Pediatric genitourinary tumors

Current Opinion in Oncology

Volumn 8, Issue 3, 1996, Pages 240-246

  Angell, Scott K ^a   Pruthi, Raj S ^a   Merguerian, Paul A ^a  

^a STANFORD UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

IFOSFAMIDE;

ADOLESCENT; CHILD; CHILDHOOD CANCER; CLINICAL FEATURE; CLINICAL TRIAL; FEMALE; HUMAN; INFANT; MALE; NEPHROBLASTOMA; PRIORITY JOURNAL; REVIEW; RHABDOMYOSARCOMA; TESTIS CANCER; UROGENITAL TRACT CANCER;

EID: 0030008840     PISSN: 10408746     EISSN: None     Source Type: Journal    
DOI: 10.1097/00001622-199605000-00013     Document Type: Review

References (42)

1. Priestley JF, Schulte TL: The treatment of Wilms' tumor. J Urol 1942, 47:7.
2. Miller RW, Young JL Jr, Novakovic B: Childhood cancer. Cancer 1995, 75(suppl 1):395-405.
3. Coppes MJ: Wilms tumor: to cure and understanding. Crit Rev Oncol Hematol 1995, 18:179-196. This is a thorough review of the clinical and molecular aspects of Wilms' tumor, with special attention paid to the rationale for the NWTS and International Society of Pediatric Oncology protocols in addition to the results of these studies. The author also discusses the clinical aspects of treatment, including imaging, surgery, chemotherapy, and radiation.
4. Gurney JG, Severson RK, Davis S, Robison LL: Incidence of cancer in children in the United States: sex, race, and 1-year age-specific rates by histologic type. Cancer 1995, 75:2186-2195.
5. Junien C, Henry I: Genetics of Wilms' tumor: a blend of aberrant development and genomic imprinting. Kidney Int 1994, 46:1264-1279. This clearly written review updates the literature to December 1993. The article covers topics such as WT1, WT2, WT3, genomic imprinting, and interactions between genes and gene products and makes an attempt to correlate the genetics of Wilms' tumor with the histopathology.
6. Olson JM, Hamilton A, Breslow NE: Non-11p constitutional chromosome abnormalities in Wilms' tumor patients. Med Pediatr Oncol 1995, 24:305-309.
7. Kudoh T, Ishidate T, Moriyama M, Toyoshima K, Akiyama T: G1 phase arrest Induced by Wilms' tumor protein WT1 is abrogated by cyclin/CDK complexes. Proc Nat Acad Sci U S A 1995, 92:4517-4521.
8. Larsson SH, Charlieu JP, Miyagawa K, Engelkamp D, Rassoulzadegan M, Ross A, Cuzin F, van Heyningen V, Hastie ND: Subnuclear localization of WT1 in splicing or transcription factor domains is regulated by alternative splicing. Cell 1995, 81:391-401. The authors demonstrated how the wt1 protein variants, which lack the three amino acids KTS, between the third and fourth zinc fingers predominantly bind DNA, whereas the variants that have the amino acids prefer binding to other proteins, called splicing factors. These data suggest that the variants function in posttranscription processing of RNA in addition to controlling transcription itself.
9. Nakagama H, Heinrich G, Pelletier J, Housman DE: Sequence and structural requirements for high-affinity DNA binding by the WT1 gene product. Mol Cell Biol 1995, 15:1489-1498. This is a well written article with an elegant experimental design to show the wt1 protein binding site, a 10-base pair motif, and to demonstrate the relative affinity in binding compared with early growth response protein 1. If zinc finger I was mutated, the relative binding dropped 10-fold, but if the other zinc fingers were denatured, there was complete abolishment of binding.
10. Wang ZY, Qiu QQ, Huang J, Gurrieri M, Deuel TF: Products of alternatively spliced transcripts of the Wilms' tumor suppressor gene, WT1, have altered DNA binding specificity and regulate transcription in different ways. Oncogene 1995, 10:415-422.
11. Taniguchi T, Sullivan MJ, Ogawa O, Reeve AE: Epigenetic changes encompassing the IGF2/H19 locus associated with relaxation of IGF2 imprinting and silencing of H19 in Wilms' tumor. Proc Nat Acad Sci U S A 1995, 92:2159-2163. This is an important paper in which gene expression experiments show that biallelic expression of IGF-2 was associated with low or no expression of H19, indicating a loss of imprinting of IGF-2 due to methylation of H19. All Wilms' tumors had hypermethylation of 11p15; therefore, the authors concluded that methylation may be the signal involved in imprinting and may be responsible for the disruption in tumor surveillance of the repressor genes.
12. Maheswaran S, Englert C, Bennett P, Heinrich G, Haber DA: The WT1 gene product stabilizes p53 and inhibits p53-mediated apoptosis. Genes Dev 1995, 9:2143-2156. In this elegant experimental design, the authors used cotransfection assays to show that the presence of wt1 protein stabilizes p53 but inactivates its apoptotic function, inhibiting its role in tumor suppression. This effect was mapped to zinc fingers I and II on the wt1 protein. The wt1 and p53 interaction led to an increased half-life and resistance to degradation of p53 while its transcription repression was reduced.
13. Craft AW, Parker L, Stiller C, Cole M: Screening for Wilms' tumour in patients with aniridia, Beckwith syndrome, or hemihypertrophy. Med Pediatr Oncol 1995, 24:231-234.
14. Beckwith JB: Certain conditions have an increased incidence of Wilms' tumor. Am J Roentgenol AJR 1995, 164:1294-1295.
15. Lin RY, Argenta PA, Sullivan KM, Stern R, Adzick NS: Urinary hyaluronic acid is a Wilms' tumor marker. J Pediatr Surg 1995, 30:304-308. In this prospective, controlled study of Wilms' tumor patients, pre- and postoperative hyaluronic acid levels were measured. The patients had significantly higher levels preoperatively and with recurrence. Levels were significantly lower after removal of the tumor. There was no correlation between hyaluronic acid levels and stage, histology, or outcome.
16. Leckie BJ, Birnie G, Carachi R: Renin in Wilms' tumor: a prorenin as an indicator. J Clin Endocrinol Metab 1994, 79:1742-1746. This prospective, well-controlled study evaluated the usefulness of renin and prorenin as a tumor marker in 25 children. Patients with Wilms' tumors had significantly higher levels preoperatively, which dropped to control levels after surgery. Interestingly, patients with stage I and II tumors had significantly higher levels of prorenin than stage III and IV patients. The authors also used elaborate biochemical techniques to confirm that prorenin comes from the tumor and not from reactive surrounding renal tissue.
17. Plumley DA, Grosfeld JL, Kopecky KK, Buckwalter KA, Vaughan WG: The role of spiral (helical) computerized tomography with three-dimensional reconstruction in pediatric solid tumors. J Pediatr Surg 1995, 30:317-321. This case review article illustrates how the dual-spiral CT scanner is used to demonstrate the resectability of certain Wilms' tumors that were thought to be unresectable by conventional imaging techniques.
18. Ritchey ML, Green DM, Breslow NB, Moksness J, Norkool P: Accuracy of current imaging modalities in the diagnosis of synchronous bilateral Wilms' tumor a report from the National Wilms' Tumor Study Group. Cancer 1995, 75:600-604. This retrospective review illustrates the importance of diagnosing bilateral Wilms' tumors to allow for parenchymal sparing surgery. The authors concluded that current imaging modalities are inadequate to eliminate the need for surgical exploration of the contralateral kidney.
19. Ritchey ML, Pringle KC, Breslow NE, Takashima J, Moksness J, Zuppan CW, Beckwith JB, Thomas PR, Kelalis PP: Management and outcome of inoperable Wilms' tumor: a report of National Wilms' Tumor Study-3. Ann Surg 1994, 220:683-690. In this important retrospective review, NWTS-3 patients who were judged inoperable before treatment were compared with patients who underwent primary tumor resection. The study included 131 patients who were initially believed inoperable and who were followed up for a mean of almost 6 years. Patients with stage V disease were excluded. The authors derived many important conclusions from these data, and an algorithm for treatment is presented.
20. Segal LS, Palumbo RC, Robertson WW Jr: The development of rickets as a complication of chemotherapy for the treatment of Wilms' tumor. Orthopedics 1995, 18:261-264.
21. Breslow NE, Takashima JR, Whitton JA, Moksness J, D'Angio GJ, Green DM: Second malignant neoplasms following treatment for Wilm's tumor: a report from the National Wilms' Tumor Study Group. J Clin Oncol 1995, 13:1851-1859. This is an impressive, retrospective follow-up study reporting on 43 tumors in more than 5000 patients previously treated for Wilms' tumor. Based on the same population, the expected number of tumors was 5.1. By 15 years since the initial diagnosis, 1.6% of patients were projected to develop a second tumor. Abdominal irradiation significantly increased the risk, especially in combination with doxorubicin.
22. Pappo AS, Shapiro DN, Christ WM, Maurer HM: Biology and therapy of pediatric rhabdomyosarcoma. J Clin Oncol 1995, 13:2123-2139. This is a well-written review of the key developments in the biology, treatment, and late effects of therapy of rhabdomyosarcoma. Included is a thorough background and overview of relevant clincal and histopathologic features of rhabdomyosarcoma, an excellent update of current molecular and genetic understanding of rhabdomyosarcoma, and a detailed summary of therapy outcomes from recent cooperative trials including IRS and non-IRS trials.
23. Kilpatrick SE, Teot LA, Geisinger KR, Martin PL, Shumate DK, Zbieranski N, Russell GB, Fletcher CDM: Relationship of DNA ploidy to histology and prognosis In rhabdomyosarcoma. Cancer 1994, 74:3227-3233.
24. Schafer BW, Czerny T, Bernasconi M, Genini M, Busslinger M: Molecular cloning and characterization of a human PAX-7 cDNA expressed in normal and neoplastic myocytes. Nucleic Acids Res 1994, 22:4574-4582. A variant translocation involving the PAX7 gene has been implicated in aberrant transcription regulation and myogenic tumor formation. This study found the PAX7 gene, like the PAX3 gene, is weekly expressed in normal human myoblasts, yet overexpressed in rhabdomyosarcoma cell lines. PAX7 also shares similar DNA binding properties and transactivation potential as PAX3. The authors demonstrated further evidence that the overexpression of either or both genes may result in the formation of myogenic tumors such as rhabdomyosarcoma.
25. Biegel JA, Nycum LM, Valentine V, Barr FG, Shapiro DN: Detection of the t(2;13)(q35;q14) and PAX3-FKHR fusion in alveolar rhabdomyosarcoma by fluorescence in situ hybridization. Genes Chromosom Cancer 1995, 12:186-192. The characteristic chromosomal abnormality in alveolar rhabdomyosarcoma is the t(2;13)(q35;q14) translocation. Reverse transcriptase polymerase chain reaction techniques have been commonly used to detect the resultant chimeric mRNA of this translocation. This study demonstrates the use of a two-colored fluorescence in situ hybridization assay as a rapid, sensitive means of identifying the characteristic t(2;13) translocation in alveolar rhabdomyosarcoma.
26. Wijnaendts LCD, Van Der Linden JC, Van Unnik AJM, Delemarre JFM, Voute§ PA, Meijer CJLM: The expression pattern of contractile and intermediate filament proteins in developing skeletal muscle and rhabdomyosarcoma of childhood: diagnostic and prognostic utility. J Pathol 1994, 174:283-292.
27. Diller L, Sexsmith E, Gottlieb A, Li FP, Malkin D: Germline p53 mutations are frequently detected in young children with rhabdomyosarcoma. J Clin Invest 1995, 95:1606-1611.
28. Christ W, Gehan EA, Ragab AH, Dickman PS, Donaldson SS, Fryer C, Hammond D, Hays DM, Herrmann J, Heyn R, et al.: The Third Intergroup Rhabdomyosarcoma Study. J Clin Oncol 1995, 13:610-630. This important paper reports the complete results of IRS-III. Overall outcomes for IRS-III are significantly better than those for IRS-II. Overall survival improved from 63% to 71%, and progression-free survival improved from 55% to 65%. Review of this publication is recommended to obtain specific therapy protocols and outcomes based on group, grade, and primary tumor site.
29. Hays DM, Raney RB, Wharam MD, Weiner E, Lobe TE, Andrassy RJ, Lawrence W, Johnston J, Webber B, Maurer HM: Children with vesical rhabdomyosarcoma treated by partial cystectomy with neoadjuvant chemotherapy, with or without radiotherapy. J Pediatr Hematol Oncol 1994, 17:46-52. This interesting report from the IRS committee evaluates the feasibility of partial cystectomy versus total cystectomy in patients with bladder rhabdomyosarcoma. The results show that 31 of 40 patients (78.5%) were disease free at 2 to 16 years of follow-up. These results compare favorably to all cases of bladder rhabdomyosarcoma in the same interval. The authors concluded that partial cystectomy is a reasonable alternative to total cystectomy when the tumor is confined to the dome or anterior wall of the bladder.
30. Nooter K, Boersma AWM, Oostrum RG, Burger H, Jochemsen AG, Stoter G: Constitutive expression of the c-H-ras oncogene inhibits doxorubicin-induced apoptosis and promotes cell survival in a rhabdomyosarcoma cell line. Br J Cancer 1995, 71:556-561. The authors investigated the effects of the c-H-ras oncogene on doxorubicin-induced apoptosis and cell survival in rhabdomyosarcoma cell lines. The rhabdomyosarcoma cells containing the c-H-ras oncogene had a three-to fivefold less cytotoxicity and significantly reduced apoptotic rates than native rhabdomyosarcoma cells. Their results illustrate that tumors with the c-H-ras oncogene may be less susceptible to certain chemotherapeutic agents such as doxorubicin. Furthermore, these findings support the allegation that genetically controlled factors may play a significant role in drug resistance.
31. Phillips MB, Flamant F, Sommelet-Olive D, Pinkerton CR: Phase II study of rapid-scheduled etoposide in paediatric soft tissue sarcomas. Eur J Cancer 1995, 31A:782-784. The authors studied the clinical use of rapid-scheduled etoposide (three consecutive days weekly for 3 weeks) in treating resistant or refractory rhabdomyosarcoma. In 19 patients, response rates improved to 43%. This study illustrates that an alternative in overcoming drug resistance may be modification of dosing regimen rather than altering the agents themselves.
32. cdc2. Cancer Res 1994, 54:5531-5534.
33. Regine WF, Fontanesi J, Kumar P, Ayers D, Bowman LC, Pappo AS, Coffey DH, Avery L, Rao BN, Kun LE: Local tumor control in rhabdomyosarcoma following low-dose irradiation: comparison of group II and select group III patients. Int J Radiat Oncol Biol Phys 1995, 31:485-491. This paper provides the results of using low-dose irradiation to treat patients left with locally advanced microscopic disease (group III) after induction chemotherapy. The authors concluded that low-dose irradiation can effectively achieve local control in group III patients left with microscopic disease and that a dose level of at least 40 Gy should be used.
34. Regine WF, Fontanesi J, Kumar P, Zeitzer K, Greenwald C, Bowman L, Shapiro DN, Rao BN, Kun LE: A phase II trial evaluating selective use of altered radiation dose and fractionation in patients with unresectable rhabdomyosarcoma. Int J Radiat Oncol Biol Physics 1995, 31:799-805. The authors reported on results of a prospective trial of group III and group IV IRS patients with gross residual disease after induction chemotherapy. Among the patients who received hyperfractionated radiation therapy at a dose of 60 Gy, the absolute 2-year continuous local tumor control rate was 75% 33 to 67 months after irradiation. This result represents an improvement in local tumor control using this modality. The authors concluded that hyperfractionated radiation therapy can be used selectively to treat advanced rhabdomyosarcoma patients left with gross residual disease after induction chemotherapy to achieve improved local control rates with minimal late toxicity.
35. Donaldson SS, Asmar L, Breneman J, Fryer C, Glicksman AS, Laurie F, Wharam M, Gehan EA: Hyperfractionated radiation in children with rhabdomyosarcoma: results of an intergroup rhabdomyosarcoma pilot study. Int J Radiat Oncol Biol Physics 1995, 32:903-911. This paper provides the results of an IRS group pilot study (IRS IV-P) of hyperfractionated radiation therapy with chemotherapy in group II and group IV patients with gross residual disease. The results show that hyperfractionated irradiation and chemotherapy, in this patient population, is a feasible and tolerable therapeutic approach. The efficacy of hyperfractionated irradiation compared with conventional irradiation protocols is currently being evaluated.
36. Raney B, Ensign LG, Foreman J, Khan F, Newton W, Ortega J, Ragab A, Wharam M, Weiner E, Maurer H: Renal toxicity of ifosfamide in pilot regimens of the intergroup rhabdomyosarcoma study for patients with gross residual tumor. Am J Pediatr Hematol Oncol 1994, 116:286-295. The authors reviewed and characterized the potential nephrotoxicity of patients receiving ifosfamide as part of their IRS protocol. The findings suggest that patients younger than 3 years of age who receive more than eight courses of ifosfamide should be carefully monitored for nephrotoxicity. Furthermore, patients with preexisting renal abnormalities are at an increased risk, and the authors recommend the use of ifosfamide only if its benefits clearly exceed the potential risk.
37. Yeung CK, Ward HC, Ransley PG, Duffy PG, Pritchard J: Bladder and kidney function after cure of pelvic rhabdomyosarcoma in childhood. Br J Cancer 1994, 70:1000-1003. Eleven survivors of pelvic rhabdomyosarcoma underwent bladder function studies and upper-tract evaluation to review the functional sequelae of patients cured of pelvic rhabdomyosarcoma. All patients had bladder-sparing surgery, and seven received pelvic irradiation. Surprisingly, the results revealed that the extent of surgical resection did not correlate with the presence of voiding dysfunction. Instead, all seven patients who received irradiation had bladder or renal abnormalities. In contrast, each of the four patients who did not receive radiation had a normal functional bladder with a normal voiding pattern. The authors concluded that all patients, especially those who have received irradiation, should have careful follow-up including the use of frequency-volume voiding charts.
38. Moller H, Jorgensen N, Forman D: Trends in incidence of testicular cancer in boys and adolescent men. Int J Cancer 1995, 61:761-764. This article reviews the population data published previously for Denmark, Norway, and Sweden and indicates the incidence of testicular cancer, which is low, with an average of two cases per 100,000 boys, and stable, except in the adolescent population, in which the incidence is increasingly by 6% a year, possibly due to a younger age at puberty.
39. Gilliland FD, Key CR: Male genital cancers. Cancer 1995 75(suppl 1):295-315. This article reviews testicular cancer and provides information on the incidence. It also discuses yolk sac tumor, which is most common from ages 0 to 9 years. The paper also reports that 70% of yolk sac tumors are localized at the time of diagnosis.
40. Hittmair A, Rogatsch H, Feichtinger H, Hobisch A, Mikuz G: Carcinoma in situ of the testis detected by DNA flow cytometry of testicular fine-needle aspirates. Cytometry 1994, 17:327-331. In this paper, the authors described fine-needle aspiration and flow cytometry to diagnose carcinoma in situ. The technique was accurate in 15 of 16 specimens. The technique missed one area of carcinoma in situ that was a very small volume lesion by histologic section and that probably would have been missed by open biopsy as well.
41. Bischof EF Jr, Herold AH, Marty PJ: Bilateral testicular cancer. J Am Board Fam Pract 1994, 7:516-519. This case report article provides a good review of the active issues in bilateral testicular cancer treatment including fertility, carcinoma in situ, and chemotherapy beyond the testes blood barrier.
42. Grady RW, Ross JH, Kay R: Patterns of metastatic spread in prepubertal yolk sac tumor of the testis. J Urol 1995, 153:1259-1261. This is a review of the American Academy of Pediatrics tumor registry for metastatic yolk sac tumors. The authors reported on 33 cases that were metastatic at the time of presentation, with 27% localized to the retroperitoneum, 40% with hematogenous spread only, and 19% with both retroperitoneal and hematogenous disease. One of the cases presented with local invasion, and the site of metastasis was not documented in five cases.