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

Ten patients under 20 years of age, with malignant tumors of the head and neck was treated at the Department of Otorhinolaryngology of Kitasato University Hospital from August 1971 to December 1989. The primary lesions were situated in the nose and paranasal sinuses in 3, middle ear in 2, epipharynx in 2, and parapharynx, esophagus, and neck in 1 patient. Histological examination indicated 3 rhabdomyosarcomas, 2 malignant lymphomas, and 1 each of neuroblastoma, malignant neuroendocrine tumor, transitional cell carcinoma, lymphepithelioma, and squamous cell carcinoma. The sites of origin and histopathology of malignant tumors in such patients usually differ from those in adults. Well differentiated squamous cell carcinoma of head and neck is common in adults but not in children, in whom non-epithelial malignant tumors or sarcomas are not rare. Radiotherapy is more effective for treating malignant tumors of the head and neck in young than in adults. Eight of 10 patients are still alive, 7 of whom for 5 years or more. Two with rhabdomyosarcoma died.
Auris Nasus Larynx 1993
PMID:Malignant tumors of the head and neck in young patients. 821 50

Embryonal rhabdomyosarcoma of the head and neck is very rare in adults. We report on an embryonal rhabdomyosarcoma of the nose, occuring in a 74-year-old female patient presenting with nasal congestion as the only clinical symptom. Suspected to be an amelanotic melanoma, further biopsies and histological examinations provided the correct diagnosis. She first underwent a course of chemotherapy which led to a partial shrinkage of the tumor mass which was followed by a complete resection of the residual tumor mass. Biologic behaviour of this tumor must be fully appreciated as treatment is interdisciplinary. Rhabdomyosarcoma should be considered in the differential diagnosis of tumors of the nose and paranasal sinuses not only in children, but also in adults.
Auris Nasus Larynx 1998 Dec
PMID:Embryonal rhabdomyosarcoma of the nose in an elderly patient, case report and review of the literature. 985 69

An elderly woman having a nasal tumor diagnosed as alveolar rhabdomyosarcoma was referred to our hospital. Histological reexamination of the patient revealed that the tumor was composed of lobules of compactly arranged small round atypical cells. The atypical cells contained large oval to round vesicular nuclei, and scanty cytoplasm showing PAS positive glycogen material. The tumor cells were immunohistochemically positive for NSE, S-100 protein and vimentin, but showed negative reaction for myoglobin, desmin, EMA, keratin, LCA, chromogranin and MIC2. Ultrastructurally, the tumor cells contained a few cluster of glycogen particles and less organellae and filaments, and there were no cytoplasmic processes, neurosecretory granules or neurofilaments. Pathological findings suggested primitive neuroectodermal tumor resembling extraskeletal Ewing's sarcoma, but negative reaction for MIC2 immunohistochemically could not ascertain the diagnosis. Location of the tumor and age of the patient of the present case were unusual.
Auris Nasus Larynx 1999 Jan
PMID:Primitive neuroectodermal tumor in sinonasal region. 1007 61

Radiation-associated (RA) osteosarcomas (OS) are exceptional in children, presenting more frequently in middle-aged and elderly patients. This is a case report of RA-OS of the sinonasal cavities in a 13-yr-old girl after combined therapy for rhabdomyosarcoma (RMS) of the nose diagnosed at the age of five. The treatment of choice is radical surgery with wide margins and postoperative adjuvant chemotherapy and radiotherapy. Despite an episode of recurrence, at the present time the patient is alive and free of disease 5 yr after the initial treatment of OS. The association between radiation, chemotherapy and sarcomas is reviewed and discussed.
Auris Nasus Larynx 2001 Aug
PMID:Sinonasal radiation-associated osteosarcoma after combined therapy for rhabdomyosarcoma of the nose. 1148 73

Hypohidrotic ectodermal dysplasia (Christ-Siemens-Touraine syndrome) is characterized by partial or complete absence of sweat glands, hypotrichosis, hypodontia, prominent frontal ridges and chin, saddle nose, sunken cheeks, thick, everted lips, large ears and sparse hair. While association of other ectodermal dysplasia syndromes with tumors such a non-Hodgkin's lymphoma, hamartoma, keratoakanthoma, Merkel-cell cancer, squamous-cell carcinoma, syringofibroadenomatosis has been reported, association of hypohidrotic ectodermal dysplasia and a tumor has not been reported. In a five-year-old male patient admitted with nasal obstruction and nasal mass complaints, we have reported firstly an association of hypohidrotic ectodermal dysplasia and nasopharyngeal rhabdomyosarcoma.
Auris Nasus Larynx 2002 Jul
PMID:Nasopharyngeal rhabdomyosarcoma in a patient with hypohidrotic ectodermal dysplasia syndrome. 1216 59

Adult rhabdomyosarcoma of the larynx is a rare disease. Two cases of laryngeal rhabdomyosarcoma of the pleomorphic subtype are presented. One case was treated with surgery followed by chemotherapy and radiotherapy, the other by surgery alone. At present 20 months after treatment the patients are without local reccurrence or metastases. The pathology of the tumor, diagnosis, and treatment are discussed.
Auris Nasus Larynx 2007 Dec
PMID:Laryngeal pleomorphic rhabdomyosarcoma. 1748 38

We describe the histopathological temporal bone findings of metastatic rhabdomyosarcoma in the patient with hearing loss and facial nerve paralysis. A 45-year-old female was admitted to the hospital with a mass of the left tibia. Surgical operation was performed and the pathological diagnosis was alveolar rhabdomyosarcoma. She showed left facial palsy and bilateral hearing loss. She was pronounced dead because of respiratory arrest caused by multiple metastases including meningeal metastasis after 14 months of total clinical course. Histological examination of the temporal bone revealed that the VII and VIII cranial nerves were involved by tumor cells in the internal auditory canal, and that the organ of Corti was damaged. In addition, the membranous labyrinth showed labyrinthitis. No remarkable bony destruction was observed in the internal auditory canal. Histopathological examination revealed that hearing loss and facial nerve paralysis of the patient was due to temporal bone metastasis of rhabdomyosarcoma.
Auris Nasus Larynx 2009 Apr
PMID:Histopathological temporal bone study of the metastatic rhabdomyosarcoma. 1860 9