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)

An uncommon neoplasm of the larynx, rhabdomyosarcoma, was diagnosed in a 6-year-old 14-kg neutered female Spitz. In addition to the progressive onset of expected clinical signs of the tumor (exercise intolerance, respiratory stridor, inability to bark), the dog experienced hyperthermic crisis. Laryngectomy was successful in eradicating the local neoplastic tissue; however, unusual postoperative complications developed and included acute complications of pharyngotracheal fistula and hypoparathyroidism, and long-term complications of periodic collapse of the tracheal stoma and intolerance to heat. Probable causes and successful management of these complications are described. At 22 months after laryngectomy, the dog was admitted for a solitary hepatic metastasis. While hospitalized, the dog died of apparent asphyxiation attributable to stoma collapse.
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PMID:Development of hypoparathyroidism after excision of laryngeal rhabdomyosarcoma in a dog. 201 32

Life-threatening airway obstruction from large mediastinal masses in children poses a difficult diagnostic and therapeutic dilemma, requiring the close coordination of a pediatric surgeon, anesthesiologist, radiologist, and oncologist. To focus on this problem, the anesthetic and surgical management of 50 consecutive children with mediastinal masses treated between 1978 and 1984 were reviewed. Thirty children presented with respiratory symptoms; nine had life-threatening respiratory compromise with dyspnea, orthopnea, and stridor. Thirteen of these symptomatic children had marked compression of the trachea and/or mainstem bronchi on radiographic studies. The tracheal cross-sectional area which was measured by computed tomography was decreased by 35% to 93% of the normal tracheal dimensions in these children. Nonresectable malignant neoplasms including lymphoma, Hodgkin's disease, rhabdomyosarcoma, and neuroblastoma were the eventual diagnoses in 10 of these patients. The other 3 patients were less than 4 years old and had benign lesions. General anesthesia was judged to be prohibitively risky in 5 of 13 patients. The diagnosis was established by node or needle biopsy under local anesthesia, and general anesthesia was deferred until the compromised airway was alleviated by radiation and chemotherapy. General anesthesia with endotracheal intubation was administered to 8 patients, 5 of whom developed total airway obstruction. Using a variety of maneuvers, ventilation was reestablished in all 5 patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Life-threatening airway obstruction as a complication to the management of mediastinal masses in children. 408 8

A newborn male presented with respiratory distress and laryngeal stridor at the time of birth. Laryngoscopy revealed a circumscribed mass in the right vocal cord which was diagnosed as an undifferentiated malignant neoplasm on frozen section. Further light microscopic studies, special stains and electron microscopy disclosed features consistent with a special subtype of sarcoma adopted by the Intergroup Rhabdomyosarcoma Committee. Several authors have also demonstrated similarities between this type of tumor and soft tissue Ewing sarcomas. Nonepithelial malignancies of the larynx are rare in children and are only anecdotally reported in newborns. Histopathologically, the tumors predominantly include rhabdomyosarcomas among other rarer less well documented sarcomas and lymphomas. Although modern chemotherapy and radiotherapy have improved the otherwise grim prognosis of soft tissue Ewing sarcoma, this young patient was only treated with total laryngectomy at ten days of age and is alive and well two years later.
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PMID:Sarcoma of the larynx in a newborn. 618 33

At the age of three months an infant rapidly developed signs of cardiac failure as well as in- and exspiratory stridor, caused by an intrathoracic tumor. Thoracotomy and biopsy revealed an intrapericardial tumor, histologically myxosarcoma. In spite of chemotherapy and radiation the infant died at the age of seven months due to multiple intracerebral metastases now histologically rhabdomyosarcoma. This is one of the rare cases of primarily malignant intrapericardial tumors in infancy, and also shows the possible pleomorphism of childhood rhabdomyosarcoma. We know only one further case of pericardial rhabdomyosarcoma where similar histologic changes have been observed.
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PMID:[Intrapericardial rhabdomyosarcoma in infancy (author's transl)]. 732 26

A seven-month-old male presented with a one month history of an enlarging left neck mass and worsening inspiratory stridor. Upon excision of the mass, pathologic examination was consistent with embryonal rhabdomyosarcoma (RMS). Preoperative imaging and intraoperative exploration were consistent with tumor replacing the left lobe of the thyroid. No cases of either anterior neck rhabdomyosarcoma or thyroid rhabdomyosarcoma have been explicitly described in the literature. The distinction between the two malignancies becomes important when considering prognosis and treatment protocols.
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PMID:Rhabdomyosarcoma presenting as an anterior neck mass and possible thyroid malignancy in a seven-month-old. 1565 63

An immature Baird's tapir (Tapirus bairdii) with a history of seizure-like episodes developed signs of respiratory disease. The initial clinical diagnosis was pneumonia, and antibiotic therapy was started. The animal failed to improve after 14 days of therapy and developed unilateral, bloody nasal discharge. Endoscopic examination and radiography revealed a soft tissue mass in the nasopharynx depressing the soft palate. The tapir died 32 days after initial presentation. Histologic examination of the mass demonstrated a mesenchymal tumor composed of spindle cells with elongate nuclei forming densely packed fascicles. The neoplastic spindle cells showed prominent cross-striations. Immunohistochemistry revealed the cells to be positive for desmin and myoglobin, but negative for smooth muscle actin, confirming diagnosis of rhabdomyosarcoma. Embryonal rhabdomyosarcoma is the most common nasopharyngeal soft tissue tumor of humans, and it has been reported infrequently in dogs, horses, and pigs. Neoplasia should be a differential diagnosis in cases of unilateral nasal discharge and inspiratory stridor, even in young animals.
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PMID:Embryonal rhabdomyosarcoma in an immature Baird's tapir (Tapirus bairdii). 1746 86

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

Rhabdomyosarcomas in the parapharyngeal space are quite rare. We report herein on the case of a 14-month-old boy who was hospitalized with a 2-month history of stridor. On admission, right peritonsillar swelling was noted. CT demonstrated the presence of a large tumor in the right parapharyngeal space. MRI findings showed a right parapharyngeal tumor, 6-cm in diameter. Histopathologic evaluation of the tumor revealed embryonal rhabdomyosarcoma. The clinical staging of the Intergroup Rhabdomyosarcoma Study Group (IRSG) was classified as group III. According to regimen 35 of the IRSG III treatment protocol, radiochemotherapy was started, comprising combination therapy with vincristine, actinomycinD, cyclophosphamide, adriamycin, and carboplatin. The patient tolerated the therapy, but with severe pancytopenia and fever. His sedation during irradiation was difficult. After he received a total dose of 10 Gy and had undergone 9 cycles of chemotherapy, an operation for the rhabdomyosarcoma was performed, resulting in successful removal of the tumor. Doses of 20 Gy were intraoperatively administered. After surgery, residual chemotherapy and irradiation was resumed. The evaluation of the response to therapy was complete remission. After 4 years from the start of therapy, all chemotherapy was halted. At present there is no evidence of recurrence or metastasis, and the patient is a well-developed college student. His sequela following therapy consisted of facial asymmetry, dental abnormalities, and pigmentation of the neck. No adriamycin cardiomyopathy was found.
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PMID:A Case of Infantile Large Parapharyngeal Rhabdomyosarcoma Followed Up for More Than 20 Years. 3005 85