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TUMORS Significant advances have been made in the management of infants and children with malignant solid tumors.

Improved understanding of tumor behavior and response to combined modalities of treatment has resulted in improved survival in a number of neoplasms. Many of these advances have been made possible by the development of cooperative study groups and multidisciplinary care. Wilms' Tumor Wilms' tumor (nephroblastoma) is an embryonal tumor of renal origin. Appro imately !"" new cases of this pediatric malignancy are seen in the #nited States annually. Most are managed according to protocols of the $ational Wilms' %umor Study &roup ($W%S). An abdominal mass is often detected by a parent while bathing the infant or child. 'ematuria is noted in (" to (!) of cases* often after relatively minimal trauma to an unsuspected renal tumor. 'ypertension is present in +") of cases and is related to compression of the ,u taglomerular apparatus by the tumor* causing a renin-angiotensin release. %he ma,ority of patients are diagnosed between ( and . years of age. 'owever* Wilms' tumor occurs in older children and occasionally adolescents or young adults. Wilms' tumor is more commonly observed in infants with sporadic aniridia* hemihypertrophy* /ec0with-Wiedemann syndrome* neurofibromatosis* horseshoe 0idney* and 1earlman's syndrome2 in families with genitourinary anomalies2 and as a hereditary factor associated with the eighth and eleventh chromosomes. %he W%( gene is located on the eleventh chromosome (((p (3). %he W%-+ gene has been identified on the ((p (! locus in children with /ec0with's syndrome. Some children with aggressive* unresponsive tumors have an abnormal locus on the si teenth chromosome. 4n physical e amination* Wilms' tumor presents as a round* smooth* hard flan0 mass. %he mass is usually nontender. #ltrasonography demonstrates a solid intrarenal lesion and also indicates whether the tumor has e tended into the renal vein* inferior vena cava* or occasionally the right atrium. %he ne t diagnostic test obtained is a 5% study of the abdomen with intravenous contrast. %his demonstrates a renal mass and a pyelogram effect showing intrinsic distortion of the collecting system with medial displacement of the 0idney. %he 5% study usually separates the 0idney from other organs (e.g.* adrenal glands)* indicates whether the tumor involves the liver on the right side or the diaphragm on either side by direct e tension* identifies the presence of enlarged and suspicious perirenal and para-aortic lymph nodes* evaluates for a second tumor in the opposite 0idney* and determines whether the patient has liver metastases. A chest -ray and chest 5% scan are obtained to evaluate for lung metastases. /one survey is obtained in children with clear-cell renal tumors who are at ris0 for bone metastasis* which is rare in patients with Wilms' tumor. Arteriograms and M6I are rarely helpful in the preoperative evaluation of Wilms' tumor* and for most patients* these tests are both unnecessary and unduly e pensive. Special (helical) 5% with three-dimensional reconstruction is useful in determining the resectability of tumors in close pro imity to large vessels. %he treatment of Wilms' tumor depends on the staging of the specific case by preoperative and postoperative evaluation of the e tent of disease based on

preliminary studies as outlined above2 the resectability of the primary tumor2 the status of perirenal* capsular* and lymph node involvement2 local invasion of nearby organs and structures (e.g.* liver* diaphragm* mesentery)2 tumor histology2 and the presence or absence of distant metastases or contralateral involvement (second Wilms' tumor). 4perative management includes a carefully planned* wellmonitored radical resection of the affected 0idney through a long transverse transabdominal incision under general endotracheal anesthesia. %he incision should be large enough to e amine both sides and remove the tumor without spillage. %he opposite 0idney and the liver are first evaluated for possible tumor involvement. %he colon attachment and mesentery are carefully separated from the tumor and moved medially. %he duodenum and liver are carefully freed on the right side* and the splenic fle ure of the colon is mobili7ed on the left. %he spleen and pancreas are elevated and retracted anteriorly and superiorly on the left side* e posing the entire upper retroperitoneal space and diaphragm. When feasible* the hilum of the 0idney is approached initially* and the renal artery and vein are identified* doubly ligated* and divided. %his controls blood loss during the resection and also theoretically reduces the ris0 of blood-borne and lymphatic metastases during the procedure. %his may not always be possible* and mobili7ation of the tumor may be necessary in some instances to clearly identify the vascular pedicle without in,uring other structures (e.g.* superior mesenteric artery). %he tumor is dissected free from the aorta* and the specimen includes lymph node8bearing tissues in the renal hilum and ipsilateral para-aortic region* which are e cised for staging purposes. %he retrorenal fossa is freed* and any attachments to the diaphragm may re9uire en bloc e cision with the tumor. 4n the right side* direct e tension of tumor into the liver also re9uires en bloc resection in (.! to :) of cases. %he adrenal gland can be spared if the tumor is small or is located in the inferior pole2 however* this organ must be e cised if the primary lesion involves the superior pole of the 0idney. In rare instances* the tumor may not be amenable to safe resection at the first procedure. #nder these circumstances* a biopsy is obtained to confirm the histology (which is inade9uate with a needle biopsy)* and the patient is treated with two courses of chemotherapy to shrin0 the tumor. A successful second-loo0 resection of the tumor is almost always possible. 1ostoperative complications are infre9uent. %he current staging system of the $W%S-. is listed in %able (. ;ollowing surgical e cision* treatment depends on both stage and histology. %he histologic evaluation of Wilms' tumor is accomplished in a central pathology center to confirm local impressions. 'istology is divided into favorable (;') and unfavorable (#') types* which reflect invasiveness of the tumor and probable response to therapy. ;avorable lesions represent <=) of cases* unfavorable ((). %he former are characteri7ed by blastemal* epithelial* mi ed* and even glomerular elements* and the latter are anaplastic. Anaplastic tumors are characteri7ed by nuclear pleomorphism and e treme hyperdiploidy. Stage I and II cases with ;' are treated with actinomycin-> and vincristine in pulse courses and have a =!) survival. Anaplastic tumors are rare under the age of + years. If an anaplastic tumor is completely resected and considered Stage I* the outcome is similar to Stage I

infants with ;'. In more advanced stages* however* anaplastic tumors have a !!) relapse rate and .!) mortality. %he overall survival for ;' tumors is =") for all stages. %he few patients with ;' tumors who succumb are nonresponsive to treatment and usually have primitive blastemal tumors* which are more invasive in nature. In Stages I and II* these tumors are more adherent to surrounding structures* and there may be tumor in intrarenal vessels. Stage III patients have an <") survival* and even in the presence of metastatic disease (Stage I?)* @") survive. %hese advanced cases are treated with three-drug chemotherapy* with the addition of adriamycin to the treatment protocol. #nresponsive cases are treated with second-phase drugs* including ifosfamide and cis-platinum. %he treatment of pulmonary metastases is still controversial. %reatment with chemotherapy and whole-lung irradiation ((<"" c&y to each side) appears to be as effective alone as when combined with surgical e cision of multiple metastases. %he incidence of pulmonary relapse is 9uite low (:))* but (3) of patients ac9uire radiation pneumonitis as a complication of therapy. Another special circumstance is management of intracaval involvement* which occurs in .) of cases. Appropriate e cision of the primary tumor and tumor thrombus is advised. %he level of tumor thrombus is an important preoperative consideration (infrahepatic cava* @()2 intrahepatic cava* (.)2 and suprahepatic inferior vena cava or right atrium* +()). In the latter cases* cardiopulmonary bypass should be an available surgical ad,unct. 5ardiovascular bypass has been used successfully by a number of investigators to safely remove tumor thrombus from the right atrium and pulmonary artery. %he level of thrombus involvement does not affect outcome. 6ecent data suggest that intravascular tumor can be successfully managed with preoperative chemotherapy and delayed surgical intervention* if necessary. %he overall survival is <<)* <=)* and @+) for Stages II* III* and I?* respectively. %he 0ey indicator of survival is tumor histology. In cases of massive involvement* using preoperative chemotherapy to shrin0 the tumor may ma0e the resection safer. In Stage ? disease (bilateral Wilms' tumor)* a very conservative approach to therapy is recommended in an attempt to preserve renal parenchyma and to avoid bilateral nephrectomy* the need for dialysis* and renal transplantation. %reatment options include bilateral heminephrectomy if feasible* initial bilateral biopsy* chemotherapy* avoidance of irradiation therapy and second- or even third-loo0 laparotomies to limit renal resection* and total nephrectomy on one side and partial nephrectomy on the other. In a relatively large group of $W%S patients* the 3-year survival was :@)* despite institutional variation in therapy. 5urrently* unless both tumors are small and allow bilateral partial nephrectomy* bilateral biopsy and chemotherapy at the onset of treatment are favored. ;ortunately* most patients have favorable histology (<@)). %en percent have #;* and .) have discordant pathology* with ;' on one side and #' on the other. %his latter observation indicates that both 0idneys must always be biopsied. %he best prognosis in Stage ? disease is in patients less than 3 three years of age with ;'* negative lymph node involvement* and lower staging of the more advanced of the two renal lesions.

%A/AB (. Staging for Wilms' %umor Stage >escription I %umor limited to the 0idney and completely resected II %umor e tends beyond the 0idney but is completely resected2 capsule invasion* perirenal tissues may be involved III 6esidual nonhematogenous tumor confined to the abdomen* including tumor rupture (or biopsy) or peritoneal implants2 lymph nodes involved I? 'ematogenous metastases (lung* distant lymph nodes* brain) ? /ilateral renal involvement

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VUJVIHNV RIY^GZ M XSJKDLP N SOMPSJKn fGJZNE. jRHIRGSTREZZL G ucv RIY^S OSJI]DL U YSSOIRE[GSDDS\ S[ID^I SOMPSJG fGJZNE* G YJV XSJK`GDNHUE OE[GIDHSU* dHG GNOLHEDGV VUJVnHNV G DIDMbDLZG G DI]E^SDDS YSRSTGZG. hOI[GEJKDEV (UGDHSUEV) rs N HRIPZIRDS\ RI^SDNHRM^[GI\ OSJI]DE U SORIYIJIDGG resectability SOMPSJI\ U XJG]^S\ XJG]SNHG ^ XSJK`SZM NSNMYM. eXREXSH^EQwIFIDGIW SOMPSJG fGJZNE ]EUGNGH SH SORIYIJIDGV NHEYG\DSNHG SORIYIJIDDSTS NJMFEV YSSOIRE[GSDDS\ G OSNJISOIRE[GSDDS\ S[ID^S\ REN`GRIDGV XSJI]DG* SNDSUEDDS\ DE ORIYUERGHIJKDSZ GNNJIYSUEDGG ^E^ ULYIJIDS UL`I2 resectability OIRUGFDS\ SOMPSJG2 NHEHMN ORGFENHDSNHGQUSUJIFIDGVW S^SJSOSFIFDSTS* ^EONMJKDSTS* G JGZoEHGFIN^STS M]JE2 ZINHDSI UHSRbIDGI U XJG]JIbEqGI SRTEDL G NHRM^HMRL (DEORGZIR* OIFIDK* YGEoRETZM* XRLbI\^E)2 TGNHSJSTGV SOMPSJG2 G DEJGFGI GJG SHNMHNHUGI SHYEJIDDLP ZIHENHE] GJG ^SDHREJEHIREJKDS\ ORGFENHDSNHGQUSUJIFIDGVW (SOMPSJK UHSRSTS fGJZNE). eOIRE[GSDDSI MOREUJIDGI U^JnFEIH HqEHIJKDS ]EOJEDGRSUEDDMn* PSRS`SZSDGHSRGRSUEDDMn REYG^EJKDMn RI]I^[Gn OSURIbYIDDS\ OSF^G FIRI] YJGDDL\ OSOIRIFDL\ EXYSZGDEJKDL\ RE]RI] OSY SXqI\ dDYSHREPIEJKDS\ EDENHI]GI\. !!* @! RE]RI] YSJbID XLHK YSNHEHSFDS XSJK`GZ* FHSXL GNNJIYSUEHK SXI NHSRSDL G MYEJGHK SOMPSJK XI] RE]RLUE. gRSHGUSOSJSbDEV OSF^E G OIFIDK NDEFEJE S[IDIDL DE US]ZSbDMn ORGFENHDSNHKQUSUJIFIDGIW SOMPSJG. gRGJSbIDGI HSJNHS\ ^G`^G G XRLbI\^E HqEHIJKDS SHYIJIDL SH SOMPSJG G OIRIZIqIDLQHRSDMHLW U NIRIYGDI. xUIDEY[EHGOIRNHDEV ^G`^E G OIFIDK HqEHIJKDS ULYIJIDL DE OREUGJKDS\ NHSRSDI* G NIJI]IDSFDSI NTGXEDGI HSJNHS\ ^G`^G ZSXGJG]SUEDS NJIUE. hIJI]ID^E G OSYbIJMYSFDEV bIJI]E MUIJGFIDLQOSYDVHLW G SHRIFIDL REDK`I G UL`I DE JIUS\ NHSRSDI* OSYUIRTEV US]YI\NHUGnQULNHEUJVVW OSJDSI UIRPDII ]EXSRn`GDDSI ZINHS G YGEoRETZM. rSTYE ULOSJDGZS* ^ RMXFG^M NIZIDG OSF^G ORGXJGbEnHNV OIRUSDEFEJKDS* G OSFIFDEV ERHIRGV* G UIDE GYIDHGoG[GRSUEDE* UYUS\DI JGTGRSUEDE* G RE]YIJIDE. !@ tHS MOREUJVIH OSHIRn ^RSUG U HIFIDGI RI]I^[GG G HE^bI HISRIHGFIN^G MZIDK`EIHQUOREUJVIHW RGN^ OIRIDINIDDLP ^RSUKn G JGZoEHGFIN^GP ZIHENHE] U HIFIDGI ORS[IYMRL. tHS ZSbIH DI UNITYE XLHK US]ZSbDS* G ZSXGJG]E[GV SOMPSJG ZSbIH XLHK DISXPSYGZE U DI^SHSRLP NJMFEVP VNDS GYIDHGoG[GRSUEHK NSNMYGNHMn DSb^M* DI OSURIbYEV YRMTGI NHRM^HMRL (DEORGZIR* ORIUSNPSYVqEV XRLbIIFDEV ERHIRGV). eOMPSJK RENNIFIDE NUSXSYDS\ SH ESRHL* G d^]IZOJVR U^JnFEIH H^EDG OIRIDINIDGV M]JE JGZoL U OSFIFDL\ RMXFG^ NIZIDG G SHDSNVqMnNV ^ SYDS\ NHSRSDI HIJE OEREESRHEJKDMn SXJENHK* ^SHSRLI GNNIFIDL YJV SRTEDG]MnqGP [IJI\. retrorenal VZ^E ULYIJIDE* G JnXLI ORGJSbIDGV ^ YGEoRETZI ZSTMH HRIXSUEHK en ULRI]EDGV XJS^E N SOMPSJKn. pE OREUGJKDS\ NHSRSDI* ORVZSI ULHVbIDGI SOMPSJG U OIFIDK HE^bI HRIXMIH en RI]I^[GG XJS^E U (.! ^ : ) NJMFEIU. pEYOSFIFDG^ ZSbIH XLHK NSPREDIDQNd^SDSZJIDW* INJG SOMPSJK VUJVIHNV ZEJIDK^S\ GJG JS^EJG]SUEDE DG]`G\ OSJnNQOSJV^W2 SYDE^S* dHSH SRTED YSJbID XLHK GNNIFID* INJG OIRUGFDSI OSREbIDGI USUJI^EIH ORIUSNPSYVqG\ OSJnNQOSJV^EW OSF^G. f RIY^GP NJMFEVP* SOMPSJK DI ZSbIH XLHK OSYNMYDEQOSYYEnqEVNVW ^ XI]SOENDS\ RI]I^[GG ORG OIRUS\ ORS[IYMRI. gRG dHGP SXNHSVHIJKNHUEP* XGSONGV OSJMFIDE* FHSXL OSYHUIRYGHK TGNHSJSTGn

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