Información sobre riesgo, prevención, detección, síntomas, diagnosis, tratamiento y apoyo para el cáncer.
Información sobre el tratamiento del cáncer incluyendo quirúrgica, quimioterapia, radioterapia, estudios clínicos, terapia con protón, medicina complementaria avanzadas.
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Maneras que los pacientes de cáncer y las personas que le cuidan puedan enfrentar el cáncer, los efectos secundarios, nutrición, cuestiones en general sobre el apoyo para el cáncer, duelo/decisiones sobre el termino de vida, y experiencias compartidas por sobrevivientes.
National Cancer Institute
Ultima Vez Modificado: 26 de noviembre del 2012
Fortunately, cancer in children and adolescents is rare, although the overall incidence of childhood cancer has been slowly increasing since 1975. 1 Children and adolescents with cancer should be referred to medical centers that have a multidisciplinary team of cancer specialists with experience treating the cancers that occur during childhood and adolescence. This multidisciplinary team approach incorporates the skills of the primary care physician, an orthopedic surgeon experienced in bone tumors, a pathologist, radiation oncologists, pediatric oncologists, rehabilitation specialists, pediatric nurse specialists, social workers, and others to ensure that children receive treatment, supportive care, and rehabilitation that will achieve optimal survival and quality of life. (Refer to the PDQ® summaries on Supportive and Palliative Care for specific information about supportive care for children and adolescents with cancer.)
Guidelines for pediatric cancer centers and their role in the treatment of pediatric patients with cancer have been outlined by the American Academy of Pediatrics. 2 At these pediatric cancer centers, clinical trials are available for most types of cancer that occur in children and adolescents, and the opportunity to participate in these trials is offered to most patients/families. Clinical trials for children and adolescents with cancer are generally designed to compare potentially better therapy with therapy that is currently accepted as standard. Most of the progress made in identifying curative therapies for childhood cancers has been achieved through clinical trials. Information about ongoing clinical trials is available from the NCI Web site.
Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2002, childhood cancer mortality has decreased by more than 50%. For osteosarcoma, the 5-year survival rate has increased over the same time from 40% to 67% in children and adolescents. 1 Childhood and adolescent cancer survivors require close follow-up because cancer therapy side effects may persist or develop months or years after treatment. (Refer to the PDQ® summary on Late Effects of Treatment for Childhood Cancer for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.)
Osteosarcoma occurs predominantly in adolescents and young adults. Review of data from the Surveillance, Epidemiology and End Results program of the NCI resulted in an estimate of 4.4 per million new cases of osteosarcoma each year in people aged 0 to 24 years. 3 The U.S. Census Bureau estimates that there will be 110 million people in this age range in 2010, resulting in an incidence of roughly 450 cases per year in children and young adults younger than 25 years. Osteosarcoma accounts for approximately 5% of childhood tumors. In children and adolescents, more than 50% of these tumors arise from the long bones around the knee. Osteosarcoma can rarely be observed in soft tissue or visceral organs. There appears to be no difference in presenting symptoms, tumor location, and outcome for younger patients (<12 years) compared with adolescents. 4 5 Two trials conducted in the 1980s were designed to determine if chemotherapy altered the natural history of osteosarcoma following surgical removal of the primary tumor. The outcome of patients in these trials who were treated with surgical removal of the primary tumor recapitulated the historical experience before 1970; more than half of these patients developed metastases within 6 months of diagnosis, and overall, approximately 90% developed recurrent disease within 2 years of diagnosis. 6 Overall survival for patients treated with surgery alone was statistically inferior. 7 The natural history of osteosarcoma has not changed over time, and fewer than 20% of patients with localized resectable primary tumors treated with surgery alone can be expected to survive free of relapse. 6 8 9
Pretreatment factors that influence outcome include site and size of the primary tumor and presence or absence of clinically detectable metastatic disease. 10 After administration of preoperative chemotherapy, surgical resectability and the degree of tumor necrosis influence outcome. In general, prognostic factors in osteosarcoma have not been helpful in identifying patients who might benefit from treatment intensification or who might require less therapy while maintaining an excellent outcome.
The site of the primary tumor is a significant prognostic factor for patients with localized disease. Among extremity tumors, distal sites have a more favorable prognosis than proximal sites. Axial skeleton primary tumors are associated with the greatest risk of progression and death, primarily related to the inability to achieve a complete surgical resection.
Despite a relatively high rate of inferior necrosis following neoadjuvant chemotherapy, fewer patients with craniofacial primaries develop systemic metastases than do patients with osteosarcoma originating in the extremities. 19 20 21 This low rate of metastasis may be related to the relatively smaller size and higher incidence of lower grade tumors in osteosarcoma of the head and neck. This low rate of metastasis may be related to the relatively smaller size and higher incidence of lower grade tumors in osteosarcoma of the head and neck.
While small series have not shown a benefit from adjuvant chemotherapy for patients with osteosarcoma of the head and neck, one meta-analysis concluded that systemic chemotherapy improves the prognosis for these patients. Another large meta-analysis detected no benefit from chemotherapy for patients with osteosarcoma of the head and neck, but suggested that the incorporation of chemotherapy into treatment of patients with high-grade tumors may improve survival. A retrospective analysis identified a trend toward better survival in patients with high-grade osteosarcoma of the mandible and maxilla who received adjuvant chemotherapy. 18 22
Radiation therapy was found to improve local control, disease-specific survival, and overall survival in a retrospective study of osteosarcoma of the craniofacial bones, which had positive or uncertain margins after surgical resection. 23[[Level of evidence: 3iiALevel of evidence: 3iiA] Radiation-associated craniofacial osteosarcomas are generally high-grade lesions, usually fibroblastic, that tend to recur locally with a high rate of metastasis.] Radiation-associated craniofacial osteosarcomas are generally high-grade lesions, usually fibroblastic, that tend to recur locally with a high rate of metastasis. 24
In the German series, approximately 25% of patients with craniofacial osteosarcoma had osteosarcoma as a second tumor, and in 8 of these 13 patients, osteosarcoma arose following treatment for retinoblastoma. In this series, there was no difference in outcome for primary or secondary craniofacial osteosarcoma. 15
Larger tumors have a worse prognosis than smaller tumors. 10 26 Tumor size has been assessed by the longest single dimension, by the cross-sectional area, or by an estimate of tumor volume; all have correlated with outcome. Serum lactate dehydrogenase (LDH), which also correlates with outcome, is a likely surrogate for tumor volume.
Patients with localized disease have a much better prognosis than those with overt metastatic disease. As many as 20% of patients will have radiographically detectable metastases at diagnosis, with the lung being the most common site. 27 The prognosis for patients with metastatic disease appears to be determined largely by the site(s), the number of metastases, and the surgical resectability of the metastatic disease. 28 29
Patients with multifocal osteosarcoma (defined as multiple bone lesions without a clear primary tumor) have an extremely poor prognosis. 32
Resectability of the tumor is a critical prognostic feature because osteosarcoma is relatively resistant to radiation therapy. Complete resection of the primary tumor and any skip lesions with adequate margins is generally considered essential for cure. A retrospective review of patients with craniofacial osteosarcoma performed by the German-Austrian-Swiss osteosarcoma cooperative group reported that incomplete surgical resection was associated with inferior survival probability. 15[Level of evidence: 3iiB] For patients with axial skeletal primaries who either do not have surgery for their primary tumor or who have surgery resulting in positive margins, radiation therapy may improve survival. 11 35 In a European cooperative study, the size of the margin was not significant. However, having both the biopsy and resection at a center with orthopedic oncology experience conferred a better prognosis. 13
Most treatment protocols for osteosarcoma use an initial period of systemic chemotherapy prior to definitive resection of the primary tumor (or resection of sites of metastases for patients with metastatic disease). The pathologist assesses necrosis in the resected tumor. Patients with at least 90% necrosis in the primary tumor after induction chemotherapy have a better prognosis than those with less necrosis. 26 Patients with less necrosis (<90%) in the primary tumor following initial chemotherapy have a higher rate of recurrence within the first 2 years compared with patients with a more favorable amount of necrosis (90%). 36 Less necrosis should not be interpreted to mean that chemotherapy has been ineffective; cure rates for patients with little or no necrosis following induction chemotherapy are much higher than cure rates for patients who receive no chemotherapy.
Patients with osteosarcoma as a second malignant neoplasm, including those tumors arising in a radiation field, share the same prognosis as patients with de novo osteosarcoma if they are treated aggressively with complete surgical resection and multiagent chemotherapy. 43 44 45 46 Possible prognostic factors identified for patients with conventional localized high-grade osteosarcoma include the age of the patient, LDH level, alkaline phosphatase level, and histologic subtype. 26 47 48 49 50 51 A number of potential prognostic factors have been identified but have not been tested in large numbers of patients. These include the expression of HER2/c-erbB-2 (there are conflicting data concerning the prognostic significance of this human epidermal growth factor); 52 53 54 tumor cell ploidy; specific chromosomal gains or losses; 55 loss of heterozygosity of the RB gene; 56 57 Loss of heterozygosity of the p53 locus; 58 and increased expression of p-glycoprotein. 59 60 A prospective analysis of p-glycoprotein expression determined by immunohistochemistry failed to identify prognostic significance for newly diagnosed patients with osteosarcoma, although earlier studies suggested that overexpression of p-glycoprotein predicted for poor outcome. 61 In a large series, a delay of 21 days or more from the time of definitive surgery to the resumption of chemotherapy was an adverse prognostic factor. 62 Pathologic fracture at diagnosis or during preoperative chemotherapy does not have adverse prognostic significance. 63
Patients with Rothmund-Thomson syndrome have an increased risk of developing osteosarcoma compared with the general population. They also tend to develop osteosarcoma at a younger age. 64 Rothmund-Thomson syndrome, also called poikiloderma congenitale, is a rare autosomal recessive condition attributed to mutations of the RECQL4 helicase gene on 8q24. It is characterized by distinctive skin findings (e.g., atrophy, telangiectasias, pigmentation), sparse hair, cataracts, small stature, skeletal anomalies, and a significantly increased risk for osteosarcoma. There is no adverse prognostic significance for osteosarcoma in conjunction with Rothmund-Thomson syndrome.
|Bloom syndrome||15q26.1||BLM (RecQL3)||DNA helicase|
|Diamond-Blackfan anemia||Ribosomal proteins||Ribosome production|
|Li-Fraumeni syndrome||17p13.1||P53||DNA damage response|
|Paget disease||18q21-qa22||LOH18CR1||IL-1/TNF signaling; RANK signaling pathway|
|Rothmund-Thomson syndrome||8q24.3||RTS (RecQL4)||DNA helicase|
|Werner syndrome||8p12-p11.2||WRN (RecQL2)||DNA helicase; exonuclease activity|
Osteosarcoma is a malignant tumor that is characterized by the direct formation of bone or osteoid tissue by the tumor cells. The World Health Organization's histologic classification 1 of bone tumors separates the osteosarcomas into central (medullary) and surface (peripheral) 2 3 tumors and recognizes a number of subtypes within each group.
The most common pathologic subtype is conventional central osteosarcoma, which is characterized by areas of necrosis, atypical mitoses, and malignant osteoid tissue and/or cartilage. The other subtypes are much less common, each occurring at a frequency of less than 5%. Telangiectatic osteosarcoma may be confused radiographically with an aneurysmal bone cyst or giant cell tumor. This variant should be approached as a conventional osteosarcoma. 4 5
Malignant fibrous histiocytoma (MFH) of bone is treated according to osteosarcoma treatment protocols. 13 MFH should be distinguished from angiomatoid fibrous histiocytoma, a low-grade tumor that is usually noninvasive, small, and associated with an excellent outcome with surgery alone. 14 One study suggests similar event-free survival rates for MFH and osteosarcoma. 13
Extraosseous osteosarcoma is a malignant mesenchymal neoplasm without direct attachment to the skeletal system. Previously, treatment for extraosseous osteosarcoma followed soft tissue sarcoma guidelines, 15 though a retrospective analysis of the German Cooperative Osteosarcoma Study identified a favorable outcome for extraosseous osteosarcoma treated with surgery and conventional osteosarcoma therapy. 16
Historically, the Enneking staging system for skeletal malignancies was widely used. 1 This system inferred the aggressiveness of the primary tumor by the descriptors intracompartmental or extracompartmental. The American Joint Committee on Cancer (AJCC) staging system for malignant bone tumors has updated this staging system, substituting compartmentalization with size (see Table 2). 2 The AJCC classification is as follows:
|Stage||Tumor Grade||Tumor Size|
|III||Any tumor grade, skip metastasesb|
|IV||Any tumor grade, any tumor size, distant metastases|
For the purposes of treatment, there are only two stages of high-grade osteosarcoma. Patients without clinically detectable metastatic disease are considered to have localized osteosarcoma. Patients in whom it is possible to detect any site of metastasis at the time of initial presentation by routine clinical studies are considered to have metastatic osteosarcoma.
For patients with confirmed osteosarcoma, in addition to plain x-rays of the primary site that include a single plane view of the entire affected extremity to assess for skip metastasis, pretreatment staging studies should include magnetic resonance imaging (MRI) and/or computed tomography (CT) scan of the primary site. Additional pretreatment staging studies should include bone scan, postero-anterior and lateral chest x-ray, and CT scan of the chest. Positron emission tomography (PET) using fluorine-18-fluorodeoxyglucose is an optional staging modality. 3
Localized tumors are limited to the bone of origin. Patients with skip lesions confined to the bone which includes the primary tumor should be considered to have localized disease if the skip lesions can be included in the planned surgical resection. 4 Approximately one-half of the tumors arise in the femur; of these, 80% are in the distal femur. Other primary sites in descending order of frequency are the proximal tibia, proximal humerus, pelvis, jaw, fibula, and ribs. 5 Compared with osteosarcoma of the appendicular skeleton, osteosarcoma of the head and neck is more likely to be low grade 6 and to arise in older patients.
Radiologic evidence of metastatic tumor deposits in the lungs, other bones, or other distant sites is found in approximately 20% of patients at diagnosis, with 85% to 90% of metastatic disease presenting in the lungs. The second most common site of metastasis is another bone. 7 Metastasis to other bones may be solitary or multiple. The syndrome of multifocal osteosarcoma refers to a presentation with multiple foci of osteosarcoma without a clear primary tumor, often with symmetrical metaphyseal involvement. Multifocal osteosarcoma has an extremely grave prognosis. 5
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