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 pathologic material from 56 patients diagnosed initially as Ewing's sarcoma of the distal extremity and treated on National Cancer Institute protocols between 1968 and 1984 was reviewed and correlated with clinical outcome. Histologically, the tumors were categorized, based on recent pathologic criteria, into three diagnostic groups: (1) typical Ewing's sarcoma, (2) atypical Ewing's sarcoma, and (3) other (predominantly peripheral neuroepithelioma [PN]). Thirty-two patients (57%) had typical Ewing's, 13 (23%) were atypical, and 11 (20%) were in the "other" diagnostic category (seven [13%] PN, two primitive rhabdomyosarcoma, one primitive sarcoma of bone, and one synovial cell sarcoma). No cases of metastatic neuroblastoma, osteosarcoma, or lymphoma were found. Forty-five patients had localized disease at diagnosis; 11 had metastases. Patients with typical Ewing's sarcoma were less likely to have metastatic disease at the time of diagnosis. Only two of 32 patients with typical Ewing's sarcoma had metastases compared with nine of 24 patients in the two other groups. The pattern of relapse was also different in these other groups compared with typical Ewing's patients; five patients developed lymph node metastases and two patients developed brain metastases. Although the presence of metastatic disease at diagnosis was a strong negative prognostic factor, our histologic grouping was independently prognostic of clinical outcome in patients with localized disease. Patients with typical osseous Ewing's sarcoma had an improved overall survival (P2 = 0.03) and patients with other tumors (neither typical nor atypical Ewing's sarcoma) had a poorer disease-free survival (P2 = 0.02). Since no generally accepted histopathologic prognostic criteria exist for Ewing's sarcoma, the potential value of our proposed classification should be evaluated in a larger retrospective and a prospective study.
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PMID:Prognostic value of histopathology in Ewing's sarcoma. Long-term follow-up of distal extremity primary tumors. 198 13

The authors present 15 patients with middle ear malignant tumours, of which 13 were squamous cell carcinomas, one was a rhabdomyosarcoma and one a malignant lymphoma. In 11 of 13 patients with squamous cell carcinoma, long-standing chronic otitis media preceded the malignant process and only in 2 patients did the tumour develop without previous chronic infection. Most of the patients presented at an advanced stage of the disease: in 6 the tumour had destroyed surrounding bone, commonly involving the endocranium; in 7 the facial nerve was paralysed and in 8 patients there was total hearing loss. The diagnosis of malignant tumour was established before surgery in 5 patients, but 8 patients were operated upon for suspected chronic otitis. Three patients died without treatment and 10 were treated: 3 with radiotherapy because the tumour was inoperable, 3 had surgery only and 4 patients had surgery and received radiotherapy post-operatively. Of 7 operated patients only 2 are alive without signs of tumour. Our findings suggest that otologists should bear in mind this disease in patients with chronic suppurative otitis and accompanying suspected symptoms. In 2 patients with rare types of sarcoma of the middle ear the diagnosis of tumour was established at an advanced stage and they could not be treated.
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PMID:Malignant tumours of the middle ear. 203 67

The expression of transferrin receptor (Tr) was investigated by means of immunohistochemistry in 101 tumours of the human central and peripheral nervous system. The results were compared with the proliferative activity of the tumours, determined by immunostaining for the proliferation-associated antigen Ki-67. In addition to immunostaining of normal and proliferated blood vessel endothelium and of a fraction of tumour infiltrating lymphocytes, we observed staining for Tr in a variable fraction of neoplastic cells of all histological tumour types. Immunoreactivity in the majority of tumour cells was found only in anaplastic tumours such as glioblastomas. Furthermore, a positive correlation between Tr expression and the Ki-67 growth fraction was established for gliomas. Non-glial tumours strongly expressing Tr included one metastatic rhabdomyosarcoma, one intracerebral malignant lymphoma, two of four plasmocytomas and seven of nine metastatic carcinomas. Our results indicate that immunohistochemistry for Tr and Ki-67 can provide additional information about the biological behaviour of nervous system tumours, thus complementing conventional histopathological criteria for anaplasia.
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PMID:Transferrin receptor expression in tumours of the human nervous system: relation to tumour type, grading and tumour growth fraction. 211 Jun 96

Studies were made on the appearance of second malignant tumors (SMT) in children followed in a pediatric hospital at metropolitan Santiago, Chile, between years 1968 and 1987. A retrospective analysis identified SMT in 7 of 430 patients who survived a childhood cancer (incidence 1.62%). An 8th patient was added, whose first neoplasm was treated in another hospital. The initial diagnosis in the affected children were medulloblastoma, neuroblastoma, Wilm's tumor retinoblastoma, Ewing's sarcoma, Hodgkin's disease and, in two cases, acute lymphocytic leukemias. The age range was 6 months to 11 years. Treatment was done by surgery in 5/8, chemotherapy in 7/8 and radiotherapy in all patients. The latent period between the diagnosis of the first cancer and the diagnosis of the SMT was 3.5 to 12 years (median 8.5 years). Osteosarcomas were the most frequent SMT (5/8). The other SMT were a rhabdomyosarcoma, a non Hodgkin lymphoma and an astrocytoma. The majority of SMT were located in the area of prior radiotherapy (6/8). In the other two cases, one had an osteosarcoma, after a bilateral retinoblastoma, which grew outside the previously treated area, and the last one consisted of a lymphoma which was identified 9 years after an acute lymphocytic leukemia. Only 3/8 SMT patients are alive after 14.21 and 34 months follow up. The other children died between 11 and 20 months after diagnosis of SMT. Notwithstanding these kinds of outcome, benefits of therapy for patients with primary tumors greatly outweight the later risk of cancer induction in a small proportion of them.
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PMID:[Second cancer in pediatric patients]. 213 86

A case of abdominal Burkitt's lymphoma diagnosed through aspiration cytology is described. This 9-year-old boy presented with abdominal pain and distention for three months accompanied by fever and night sweat during the last month. An abdominal sonography and CT scan showed hepatosplenomegaly and an intrahepatic mass with celiac lymph node enlargement, ascites, and pleural fluid. A peripheral blood smear showed a few blast cells. Aspiration of the abdominal mass revealed very cellular aspirates consisting of diffusely scattered small monotonous round cells. The cells had little cytoplasm, along with round nuclei that showed clear-cut nuclear membrane, coarse chromatin pattern, and multiple small prominent nucleoli. Differential diagnoses considered were small round cell sarcomas such as malignant lymphoma, neuroblastoma, Ewing's sarcoma, and rhabdomyosarcoma. Of these, malignant lymphoma of the small noncleaved cell type was most consistent with the results of several studies including immunohistochemical staining, peripheral blood smear, and bone marrow biopsy. The cells were positive for leukocyte common antigen (LCA) and showed finely vacuolated basophilic cytoplasm in both the peripheral blood smear and bone marrow biopsy, characteristic of Burkitt's lymphoma cells.
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PMID:Abdominal Burkitt's lymphoma diagnosed by fine needle aspiration cytology--a case report. 227 68

Lymphoma of the head and neck in children can pose a significant diagnostic problem, especially when histologic analysis indicates non-Hodgkin's lymphoma and the initial site of involvement is extranodal. This report describes 15 pediatric cases of lymphoma seen from 1981 to 1987 with an initial presentation in the head and neck. Cervical lymph nodes represented the initial site of involvement in 10 of the cases. The other five cases presented with disease in the tonsillar fossa; maxillary sinus and mandible; parotid; pharyngeal wall; trachea and thyroid gland; and ethmoid sinus, sphenoid sinus, and anterior fossa. The histologic type was non-Hodgkin's lymphoma in 12 cases and Hodgkin's lymphoma in 3 cases. Our experience has shown that lymphoma of the head and neck in children presents a confusing clinical picture and was initially confused with inflammatory disease, polymorphic reticulosis, and other neoplasms such as rhabdomyosarcoma. In one patient, Epstein-Barr virus infection and an inherited immunodeficiency state probably played a role in the pathogenesis of the lymphoma.
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PMID:Unusual presentations of lymphoma of the head and neck in childhood. 231 81

A review of 52 patients with celiac disease showed the development of malignant tumors in eight cases (15%). The following malignomas were diagnosed: one malignant lymphoma, one multiple myeloma, one rhabdomyosarcoma, one carcinoma of the uterus, one carcinoma of the sigmoid colon and three adenocarcinomas of the small bowel. Patients with tumors showed significantly lower hemoglobin, lower serum albumin, and higher sedimentation rates than patients without tumors. The possibility of underlying malignoma must always be considered in all patients with newly diagnosed coeliac disease and in patients where symptoms of a known celiac disease change without alteration of the prescribed diet.
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PMID:[Risk of malignancies in celiac disease--a retrospective study]. 233 21

One hundred and sixty-three male and 78 female cases of lung sarcoma, registered over 29 years from 1958 to 1986, were selected and analyzed, and were compared with lung cancer cases. The incidence of the lung sarcoma cases was one per 2,600 in males and one per 3,600 in females among all autopsy cases, and one per 1,400 in males and one per 1,800 in females among all autopsied cases of fatal malignancies. The relative incidence of lung sarcoma was one per 240 lung cancer cases in males and one per 170 in females and gradually decreased with each decade (1st to 3rd periods). The male/female ratio was 2.1 overall and 2.6 in the 3rd period. In the 3 decades, the average age at detection 55, 55 and 66 years in males and 38, 51 and 49 years in female lung sarcoma cases. Female sarcoma cases were significantly younger than male cases, except for the 2nd period. In the 3rd period male cases were almost the same as lung cancer cases in terms of average age. Significant elevation was observed in lung sarcomas. The most frequent lung sarcomas in males were rhabdomyosarcoma, leiomyosarcoma, and malignant lymphoma in the 3 decades. In females, the above three sarcomas were most frequent in each chronological period. Malignant fibrous histiocytomas appeared remarkably in the 3rd period in both sexes. The peak and mean ages for each type of lung sarcoma were higher than other reports. In malignant lymphoma and rhabdomyosarcoma, male cases were significantly older than female cases.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Chronological changes of lung sarcoma and lung cancer incidence based on the annual of the pathological autopsy cases in Japan (1958-1986)]. 235 73

Postirradiation sarcomas are an unusual but well-recognized late effect of cancer therapy. In this article, a fine-needle aspiration (FNA) series of four cases is presented. There were three female patients and one male patient, with an age range of 28-55 yr (mean, 41). Two of the patients were irradiated for uterine cervical carcinoma while the other two received irradiation for malignant lymphoma. The time interval to the development of the postirradiation sarcoma ranged from 10 to greater than 20 yr. There were a postirradiation synovial sarcoma of the buttock region, malignant fibrous histiocytoma of the bone (femur), and rhabdomyosarcoma and angiosarcoma of the retroperitoneum. A spectrum of cytologic findings was encountered, reflecting the specific types of sarcomas. Immunocytochemical studies performed on the aspirated material from the angiosarcoma demonstrated the utility of immunoperoxidase stains for ULEX europaeus agglutinin-1 (UEA-1) and, to a lesser degree, factor VIII-related antigen antibody, confirming the vascular nature of this malignancy. The FNA findings from all four cases demonstrated cytologic features that allowed recognition of this unusual complication of irradiation treatment. This article confirms the utility of FNA cytology in following patients with previous malignancies and differentiating a postirradiation sarcoma from recurrent carcinoma.
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PMID:Fine-needle aspiration cytology of postirradiation sarcomas, including angiosarcoma, with immunocytochemical confirmation. 247 5

Sinonasal neoplasms and neoplasm-like proliferations composed of light microscopically poorly differentiated or undifferentiated, small- to medium-sized cells cause considerable diagnostic confusion. Lesions in this category include lymphoepithelioma (undifferentiated carcinoma), olfactory neuroblastoma, small-cell undifferentiated (oat cell) carcinoma, sinonasal undifferentiated carcinoma, malignant melanoma, pituitary adenoma, lymphoid hyperplasia, malignant lymphoma, plasmacytoma, lymphomatoid granulomatosis, rhabdomyosarcoma, mesenchymal chondrosarcoma, small cell osteosarcoma, Ewing's sarcoma, and synovial sarcoma. Many of these lesions can be definitively diagnosed based on light microscopic features alone, but, in some instances, additional techniques such as immunohistochemistry are of value. The authors review the pertinent clinicopathologic features of the above lesions, with emphasis on light microscopic, immunohistochemical, and ultrastructural features of particular utility in differential diagnosis.
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PMID:"Undifferentiated" neoplasms of the sinonasal region: differential diagnosis based on clinical, light microscopic, immunohistochemical, and ultrastructural features. 269 5


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