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)

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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PMID:Adult rhabdomyoma of the extremity: a case report and review of the literature. 1101 74

To examine the clinical features of primary cardiac tumors, 34 patients who underwent surgical treatment from 1973 to 2000 at the Kanazawa University Hospital were analyzed and the literature was reviewed. The 34 patients were divided into 3 categories: (i) myxomas; (ii) benign non-myxomas; and (iii) malignant tumors. Twenty-three patients (70%) were diagnosed with myxomas, including 22 left atrial myxomas and 1 right atrial myxoma. Seven patients (18%) were diagnosed with benign non-myxoma tumors, including 3 hemangiomas, 1 fibroma, 1 rhabdomyoma, 1 pheochromocytoma, and 1 lipoma. Four patients (12%) were diagnosed with malignant tumors, including 2 angiosarcomas, 1 rhabdomyosarcoma, and 1 malignant fibrous histiocytoma. Among the myxoma patients, in-hospital mortality was 9% (2/23), late mortality was 10% (2/21), and no recurrent myxomas have been identified. Among benign non-myxoma patients there were no perioperative deaths; however, 1 patient died 11 years after surgery, with no linked cause. No recurrent tumors have been identified. Among malignant tumor patients, 1 patient died the day following surgery and the rest died within 14 months. Early and late results of surgery were acceptable for those patients with benign tumors, while the prognosis for patients with malignant tumors was very poor.
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PMID:Surgical treatment of primary cardiac tumors: 28 years' experience in Kanazawa University Hospital. 1131 30

Rhabdomyoma in adults is a rare benign neoplasm of the skeletal muscles generally arising in the pharyngolaryngeal region. These slow-growing tumors remain asymptomatic for a long period. We report a case of basilingual rhabdomyoma. Computed tomography and magnetic resonance imaging demonstrated a well-encapsulated tumor with a line of cleavage between the base of the tongue muscles. Deep transmucosal biopsies were obtained. Well-differentiated skeletal muscle cells with large eosinophilic granulular cytoplasm and cross striations gave the histologic diagnosis. Complete endoscopic resection was successful. The differential diagnoses of rhabdomyoma in adults are myoblastoma or Abrikossof tumor, rhabdomyosarcoma and hibernoma. The risk of late recurrence implies long-term clinical and radiologic follow-up.
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PMID:[Basilingual rhabdomyoma treated by endoscopy]. 1167 44

A case of rhabdomyoma of the spermatic cord in an infant with cryptorchidism is reported. This extremely rare tumor has not been described in an extrascrotal position or at such a young age. Differential diagnosis includes malignant rhabdomyosarcoma. Although fetal-type rhabdomyoma of the head and neck generally is a benign tumor, the prognosis of paratesticular fetal-type rhabdomyoma is unknown. Long-term follow-up is recommended.
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PMID:Paratesticular rhabdomyoma. 1457 86

Cardiac tumors in infants and children are extremely rare. Their clinical manifestations vary widely from asymptomatic presentations to life-threatening cardiac events. Improvements in diagnostic techniques, such as those offered by echocardiography, have made early detection of cardiac masses possible, with or without the presence of clinical symptoms. Fifteen pediatric cases of cardiac tumor were diagnosed at our institution between July 1989 and July 2002 (male-female ratio, 10:5; age range, one day to nine years). Eleven of the cases involved primary cardiac tumors [rhabdomyoma (n = 10) and fibroma (n = 1)]. Ninety percent of the rhabdomyomas (9/10) were associated with tuberous sclerosis. Four of the fifteen cases were secondary metastatic tumors [hepatoblastoma (n = 2), hepatoma (n = 1) and rhabdomyosarcoma (n = 1)]. Clinical manifestations of the cardiac tumors included shortness of breath (n = 5), seizure (n = 4), cardiac murmur (n = 6), and cyanosis (n = 3). Surgery was performed for three of 11 patients with primary cardiac tumor (27%) due to severe obstruction of flow (n = 2) and other cardiac defects (n = 1). The primary cardiac tumor spontaneously regressed in five of the tuberous sclerosis patients. All four of the patients with secondary cardiac tumors continued to receive chemotherapy, and only one of them subsequently experienced regression. Based on our experiences, we conclude that: 1) rhabdomyoma is the most common primary cardiac tumor in children; 2) most pediatric tumors are associated with tuberous sclerosis; 3) clinical presentation is determined by the tumor size and number of tumors, and whether expansion of the malignancy has resulted in cardiac blood-flow obstruction; 4) there is a strong possibility of regression of the primary cardiac tumor, with surgery recommended only when cardiac symptoms are severe; and, 5) unless there is a significant obstruction of blood flow, chemotherapy is still the treatment of choice for secondary cardiac tumors.
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PMID:Cardiac tumors in infants and children. 1467 25

A cystectomy for indications other than transitional cell cancer of the bladder or general bladder cancer is frequently performed in cooperation with other surgical specialties such as general surgery or gynecology. In these cases the urological procedure as well as the oncological and surgical concepts of other specialties have to be combined. We studied our cystectomy patients who had undergone such a combined procedure for a non-urothelial indication concerning perioperative and postoperative complications.A total of 204 cystectomies were performed by the Department of Urology at the University of Hamburg, Germany between 1995 and 2003. Bladder cancer was the indication for cystectomy in 162 patients, but 42 patients had a non-urothelial indication for this procedure. These patients included 12 cases of advanced rectal cancer, 9 cases of advanced cervical cancer, 6 cases of advanced sigmoid cancer, 4 cases of advanced prostate cancer, 1 case of prostate sarcoma, 5 cases of complex vesicointestinal fistulae, 2 cases of urachal cancer, 1 leiomyosarcoma, 1 rhabdomyoma, and 1 rhabdomyosarcoma, respectively. Perioperative and postoperative complications of those patients were compared to patients who underwent cystectomy for transitional cell cancer of the bladder.Those 42 patients who underwent cystectomy for non-urothelial indications included 14 male and 28 female patients. The mean age was 58.2 years with a range of 3-78 years. For urinary diversion 30 ileum conduits, 4 sigma conduits, and 8 ileum neobladders were used. The mean operative time was 6.25 h. The mean blood loss was 2200 ml. An average of four red blood cell concentrates (RBC) had to be given. Postoperative hydronephrosis had to be treated in three (7%) patients unilaterally and in two (5%) patients bilaterally with a temporary nephrostomy. Postoperative urinary leakage lasting more than 30 days was found in two (5%) patients. A deep vein thrombosis as well as an ileus was found in five (12%) patients each, respectively. There was no perioperative mortality in this study. When comparing the complications of those patients with the 162 patients who underwent cystectomy for bladder cancer, the only significant difference ( p=0.033, chi-square test) was a higher ileus rate in the patients with cystectomy for a non-urothelial indication. Complications with cystectomy for non-urothelial indications are in large comparable to those for transitional cell carcinoma of the bladder. The higher ileus rate in non-urothelial patients can be explained by the more radical procedures in this group of patients. Even though the group of patients undergoing cystectomy for indications other than bladder cancer was small in this trial, the procedure is standardized in combination with other specialties. Larger patient numbers and a longer follow-up will lead to more data in this special group of patients.
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PMID:[Cystectomy for indications other than bladder cancer]. 1499 Nov 19

A mass of the neck in an 8-year-old boy diagnosed with recurrent fetal cellular rhabdomyoma was reported.The recurrent tumor consisted of undifferentiated round and short spindle cells and immature striated muscle cells,which was similar to the primary tumor occured in five years ago.However,there were more large round myoblasts which mainly located at the periphery of the tumor and osteoid stroma in the recurrent case immuno-histochemistry stain and ultrstructure observation suggested that these were undifferntiated mesenchymal cell and myoblasts.The differential diagnosis from fibrosarcoma,highly differentiated rhabdomyosarcoma,and hemangiopericytoma were dicussed.Author considered FRMs has a recurrent and malignant change tendency though it belongs to benign tumor.
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PMID:[Recurrent fetal cellular rhabdomyoma:histopathological and ultrastructural observations] 1515 99

Malignant tumors are extremely uncommon in infants, specifically in the head and neck region. We present a three-day-old infant with a large, polypoid, soft tissue mass arising from the floor of the mouth. Histologically, this neoplasm consisted of hypercellular and myxoid areas. A mixture of poorly oriented, small, undifferentiated, hyperchromatic, and round to elongate spindle cells was seen. A high degree of striated muscle differentiation was present, along with areas marked by a herringbone pattern, as well as hemangiopericytic vessels and rare mitosis. Immunohistochemical examinations revealed strong nuclear staining for myogenin and diffuse cytoplasmic staining for desmin and muscle-specific actin (HHF-35). The tumor did not stain for S-100. Based on histologic results and immunostains, this lesion was diagnosed as spindle cell rhabdomyosarcoma. This type of lesion involving the tongue is rarely seen in females, neither in association with a herringbone pattern nor with hemangiopericytic vessels. Furthermore, rare benign and malignant spindle lesions, such as cellular fibromatosis, fetal rhabdomyoma, infantile hemangiopericytoma, infantile rhabdomyofibrosarcoma, and infantile fibrosarcoma, should be in the differential diagnosis and excluded.
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PMID:Spindle cell rhabdomyosarcoma of the tongue in an infant: a case report with emphasis on differential diagnosis of childhood spindle cell lesions. 1546 1

A case of fetal rhabdomyoma (myxoid type) of the head and neck demonstrated on prenatal magnetic resonance imaging (MRI) is presented. This benign tumor of skeletal muscle is uncommon and should not be confused with its malignant counterpart-rhabdomyosarcoma. With the increasing use of ultrafast MRI, the radiologist is more likely to encounter head and neck masses in the fetus.
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PMID:Rhabdomyoma of the head and neck demonstrated by prenatal magnetic resonance imaging. 1566 99

Fetal rhabdomyoma is a rare benign neoplasm of skeletal muscle that must be distinguished histologically from various malignant lesions, including rhabdomyosarcoma. They have recently been subdivided into two histological variants myxoid and cellular fetal rhabdomyomas. This article describes a case of an 8 month infant with a fetal rhabdomyoma in the upper lip. Illustrate the histological features and discusses the means of distinguishing these lesions from various other conditions with which they may be confused.
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PMID:Fetal intermediate rhabdomyoma of the lip: case report. 1571 26


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