1/17. Antithrombin treatment of severe hepatic veno-occlusive disease in children with cancer.hepatic veno-occlusive disease (VOD) is a well-known complication of chemotherapy in wilms tumor patients, particularly young children. Although this complication resolves uneventfully in most patients, fatal cases have been reported. Severe VOD after transplantation has a high mortality rate ranging from 45% to 98%. New hemostatic therapeutic strategies have significantly improved the prognosis of VOD. Chemotherapy-related VOD in wilms tumor usually has a good prognosis. We describe two patients with wilms tumor and one with acute lymphoblastic leukemia, who developed severe veno-occlusive disease of the liver according to the baltimore criteria while undergoing chemotherapy; the symptoms were hepatomegaly, ascites, hyperbilirubinemia, weight gain and, in one patient, short-term lethargy. Elevated LDH levels of 872 to 12,000 U/l were observed in our patients. All patients had thrombocytopenia between 29,000 and 40,000/microl and decreased antithrombin (AT) and protein c levels; two patients had gastrointestinal bleeding. All patients developed a coagulopathy because of severe hepatic dysfunction. Two patients received low-dose heparin at the onset of VOD. The thrombolytic therapy was rapidly changed to AT supplementation (20-80 IU/kg bw 2x per day) without heparin when thrombocytes were very low or gastrointestinal bleeding occurred. Resolution of VOD was observed in all patients receiving AT alone. The chemotherapy was discontinued in a patient with accidental actinomycin D overdosage in view of the severity of symptoms. The remaining two patients received chemotherapy according to the therapy protocol after restitution. All patients survived without sequelae with a median follow-up of 28 months (range 8-48 months). CONCLUSION: hepatic veno-occlusive disease is a rare but increasingly recognized complication in pediatric cancer patients receiving conventional chemotherapy. AT supplementation constitutes a good alternative treatment of severe VOD in comparison with other thrombolytic therapies, particularly in patients at high risk of bleeding.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
2/17. Presentation and revascularization outcomes in patients with radiation-induced renal artery stenosis.This study analyzed the initial presentation and revascularization outcomes of patients with radiation-induced renal artery stenosis, a rare complication of therapeutic irradiation. Of 11 patients with renal artery stenosis after irradiation, 7 patients fulfilled the following criteria: normotension before irradiation, radiation dose greater than 25 grays delivered to the renal arteries, associated perirenal radiation-induced lesions, and absence of arterial disease outside the radiation field. The median age at irradiation was 30 years, and the median local irradiation dose was 40 grays. The median time from irradiation to referral was 13 years. All patients were hypertensive at referral, with a median blood pressure (BP) of 171/102 mm Hg and median treatment score of two. The median glomerular filtration rate was 67 mL/min. Two patients had bilateral stenoses and 1 patient had stenosis affecting a single kidney. Stenoses were proximal in 6 patients and truncal in 1 patient, and all had the appearance of atherosclerotic stenosis. Percutaneous transluminal renal artery angioplasty (PTRA) was successful in 5 patients, but required multiple insufflations. PTRA failed in 1 patient, who subsequently underwent an aortorenal bypass. After a median follow-up of 36 months, 2 patients had died of noncardiovascular causes and 4 patients remained hypertensive, with a median BP of 136/85 mm Hg and median treatment score of two. No restenosis occurred, but aneurysms developed at the site of angioplasty in 1 patient. If hypertension occurs even decades after irradiation, a radiation-induced renal artery stenosis should be sought in patients who have undergone abdominal irradiation.- - - - - - - - - - ranking = 242.80996199625keywords = radiation-induced (Clic here for more details about this article) |
3/17. Potential role of hematopoietic stem cell transplantation in children with secondary acute lymphocytic leukemia.Secondary acute lymphocytic leukemias (ALL) are uncommon events in the pediatric patient population. There are few detailed reports on the laboratory characteristics and clinical course of patients with secondary lymphocytic leukemia. Historically, these patients have had a poor outcome. We report two patients treated at one institution who developed treatment-related secondary ALL. Both patients underwent hematopoietic stem cell transplantation, one with a compatible unrelated donor cord blood unit and one with an HLA-matched sibling donor bone marrow. One of the two patients survives disease-free 3 years after transplantation.- - - - - - - - - - ranking = 6keywords = leukemia (Clic here for more details about this article) |
4/17. radiation arteritis following treatment for Wilms' tumor: an unusual case of weight loss.Wilms' tumor is the most common pediatric primary renal cancer and is highly responsive to surgery and chemotherapy. The role of radiotherapy has evolved in the last three decades from the use of stepwise incremental doses in all patients to the current concept of added radiotherapy in advanced cases or in those with unfavorable pathology. To the authors' knowledge, this is the first case of a young woman with prior history of Wilms' tumor and significant weight loss due to radiation arteritis involving the abdominal vasculature. A 31-year-old woman presented with a history of weight loss and severe malnutrition. An angiogram revealed that the aorta was occluded below the renal artery. The celiac artery and the superior mesenteric artery were occluded at the origin, and large intercostal collaterals reconstituted the pelvic circulation. She was initially treated with total parenteral nutrition (TPN) and underwent an infrarenal aortic bypass surgery. Histopathology of the aorta revealed intimal fibrocalcific thickening and changes consistent with radiation-induced arteritis. Later, she was readmitted with progressive hepatic insufficiency. Despite intensive medical therapy, she died of multi-organ failure.- - - - - - - - - - ranking = 34.687137428036keywords = radiation-induced (Clic here for more details about this article) |
5/17. Acute lymphoblastic leukemia occurring as a second malignant neoplasm in childhood: report of three cases and review of the literature.PURPOSE: The long-term effects of childhood cancer and its therapy are a problem of increasing concern. One of the most important of these late effects is the development of second malignant neoplasms (SMNs), which occur in approximately 8% of children within 20 years of diagnosis of a malignancy. These secondary cancers may result (individually or in combination) from increased genetic susceptibility, the mutagenic effects of chemotherapy and/or radiation therapy, or chance. Whereas the development of acute nonlymphocytic leukemia (ANLL) as an SMN is a well-recognized phenomenon, acute lymphoblastic leukemia (ALL) has been infrequently described as an SMN in either adults or children. patients AND methods: We report three patients treated at our institution in whom ALL developed as an SMN after treatment for neuroblastoma, Wilms' tumor, and Hodgkin's disease. These cases prompted us to review the published literature for cases of secondary ALL in childhood. patients whose initial malignancy was diagnosed at age less than 16 years were classified as pediatric patients. SMNs were defined as cancers of clearly distinct histologic type occurring 6 or more months after diagnosis of the first malignant neoplasm. RESULTS: Including the three index cases, a total of 18 children with secondary ALL are reviewed, and the clinical features are discussed and compared with those of secondary ANLL. CONCLUSIONS: This review summarizes the published case histories of secondary ALL. The data suggest that ALL represents approximately 5% to 10% of the cases of acute leukemia that arise as SMNs in both adults and children.- - - - - - - - - - ranking = 7keywords = leukemia (Clic here for more details about this article) |
6/17. Combined computed tomography-guided radiofrequency ablation and brachytherapy in a child with multiple recurrences of Wilms' tumor.BACKGROUND: Treatment of relapsed Wilms' tumor remains a challenge. We describe a case of an 11-year-old girl with multiply relapsed Wilms' tumor in whom combined percutaneous computed tomography (CT)-guided radiofrequency ablation and brachytherapy directed at a retroperitoneal tumor mass resulted in pain palliation and local tumor control. OBSERVATIONS: Over the course of few weeks, her requirement for narcotic pain medications dramatically decreased. A contrast-enhanced CT scan obtained at 8 months after the procedure showed no evidence for local tumor recurrence. However, she subsequently developed myelodysplasia with evolution into leukemia, presumably secondary to chemotherapy, and died 9.5 months after the procedure. CONCLUSION: Combined CT-guided radiofrequency ablation and brachytherapy is a promising new minimally invasive palliative treatment of recurrent Wilms' tumor.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
7/17. Secondary B-cell acute lymphoblastic leukemia following Wilms' tumor: clinical and in vitro chemosensitivity studies.We report the clinical features and in vitro chemosensitivity assay findings of a 13-year-old girl who developed secondary B-cell acute lymphoblastic leukemia (ALL) 7 years after a diagnosis of Wilms' tumor. The patient was treated using the berlin - Frankfurt - Muenster (BFM) ALL chemotherapy protocol with poor response to initial therapy before succumbing to sepsis. An in vitro chemosensitivity assay on her peripheral blood lymphoblasts was performed while she was undergoing induction therapy and showed a high level of resistance to drugs commonly used for ALL therapy, e.g. steroids, anthracyclines, vincristine and L-asparaginase. The mechanism of chemoresistance was not elicited, but was probably not related to p-glycoprotein (P-gp) over-expression. We believe that the in vitro chemosensitivity assay is a good indicator of cellular response to chemotherapy and may provide reliable information for the basis of the selection of drugs to be used for the treatment of similarly rare patients rather than relying on "standard" protocols.- - - - - - - - - - ranking = 5keywords = leukemia (Clic here for more details about this article) |
8/17. Bilateral nephroblastomatosis.Bilateral nephromegaly with a histological picture of diffuse Wilms' tumor or so-called bilateral nephroblastomatosis is a rare condition. We present an additional case documented by urography. To our knowledge, this is the first report of diffuse nephroblastomatosis recognized at 2 hours of age. A few cases are reported but urographic documentation is available in only 4 of those and in our own. The review of the urographic findings reveals features which seem seem to be nearly pathognomonic of this condition. Urographic appearance similar to that of adult type polycystic disease without the typical radiolucent cysts, should raise the suspicion of a diffuse bilateral process in the kidneys. Exclusion of other infiltrative diseases, like leukemia and glycogen storage disease, should lead to radiologic diagnosis of nephroblastomatosis. Renal biopsy is then indicated.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
9/17. A highly polymorphic locus cloned from the breakpoint of a chromosome 11p13 deletion associated with the wagr syndrome.Children with constitutional deletions of chromosome 11p13 suffer from aniridia, genitourinary malformations, and mental retardation and are predisposed to develop bilateral wilms tumor (the wagr syndrome). The critical region for these defects has been narrowed to a segment of band 11p13 between the catalase and the beta-follicle-stimulating hormone genes. In this report, we have cloned the endpoints from a WAGR patient whose large cytogenetic deletion, del(11)(p14.3::p13), does not include the catalase gene. The deletion was characterized using dna polymorphisms and found to originate in the paternally derived chromosome 11. The distal endpoint was identified as a rearrangement of locus D11S21 in conventional Southern blots of the patient's genomic dna, but was not detected in leukocyte dna from either parent or in sperm dna from the father. The proximal endpoint was isolated by cloning the junction fragment and was mapped in relation to other markers and breakpoints. It defines a new locus in 11p13-delta J, which is close to the wilms tumor gene and the breakpoint cluster region (TCL2) of the frequent t(11;14)(p13;q11) translocation in acute T-cell leukemia. An unusual concentration of base pair substitutions was discovered at delta J, in which 9 of 44 restriction sites tested (greater than 20%) vary in the population. This property makes delta J one of the most polymorphic loci on chromosome 11 and may reflect an underlying instability that contributed to the original mutation. The breakpoint extends the genetic map of this region and provides a useful marker for linkage studies and the analysis of allelic segregation in tumor cells.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
10/17. hypercalcemia in association with mesoblastic nephroma: report of a case and review of the literature.hypercalcemia, often associated with certain types of adult tumors, has also been described in pediatric neoplasms. In childhood, the more common associations include lymphoma, leukemia, rhabdomyosarcoma and rarely neuroblastoma. However, recently, several infants with hypercalcemia were described having renal tumors without bone metastases. The following is a case report of a 2-month-old infant who presented with severe hypercalcemia and a large right-sided abdominal mass, which at surgery was diagnosed as a cellular mesoblastic nephroma.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
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