Heritable Fraction of Unilateral Wilms Tumor

PEDIATRICS ◽  
1988 ◽  
Vol 81 (1) ◽  
pp. 147-149
Author(s):  
Frederick P. Li ◽  
Wick R. Williams ◽  
Kathreen Gimbrere ◽  
Francoise Flamant ◽  
Daniel M. Green ◽  
...  
Keyword(s):  
Genetic Counseling ◽  
Maximum Likelihood ◽  
Upper Bound ◽  
Maximum Likelihood Estimate ◽  
Confidence Limit ◽  
Wilms Tumor ◽  
Upper Confidence Limit ◽  
Familial Form ◽  
Long Term Survivors

Heritability of the unilateral-sporadic (non-familial) form of Wilms tumor was examined in the offspring of 96 long-term survivors of the neoplasm. No Wilms tumor has developed in any of the 179 offspring of these patients. The maximum likelihood estimate of a hereditary Wilms tumor in our patients is zero and the corresponding 95% upper confidence limit ranges between 0.06 and 0.11, depending on penetrance. Among their offspring, the 95% upper bound of the risk of Wilms tumor is 0.02. These figures can be applied in genetic counseling of other survivors of unilateral-sporadic Wilms tumor.

Hypertension in long-term survivors of childhood renal cancers.

1989 ◽  
Vol 7 (7) ◽  
pp. 912-915 ◽  
Author(s):  
A F Kantor ◽  
F P Li ◽  
A J Janov ◽  
N J Tarbell ◽  
S E Sallan
Keyword(s):  
Wilms Tumor ◽  
Late Complication ◽  
Renal Surgery ◽  
Borderline Hypertension ◽  
Case Comparison ◽  
History Of ◽  
Family History Of Hypertension ◽  
Male Patients ◽  
Long Term Survivors

The prevalence of hypertension was investigated in 119 adults who have survived for up to 53 years following the diagnosis of renal cancer in childhood (Wilms' tumor, 116 patients; renal carcinoma, three patients). Twenty-four (20%) have developed definite or borderline hypertension, as compared with 18.1 cases expected based on US population rates (relative risk [RR], 1.3; 95% confidence interval [CI], 0.9 to 2.0; P = .20). This nonsignificant excess is due to the heightened prevalence of definite hypertension among one subgroup of male patients. The findings are not explained by cigarette smoking, obesity, age, and stage at diagnosis of Wilms' tumor, or family history of hypertension. A case-comparison analysis within the cohort showed no consistent hypertensive effect associated with radiation therapy dose, radiotherapy concurrent with dactinomycin chemotherapy, or extent of renal surgery. Hypertension is not a common late complication of Wilms' tumor in our patients.

Long-term survivors of leukemia treated in infancy: factors associated with neuropsychologic status.

1992 ◽  
Vol 10 (7) ◽  
pp. 1095-1102 ◽  
Author(s):  
R K Mulhern ◽  
E Kovnar ◽  
J Langston ◽  
M Carter ◽  
D Fairclough ◽  
...  
Keyword(s):  
Young Children ◽  
Wilms Tumor ◽  
Lymphoblastic Leukemia ◽  
Memory Performance ◽  
Auditory Memory ◽  
Very Young Children ◽  
Cns Relapse ◽  
Neuropsychologic Testing ◽  
Long Term Survivors

PURPOSE Because of concerns about late toxicities of treatment among infants diagnosed with acute lymphoblastic leukemia (ALL), and especially the effects of cranial radiation therapy (CRT), we compared the functional and neuropsychologic status of 26 long-term survivors of ALL who were diagnosed in the first 24 months of life versus 26 children who were treated previously for Wilms' tumor. PATIENTS AND METHODS Of the children with ALL, CNS prophylaxis included no CRT in six, 18 Gy CRT in five, 20 Gy CRT in seven, and 24 Gy CRT in five. Three additional children experienced CNS relapse and received total CRT doses of 24, 40, and 44 Gy. All children received neuropsychologic testing; children with ALL also participated in diagnostic imaging studies. RESULTS As a group, the children who were treated for ALL did not differ significantly from those who were treated for Wilms' tumor on objective measures of global functional status. However, children treated for ALL had a significantly lower mean intelligence quotient (IQ) (87 v 96), poorer performance on four of six measures of visual and auditory memory, lower achievement with regard to arithmetic skills, and a greater frequency of special educational intervention than those who were treated for Wilms' tumor. IQ and auditory memory performance in the ALL group was correlated inversely with time since the completion of therapy and total CRT dose. CONCLUSIONS These results reinforce the contemporary trend of prophylactic CRT omission in very young children except for those who are at risk for CNS relapse. For infants and very young children who require CRT, evidence is presented that supports the approach for the delay of CRT until the child is older.

Cancer in Neonates: The Experience at The Children's Hospital of Philadelphia

PEDIATRICS ◽  
1982 ◽  
Vol 70 (3) ◽  
pp. 409-413
Author(s):  
Gordon B. Gale ◽  
Giulio J. D'Angio ◽  
Antonia Uri ◽  
Jane Chatten ◽  
C. Everett Koop
Keyword(s):  
Cancer Research ◽  
Radiation Therapy ◽  
Wilms Tumor ◽  
Surgical Excision ◽  
Careful Monitoring ◽  
Actuarial Survival ◽  
Parotid Carcinoma ◽  
Complete Surgical Excision ◽  
Long Term Survivors

Among 22 neonates treated at the Children's Cancer Research Center of Philadelphia, 11 had neuroblastoma, which in two cases was widely metastatic. There were three infants with teratoma, three with sarcoma, three with leukemia, one with Wilms' tumor, and one with parotid carcinoma. Nine of eleven patients (82%) are long-term survivors following complete surgical excision of tumor, whereas only one of eight (13%) has survived following incomplete surgical excision. All three neonates with leukemia died. The overall two-year actuarial survival is 45% (10/22). The problems associated with treating neonates with chemotherapy, radiation therapy, or both are especially difficult because of the immaturity of the organs and structures. Surgical excision alone has been the treatment of choice for solid tumors. Chemotherapy or radiation therapy, when indicated, require careful monitoring for both acute toxicities and potential long-term morbidities.

Renal functional reserve in long-term survivors of unilateral Wilms tumor

1991 ◽  
Vol 118 (5) ◽  
pp. 698-702 ◽  
Author(s):  
Donna M. Bhisitkul ◽  
Elaine R. Morgan ◽  
Michele A. Vozar ◽  
Craig B. Langman
Keyword(s):  
Wilms Tumor ◽  
Functional Reserve ◽  
Renal Functional Reserve ◽  
Long Term Survivors

Reduced therapy for Wilms' tumor: analysis of treatment results from a single institution.

1988 ◽  
Vol 6 (10) ◽  
pp. 1630-1635 ◽  
Author(s):  
J A Wilimas ◽  
E C Douglass ◽  
S Lewis ◽  
D Fairclough ◽  
G Fullen ◽  
...  
Keyword(s):  
Wilms Tumor ◽  
Survival Rates ◽  
Stage Iv ◽  
Single Institution ◽  
Relapse Free Survival ◽  
Free Survival ◽  
Favorable Histology ◽  
Long Term Survivors ◽  
Extent Of Disease

From 1968 to 1986, 192 patients from 0 to 17 years of age were enrolled in three consecutive protocol-controlled studies of Wilms' tumor at St Jude Children's Research Hospital. Tumors were completely excised at the time of diagnosis whenever possible, and patients were subsequently treated with chemotherapy and radiotherapy according to the initial extent of disease. All patients received dactinomycin and vincristine, with doxorubicin added to the regimens in studies 2 and 3. Chemotherapy was extended to 18 months in study 2 (n = 53), but was limited to 12 months for most patients in study 3 (n = 107). In the third study, radiation was eliminated altogether for patients with stage I or II tumors and was reduced to 12 Gy for those with more advanced disease. Intensification of chemotherapy in study 2 improved the 5-year relapse-free survival rate over that in study 1 (82% v 52%), but the accompanying increase in toxicity was considered unacceptable. Comparison of 2-year relapse-free survival rates in studies 2 and 3 indicated that the reduction of therapy in the latter trial did not jeopardize disease control: 88% v 86% for patients with stage II or III disease, favorable histology; 75% v 57% for the same stages, unfavorable histology; and 57% v 61% for stage IV patients. At least 80% of all patients enrolled in study 3 will be long-term survivors. We conclude that rescheduling of effective antitumor drugs and eliminating or reducing radiotherapy are feasible alternatives in the treatment of Wilms' tumor with favorable histologic features.

scholarly journals Neurocognitive outcomes in long-term survivors of Wilms tumor: a report from the St. Jude Lifetime Cohort

2019 ◽  
Vol 13 (4) ◽  
pp. 570-579
Author(s):  
Ingrid Tonning Olsson ◽  
Tara M. Brinkman ◽  
Geehong Hyun ◽  
Pia Banerjee ◽  
Daniel A. Mulrooney ◽  
...  
Keyword(s):  
Wilms Tumor ◽  
Neurocognitive Outcomes ◽  
Long Term Survivors

scholarly journals Randomized Noninferiority Trial of Telephone vs In-Person Genetic Counseling for Hereditary Breast and Ovarian Cancer: A 12-Month Follow-Up

2017 ◽  
Vol 1 (1) ◽  
Author(s):  
Mary K. Interrante ◽  
Hannah Segal ◽  
Beth N. Peshkin ◽  
Heiddis B. Valdimarsdottir ◽  
Rachel Nusbaum ◽  
...  
Keyword(s):  
Genetic Counseling ◽  
Genetic Testing ◽  
Lower Bound ◽  
Upper Bound ◽  
Telephone Counseling ◽  
Noninferiority Trial ◽  
Patient Reported ◽  
Risk Reducing

Abstract Background Telephone delivery of genetic counseling is an alternative to in-person genetic counseling because it may extend the reach of genetic counseling. Previous reports have established the noninferiority of telephone counseling on short-term psychosocial and decision-making outcomes. Here we examine the long-term impact of telephone counseling (TC) vs in-person counseling (usual care [UC]). Methods We recruited high-risk women for a noninferiority trial comparing TC with UC. Of 1057 potentially eligible women, 669 were randomly assigned to TC (n = 335) or UC (n = 334), and 512 completed the 12-month follow-up. Primary outcomes were patient-reported satisfaction with genetic testing decision, distress, and quality of life. Secondary outcomes were uptake of cancer risk management strategies. Results TC was noninferior to UC on all primary outcomes. Satisfaction with decision (d = 0.13, lower bound of 97.5% confidence interval [CI] = –0.34) did not cross its one-point noninferiority limit, cancer-specific distress (d = –2.10, upper bound of 97.5% CI = –0.07) did not cross its four-point noninferiority limit, and genetic testing distress (d = –0.27, upper bound of 97.5% CI = 1.46), physical function (d = 0.44, lower bound of 97.5% CI = –0.91) and mental function (d = –0.04, lower bound of 97.5% CI = –1.44) did not cross their 2.5-point noninferiority limit. Bivariate analyses showed no differences in risk-reducing mastectomy or oophorectomy across groups; however, when combined, TC had significantly more risk-reducing surgeries than UC (17.8% vs 10.5%; χ2= 4.43, P = .04). Conclusions Findings support telephone delivery of genetic counseling to extend the accessibility of this service without long-term adverse outcomes.

The Evolving Role of Radiation Therapy in the Optimal Multimodality Treatment of Childhood Cancer

1998 ◽  
Vol 84 (2) ◽  
pp. 270-273 ◽  
Author(s):  
Fabrizio Lombardi ◽  
Pierina Navarria ◽  
Lorenza Gandola
Keyword(s):  
Radiation Therapy ◽  
Childhood Cancer ◽  
Wilms Tumor ◽  
Multimodality Treatment ◽  
Pediatric Cancers ◽  
Second Tumors ◽  
Total Cancer ◽  
Long Term Survivors

Childhood cancer is rare, representing only 1% of the total cancer problem. Of children diagnosed with cancer today, more than 70% are predicted to be long-term survivors. Essentially all pediatric cancers are treated by interdigitating radiation with surgical resection and systemic chemotherapy. The use of irradiation, important to achieve high rates of disease local control, must be always balanced against late effects specifically related to this treatment modality, principally growth retardation and second tumors induction. Using neuroblastoma, Wilms’ tumor and rhabdomyosarcoma as examples, the advances in the optimal multimodality treatment of childhood cancer and the evolution of the role of radiation therapy are discussed.

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