Medical and pediatric oncology最新文献

Growth hormone (GH) is a peptide hormone secreted from the pituitary gland under the control of the hypothalamus. It has a many actions in the body, including regulating a number of metabolic pathways. Some, but not all, of its effects are mediated through insulin-like growth factor-I (IGF-I). Both GH and IGF-I play significant roles in the regulation of growth and bone metabolism and hence are regulators of bone mass. Bone mass increases steadily through childhood, peaking in the mid 20s. Subsequently, there is a slow decline that accelerates in late life. During childhood, the accumulation in bone mass is a combination of bone growth and bone remodeling. Bone remodeling is the process of new bone formation by osteoblasts and bone resorption by osteoclasts. GH directly and through IGF-I stimulates osteoblast proliferation and activity, promoting bone formation. It also stimulates osteoclast differentiation and activity, promoting bone resorption. The result is an increase in the overall rate of bone remodeling, with a net effect of bone accumulation. The absence of GH results in a reduced rate of bone remodeling and a gradual loss of bone mineral density. Bone growth primarily occurs at the epiphyseal growth plates and is the result of the proliferation and differentiation of chondrocytes. GH has direct effects on these chondrocytes, but primarily regulates this function through IGF-I, which stimulates the proliferation of and matrix production by these cells. GH deficiency severely limits bone growth and hence the accumulation of bone mass. GH deficiency is not an uncommon complication in oncology and has long-term effects on bone health.

Background: Overt extraocular retinoblastoma is common in developing countries and little information about its treatment is available. The aim of this study is to report our experience in the treatment of these cases using a uniform approach.

Procedure: Patients with overt extraocular retinoblastoma including orbital extension, preauricular lymph node invasion and/or metastatic disease on diagnosis or after extraocular relapse admitted to the Hospital JP Garrahan from August 1987 to December 2000 were retrospectively reviewed. Patients were treated according to two different protocols (1987-1993 and 1994-2000). Treatment included: neoadjuvant combination chemotherapy followed by limited surgery in case of orbital extension (enucleation or resection of residual orbital mass) and adjuvant chemotherapy and radiotherapy. Chemotherapy included cyclophosphamide, vincristine, etoposide, doxorubicin (in protocol 87), idarubicin (in protocol 94), cisplatin (in protocol 87), and carboplatin (in protocol 94).

Results: Forty-one patients were included. Fifteen of them had orbital or preauricular disease and had a 5-year event-free survival (pEFS) of 84%. Twenty-six had distant metastatic disease and non survived 5-years. One patient died of toxicity and one died in complete remission. One patient had a secondary leukemia. The remaining adverse events included CNS and/or systemic relapse.

Conclusions: This treatment strategy was highly efficacious for patients with orbital and/or lymph node extension. Orbital exenteration is not necessary for these patients. Those patients with distant metastatic or CNS disease were not curable with this approach.

Background: Thoracoscopic and laparoscopic techniques play a major role in pediatric surgery. However, minimally invasive surgery (MIS) has not yet established itself in pediatric surgical oncology. The authors present a prospective study investigating the role of MIS in children with cancer.

Procedure: All children with abdominal or thoracic tumors requiring surgery were registered between September, 2000 and February, 2002. Decisions regarding procedures and approaches-conventional or minimally invasive-were made by the interdisciplinary team. Data on diagnoses, surgical procedures, complications, and conversion rates were registered prospectively.

Results: Seventy-four patients received 78 operations, 21 (26.9%) of the 78 operations were minimally invasive. Seven of 16 tumor biopsies (43.8%) and 9 of 57 tumor resections (15.8%) were performed using MIS, which was also exclusively used for diagnostic interventions. Conversions to standard techniques only occurred in 5 of 9 tumor resections. No major complications were encountered in the MIS group.

Conclusions: MIS was practical in every fourth patient in our experience so far. It proved to be an excellent approach in diagnostic interventions and tumor biopsies, whereas efficacy is limited in tumor resections. Further factors (tumor recurrence, trocar site recurrence, tumor growth, and dissemination after CO(2) insufflation) have to be evaluated. Our data encourage the continuation of the study.

Background: To determine the presenting clinicopathologic features and treatment outcomes of 11 ataxia-telangiectasia (A-T) patients with Hodgkin disease.

Procedure: We reviewed the charts of 412 A-T patients to ascertain cases of Hodgkin disease. The data analyzed included date of diagnosis, duration of symptoms, chest radiographic findings, stage and histology, therapy, and outcome.

Results: The six male and five female patients had a median age at diagnosis of 12.2 years. Eight patients presented with fever, cough, and/or cervical lymphadenopathy with a median duration of symptoms of 3 months. Five patients had abnormal chest radiographic findings a median of 3 months prior to diagnosis and were treated with antibiotics for presumed pneumonia. Mediastinal and hilar adenopathy in addition to bilateral infiltrates were present. Histopathology reports were available for nine patients. Three had nodular sclerosing and two each had lymphocyte depleted, mixed cellularity, and not otherwise specified histology. Eight patients had stage IV disease, one had stage III, and in two the staging was not documented. Six patients received reduced-dose chemotherapy, two received radiation therapy, two did not receive therapy, and in one the treatment was not documented. In no patient did the Hodgkin disease remit and all died with a median survival of 3 months. Eight died of pneumonia and three of multiple system organ failure.

Conclusions: A-T patients with Hodgkin disease have markedly reduced survival compared to Hodgkin disease in the general population. Their poor outcomes may be due to advanced Hodgkin disease, failure to recognize coincident chronic lung disease, and the use of non-standard treatment regimens.