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)

As children with cancer survive longer, the incidence of second malignant neoplasms has increased considerably. We describe here three cases of second solid tumors after 12, 8 and 2 years of initial diagnosis of cancer: one osteosarcoma of left maxilla in a previously treated child with bilateral retinoblastoma, a temporal astrocytoma associated with acute lymphoblastic leukemia and a glioblastoma multiforme in a girl with neurofibromatosis de Von Recklinghausen, after Non Hodgkin lymphoma, respectively. We review the literature about the influence of genetic, immunologic and therapeutic factors involved in the appearance of these second tumors.
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PMID:[Second solid tumors in childhood. Review based on three cases]. 166 21

Excluding multiple myeloma, osteosarcoma is the most frequent primary malignant bone tumor. However, it is rare in the jaw bones, where it mainly occurs in young adult men. This report describes two mandibular osteosarcomas found in two males aged 32 and 30. In the former patient, osteosarcoma developed after bilateral mandibular sclerotic changes had been diagnosed radiologically as cementifying fibroma. This patient had received radiation therapy (56 Gy) because of a high grade astrocytoma of the brain three and a half years earlier. In the latter patient the tumor arose de novo in the mandibular molar area. The initial main symptom both patients was pain, which had been considered to be of dental origin because radiographs showed no evidence of malignancy. Radical surgery using immediate mandibular reconstruction with revascularized iliac bone grafts gave good results in both cases. The former patient died eight months after surgery because of recurrent astrocytoma and the latter is now free of disease five years after surgery. Immediate reconstruction of mandibular defects is possible with osteocutaneous groin flaps.
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PMID:Immediate reconstruction of mandibular defects with revascularized iliac bone grafts after radical surgery for osteosarcoma. 209 48

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

Survival rates were analysed for a population-based series of over 15,000 childhood cancers registered in Great Britain during 1971-85. There were highly significant improvements (P less than 0.001 for trend) in survival for many major diagnostic groups. Between 1971-73 and 1983-85 the actuarial 5-year survival rates increased from 37% to 70% for acute lymphoblastic leukaemia, from 4% to 26% for acute non-lymphoblastic leukaemia, from 76% to 88% for Hodgkin's disease, from 22% to 70% for non-Hodgkin's lymphoma, from 61% to 72% for astrocytoma, from 24% to 42% for medulloblastoma, from 15% to 43% for neuroblastoma, from 58% to 79% for Wilms' tumour, from 17% to 54% for osteosarcoma, from 26% to 61% for rhabdomyosarcoma, from 59% to 94% for malignant testicular germ-cell tumours and from 43% to 77% for malignant ovarian germ-cell tumours. These increases in population-based survival rates reflect the substantial advances in treatment of a wide range of childhood cancers since 1970. The two principal diagnostic groups for which there was no evidence of any trend were retinoblastoma, which already had an excellent prognosis with a 5-year survival rate of over 85%, and Ewing's sarcoma, for which the survival rate remained below 45%.
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PMID:Trends in survival for childhood cancer in Britain diagnosed 1971-85. 217 43

A secondary astrocytoma appearing 3 years after diagnosis of osteosarcoma is reported in a boy 11 years old. The boy had been treated with cytostatics according to the T-10 protocol. Treatment had been discontinued 2 years before diagnosis of the astrocytoma.
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PMID:Secondary cerebral astrocytoma 3 years after diagnosis of primary osteogenic sarcoma: a case report. 248 49

Bone sarcomas are the most common second malignant neoplasms in survivors of a malignant solid tumor in childhood. In contrast to de novo tumors, secondary bone cancers are typically associated with a poor prognosis, reflecting both a preponderance of primary sites that preclude complete resection in the flat bones of the axial skeleton, and the tendency for local invasiveness or distant metastasis. We describe a patient who developed malignant extradural osteosarcoma of the temporal bone 6 years after successful treatment for a malignant cerebellar astrocytoma with surgical resection and local irradiation. A complete resection of the sarcoma was not possible; however, she achieved a biopsy-proven complete response after intensive chemotherapy with ifosfamide, followed by cisplatin and doxorubicin. At age 13, she remains free of recurrence 3 years after completing all therapy. In view of the rarity of prolonged disease control after incomplete resection for osteosarcoma, this report suggests the value of intensive combination chemotherapy in achieving a durable unmaintained remission in our patient.
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PMID:Successful treatment of a radiation-associated extradural osteosarcoma with chemotherapy in an adolescent girl. 258 66

The determination and comparison of genotypic combinations at genomic loci in constitutional and tumor tissues from patients with various types of cancer have defined the chromosomal locations of loci in which recessive mutations play a role in disease development. The predisposing nature of some of these mutant alleles is exemplified by studies of retinoblastoma and osteogenic sarcoma, two clinically associated diseases that share a pathogenetically causal predisposition mapping to 13q14. Genomic alteration of chromosome 10 is apparent in glioblastomas and mixed tumors of glioblastoma/astrocytoma grade III but not in homogeneous astrocytoma grades II or III; this suggests the definition of a locus involved in tumor progression and, perhaps, an approach to molecular genetic staging of tumors.
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PMID:Loss of heterozygosity in stages of malignancy. 266 35

The determination and comparison of genotypic combinations at genomic loci in normal and tumour tissues from patients with various types of cancer have defined the chromosomal locations of loci at which recessive mutations play a role in disease. The predisposing nature of some of these mutant alleles is exemplified in studies of retinoblastoma and osteogenic sarcoma. These two clinically associated diseases share a pathogenetically causal predisposition that maps to chromosome position 13q14. A similar mechanism at 11p15.5 is involved in the development of the embryonal variant of rhabdomyo-sarcoma, Wilms' tumour and hepatoblastoma. Finally, genomic alteration of chromosome 10 is apparent in glioblastomas and mixed tumours of glioblastoma/astrocytoma grade III but not in homogenous astrocytoma grades II or III, suggesting the definition of a locus involved in tumour progression and, perhaps, an approach to molecular genetic staging of tumours.
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PMID:Loss of genetic information in cancer. 274 36

A multicentre registry of children who had been successfully removed from therapy for some common childhood cancers (Hodgkin's disease, non-Hodgkin's lymphoma, neuroblastoma, nephroblastoma, acute lymphatic leukaemia and other leukaemias) was established in Italy in 1981. The present study describes mortality and occurrence of second primary malignancies (SPMs) among 1467 children who were alive when the registry was established. Follow-up ended on December 31, 1983 for mortality and 1 year later for the occurrence of SPMs. Sixty-seven deaths were recorded, 11 of which were due to causes other than progression of the original disease. Eleven incident SPMs were identified (i.e. 3 acute myeloid leukaemias, 3 thyroid carcinomas, 1 bilateral breast carcinoma, 1 liver malignant mesenchymoma, 1 astrocytoma, 1 chondrosarcoma and 1 osteosarcoma) corresponding to an incidence rate of 2.1/1000 patient-years at risk. Anecdotal reports were collected regarding 2 further SPMs (a thyroid carcinoma and a myeloid leukaemia) as well as several benign tumours, including 2 mammary fibroadenomas.
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PMID:Late deaths and second primary malignancies among long-term survivors of childhood cancer: an Italian multicentre study. 365 74

In a tri-institutional, retrospective study with long-term follow-up, forty-four patients who had multiple enchondromas were identified. Thirty-seven patients did not have hemangiomas (Ollier disease) and seven did (Maffucci syndrome). Of the thirty-seven patients who had Ollier disease, a low-grade chondrosarcoma developed in four; an astrocytoma, in one; and a granulosa-cell ovarian tumor, in one. In four of the seven patients who had Maffucci syndrome, there were six low-grade chondrosarcomas, one high-grade osteosarcoma, one pancreatic adenocarcinoma, one biliary adenocarcinoma, and one astrocytoma. None of the patients in either group died of the skeletal sarcoma, but four of five patients who had a non-skeletal malignant lesion died. From life-table analyses of these patients, we estimated that the incidence of secondary chondrosarcoma in patients who have Ollier disease is about 25 per cent at the age of forty years, and that malignant degeneration is almost a certainty in patients who have Maffucci syndrome. We concluded that periodic surveillance of the brain and abdomen for occult malignant lesions is indicated in patients who have enchondromatosis.
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PMID:The malignant potential of enchondromatosis. 380 90


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