinogens, smoking or tobacco exposure were lacking in those patients with a detailed clinical history. Probably, cancer of the larynx in these unusual patients is the final result of an interaction of immunological and genetic factors. The choice of therapy depends on several factors, including the clinical stage at presentation, histological type and potential radio-chemosensitivity of the tumor.
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PMID:Laryngeal malignant neoplasms in children and adolescents. 1042

A 29-year-old patient presented with dysphonia, dysphagia and a progressive history of stridor over 6 weeks. His past medical history included childhood nasolabial rhabdomyosarcoma treated by surgery, chemotherapy and radiotherapy. This had resulted in marked abnormalities of the facial skeleton, limited neck extension and restricted mouth opening of 1 cm, in part due to dental implants. After careful discussion and planning within a multidisciplinary team, the airway was optimised by temporary removal of the dental implants. This enabled a suspension laryngoscope to be passed, permitting carbon dioxide laser treatment to an obstruction at the laryngeal inlet and eliminating the need for a tracheostomy.
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PMID:Dental implant removal to facilitate suspension laryngoscopy and laser treatment of an obstructed airway. 1984 79

A 40-year-old male presented with 2 weeks of left facial pain, nasal congestion, dysphonia, and epistaxis along with left-sided epiphora. CT showed a large infiltrative mass centered in the left maxillary sinus with extension into the left orbit, bilateral paranasal sinuses, nasal cavity, and bilateral enlarged cervical lymph nodes. Biopsy results confirmed adult alveolar rhabdomyosarcoma (RMS). Systemic workup confirmed bilateral cervical lymph node metastasis. Currently the patient is undergoing chemotherapy. We describe a rare case of adult paranasal sinus RMS with orbital invasion.
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PMID:Adult rhabdomyosarcoma of the maxillary sinus with orbital extension. 2483 9