Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029463 (osteosarcoma)
 16,637 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

One hundred and ninety six thoracotomies were performed on 152 patients with pulmonary metastases up to 1988 in the Second Surgical Department, University of Vienna. Age ranged from 2 to 78 years, 13 patients were younger than 18 years. The primary tumour was carcinoma in 103 cases, sarcoma in 38 cases and melanoma in 11 cases. The primary tumour in young patients was osteosarcoma in 7 cases, Ewing sarcoma in 2 and Wilms tumour in 2 cases. With a minimal follow-up period of 2.5 years the actuarial 5 years survival rate of 37% was observed for carcinoma, and 29% for sarcoma patients. A statistical difference was found between the carcinoma and sarcoma groups with respect to survival rate; the prognosis for patients with melanoma was markedly worse. A prognostic factor was the duration of disease-free interval in carcinoma patients. Actuarial post-thoracotomy survival in patients with osteogenic sarcoma was 32% at 5 years and only 10% in the soft-tissue sarcoma group. Size of lesions, vitality of the metastases and disease-free interval correlated with survival in the osteogenic group, whilst the number of lesions was of importance in the soft-tissue group. On account of the lesser functional morbidity and the ability to assess both lungs for exploration, palpation and resections, the importance of median sternotomy is constantly increasing for the treatment of pulmonary metastatic disease and the results justify an aggressive approach. In those cases which the primary tumour is sensitive to chemotherapy the procedure of metastatic resection must be incorporated into the general scheme of oncological therapy.
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PMID:[Resection of pulmonary metastases: indications, surgical technic, results and prognostic factors]. 291 41

Successive loss of function of both alleles of the retinoblastoma susceptibility gene (RB) on human chromosome 13 seems to be critical in the development of retinoblastoma and osteosarcoma. In cases where the tumour is familial and susceptibility is inherited, a mutation in one of the alleles is carried in the germline. We have recently shown that cytogenetically visible germline mutations are usually in the paternally derived gene. Such a bias would not be expected for sporadic (non-familial) tumours, where both mutations occur in somatic tissue, but there has been some indication of a bias towards initial somatic mutation in the paternally derived gene on chromosome 11 in sporadic Wilms tumour. We have now examined 13 sporadic osteosarcomas and find evidence which indicates that in 12 cases the initial mutation was in the paternal gene, suggesting the involvement of germinal imprinting in producing the differential susceptibility of the two genes to mutation.
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PMID:Preferential mutation of paternally derived RB gene as the initial event in sporadic osteosarcoma. 291 36

A heritable mutation predisposes an individual to certain childhood malignancies, such as retinoblastoma and Wilms' tumor. The chromosomal locations of the genes responsible for the predisposition are known by linkage with chromosomal deletions and enzyme markers. A study of these tumors in comparison to the normal constitutional cells of the patients, using enzyme and DNA markers near the predisposing genes, has shown that these genes are recessive to normal wild-type alleles at the cellular level. Expression of the recessive phenotype (malignancy) involves the same genetic events that were observed in Chinese hamster cell hybrids carrying recessive drug resistance genes. In both the experimental and clinical situations, the wild-type allele is most commonly eliminated by chromosome loss with duplication of the mutant chromosome. Simple chromosome loss and mitotic recombination have been documented in both systems. In the remaining 30% of cases, inactivation or microdeletion of the wild-type allele are assumed to be responsible for expression of the recessive phenotype. Osteosarcoma is a common second tumor in patients who have had retinoblastoma. Studies with markers in osteosarcoma show that these tumors also result from unmasking of the recessive phenotype by loss of the normal allele at the retinoblastoma locus, whether or not the patient had retinoblastoma. Subsequent chromosomal rearrangements and amplification of oncogenes that occur in these homozygous tumors provide progressive growth advantage. In other malignancies, in which studies have so far focused on oncogene amplification and chromosomal rearrangements, unmasking of recessive mutations may also be the critical initiating events.
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PMID:Somatic events unmask recessive cancer genes to initiate malignancy. 302 9

Between 1981 and May 1986 31 children with solid abdominal tumor masses were observed in our clinic. The first diagnostic procedure was a sonographic examination, followed by further radiological investigations if necessary. 30 cases were examined histologically; in one case the sonographic findings were confirmed by an angiography. The most frequent abdominal masses were neuroblastomas and Wilms tumors (7 cases each). A mesoblastic nephroma was diagnosed in 3 cases, a lymphoma, a hepatoblastoma and a rhabdomyosarcoma 2 times each. One time we found a pancreas carcinoma, a teratoma, a hemangiomatosis of the liver, a malignant Schwannoma, a Ewing sarcoma, an adenoma of the adrenal gland, a pheochromocytoma and an osteosarcoma. According to our own experience and recent reports in the literature it seems possible in most cases, to predict the correct diagnosis of solid abdominal masses using the informations of sonographic imaging. Sonography is a highly specific non-invasive diagnostic tool for planning treatment (e.g. early surgery, cytostatic therapy and/or radiation) of solid abdominal masses. Nevertheless the histological examination should be performed in every case to confirm the definitive diagnosis.
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PMID:[Sonographic diagnosis of solid space-occupying abdominal lesions in childhood]. 303 88

A total of 452 cases of childhood malignant solid tumors were treated over the last twenty years at the National Children's Hospital. These included 175 cases of neuroblastoma, 64 cases of Wilms' tumor, 65 cases of malignant lymphoma, 45 cases of soft tissue sarcoma, 31 cases of hepatoma, 20 cases of malignant teratoma, 17 cases of testicular tumor, 7 cases of ovarian tumor and 28 cases of other forms of malignant solid tumor. Bone metastasis was observed in 62 of 175 cases of neuroblastoma, 3 of 64 cases of Wilms' tumor, one of 65 cases of malignant lymphoma, 4 of 45 cases of soft tissue sarcoma, one case of pulmonary blastoma and one case of osteogenic sarcoma, giving a total occurrence of bone metastasis in 72 of the 452 cases. The main sites of bone metastasis in neuroblastoma were the skull (61.4%), femur (56.8%), orbit (27.3%) and spine (22.7%). The average values of serum calcium and alkaline phosphatase activity showed no significant difference. The patients with bone metastasis were treated with a combination of radiation therapy and intensive chemotherapy, resulting in temporary improvement. The survival of patients with stage IV neuroblastoma with bone metastasis was worse than that of similar patients without bone metastasis.
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PMID:[Bone metastasis of malignant solid tumors in childhood]. 303 15

Ifosfamide was given to 61 patients with malignant solid tumors diagnosed before the age of 21 years. In this phase II study, all patients received 1.6 g/m2/day X 5 iv over 15 minutes followed by mesna at a dose of 400 mg/m2 iv at 15 minutes and 4 and 6 hours after ifosfamide. Responses were observed in five of 15 patients with osteosarcoma, two of ten with neuroblastoma, two of six with Wilms' tumor, two of five with rhabdomyosarcoma, four of eight with other soft tissue sarcomas, one of one with retinoblastoma, one of two with germ cell tumors, one of one with B-cell lymphoma, and one of one with a primitive neuroectodermal tumor. Fifty-nine of 61 patients had received prior alkylating agent therapy which included cyclophosphamide, cisplatin, mechlorethamine, melphalan, or dacarbazine. Fourteen of 19 responses developed in patients whose tumors were resistant to treatment with cyclophosphamide. A patient with malignant Schwannoma who had received no prior chemotherapy developed a complete response which lasted 12 months. A patient with brain metastases of osteosarcoma has had complete response for greater than 2 years. Complete response was also observed in a patient with B-cell lymphoma. Toxicity consisted of mild to moderate nausea and vomiting, transient reversible myelosuppression, occasional elevation of serum BUN or creatinine, and transient neurotoxicity characterized by somnolence, confusion, weakness, tremor, hallucinations, or seizures. We conclude that ifosfamide is an important alkylating agent without apparent complete cross-resistance with cyclophosphamide, and as such should be further investigated for determination of its activity in patients with pediatric neoplasms and considered for incorporation into phase II-III trials for certain tumors.
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PMID:Phase II trial of ifosfamide in children with malignant solid tumors. 310 34

This report evaluates the efficacy of extensive chest wall resection and prosthetic reconstruction in 15 children with chest wall malignancies. There were nine boys and six girls, with a mean age of 9.6 years. Eleven patients had primary chest wall tumors including Ewing's sarcoma (ES), six; rhabdomyosarcoma (RH), two; chondrosarcoma (CS), one; Askin's malignant neuroectodermal tumor, one; and mesenchymal sarcoma, one. Four children had metastases to chest wall and lung from Wilms' tumor (WT), two; osteogenic sarcoma (OS), one; and neuroblastoma (NB), one. Chest wall resection of two to six ribs and reconstruction with Marlex mesh (seven), lattisimus flap (two), prolene mesh (one), and more recently, a Gortex patch (five), was performed. Eight of the patients required concomitant en-bloc pulmonary resection (wedge, five; lobectomy, two; pneumonectomy, one) and two required resection of diaphragm. Fourteen received adjunctive therapy (chemotherapy, 14; irradiation, eight [preoperative, five; postoperative, three]. Six patients had second-look resections after chemotherapy. There was no operative mortality. Early pulmonary function was normal; however, pulmonary restrictive disease and scoliosis occurred with growth. One ES patient developed a radiation-induced second malignant tumor at age 10 and one ES child died at age 6 (no evidence of disease) of meningitis. Average survival length for ES patients was 77 months (range, 18 to 132 months.) Currently, eight patients are alive and five are free of disease. Extensive chest wall resection and reconstruction is useful in the treatment of primary chest wall tumors, but is palliative in metastatic cases. The Gortex patch is the current prosthetic of choice.
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PMID:Chest wall resection and reconstruction for malignant conditions in childhood. 320 68

One hundred and fifty-nine thoracotomies were performed in 122 patients with pulmonary metastases. The patients' ages ranged from 2 to 76 years, and 13 patients were younger than 18 years. The primary tumour was carcinoma in 83 cases, sarcoma in 29 cases and melanoma in 10 cases. The primary tumour in children was osteogenic sarcoma (6 patients), Ewing's sarcoma (2 patients) and Wilms' tumour (2 patients). With a minimum follow-up of 2 years, an actuarial 5-year survival rate of 38% was observed for carcinoma and 28% for sarcoma. Four of the children survived disease-free for 3 years or more after pulmonary metastasectomy. The primary tumour in these cases was osteogenic sarcoma and Ewing's sarcoma. A statistically significant difference in survival was found between the groups of carcinoma and sarcoma, but the prognosis for melanoma patients was markedly worse. In carcinoma patients the main prognostic factor was the duration of the disease-free interval. The actuarial postthoracotomy survival in patients with osteogenic sarcomas was 31% at 5 years, and 18% at 5 years in soft-tissue sarcomas. The size of the lesions, activity and disease-free interval correlated with survival in the osteogenic sarcoma group, and the number of lesions in the soft-tissue sarcoma group. An aggressive surgical approach towards pulmonary metastatic disease thus appears to be justified.
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PMID:Results and prognostic factors after resection of pulmonary metastases. 327 51

Hyperplasia of the thymus is a normal physiologic response in infants and children during recovery from life-threatening illness. New, recurrent, or residual mediastinal masses in children treated for malignant disease present a diagnostic dilemma. Are these masses recurrent disease or simply normal reactive thymic hyperplasia? Our experience from 1979 to 1986 includes 14 children aged 1 to 17 years (mean 7.4 years) who were identified with new or recurrent mediastinal masses during or after chemotherapeutic treatment for malignant disease (lymphoma 9, Wilms tumor 2, leukemia 1, osteosarcoma 1, malignant teratoma 1). The mediastinal masses were treated by a variety of methods depending on the attending physician's preference (close observation 2, oral steroids 5, steroids and subsequent biopsy 1, open biopsy 6). Chest roentgenograms of "observation only" patients have showed stable mediastinal changes without clinical evidence of recurrent disease. Patients treated with steroids showed resolution of the mediastinal masses in 48 hours to seven days, without recurrence. Patients undergoing open biopsy showed only thymic hyperplasia and/or lymph nodes. We suggest a stepwise approach to evaluation of these patients. Mediastinal masses occurring during, or shortly following, chemotherapeutic treatments of malignant disease should first be treated with oral prednisone (60 mg/m2/d x 7 to 10 d). If the patient shows a complete or partial resolution, then follow-up includes frequent chest roentgenograms and/or a second course of steroids. If the mass fails to respond to steroids, or enlarges, then open biopsy through a minithoractomy will clarify the diagnosis. Follow-up of our patients is from 3 months to 7 1/2 years (mean 5 years).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Mediastinal mass following chemotherapeutic treatment of Hodgkin's disease: recurrent tumor or thymic hyperplasia? 344 Sep 3

Twenty-nine consecutive patients 2-35 years old underwent serial thoracic CT evaluations for metastatic disease. Thymic volumes were determined for each patient during cycles of chemotherapy and were compared with the patient's clinical status. This group included patients with Hodgkin's disease (13 patients), osteogenic sarcoma (five), testicular neoplasm (four), Wilms' tumor (three), rhabdomyosarcoma (two), malignant fibrous histiocytoma (one), and Ewing's sarcoma (one). Seven patients with mediastinal lymphoma had tumor involvement of the thymus and therefore were excluded. The 22 remaining patients showed cyclic thymic volume changes in response to chemotherapy or its discontinuance. During the first course of chemotherapy the thymic volume decreased by an average of 43% in 20 of 22 patients. Between the first and second course, regrowth was observed in all 20 of these patients. Among the six patients who received a second course of therapy, an average volume decrease of 36% was observed during the second course with regrowth again occurring during recovery from chemotherapy. Thymic rebound (regrowth 50% greater than baseline volume) occurred in five patients, three of whom were in clinical remission. The thymus appears to atrophy during the administration of chemotherapy and regrow during the recovery phase of chemotherapy in 90% of the patients studied. Thymic hyperplasia or rebound is a relatively common phenomenon occurring in 25% of patients. The size of the thymus appears to be extremely sensitive to chemotherapy.
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PMID:Thymic atrophy and regrowth in response to chemotherapy: CT evaluation. 349 49


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