LWBK1006-29 LWW-Govindan-Review December 9, 2011 15:36
Chapter 29•Cancers of Childhood 407small chance that involvement of the opposite kidney may be missed by
all other imaging studies.Answer 29.22. The answer is D.
Surgical resection of the tumor is combined with lymph node sampling
and examination of the contralateral kidney (bilateral tumor is present in
5% of patients) to complete staging. Stage III disease involves the pres-
ence of residual nonhematogenous tumor confined to the abdomen. The
chemotherapy combination used through serial NWTSG trials is vin-
cristine and dactinomycin for patients with a low stage (I and II) and
doxorubicin with or without cyclophosphamide for patients with a higher
stage and those with unfavorable histology, such as anaplastic Wilms’
tumor or clear cell sarcoma.Answer 29.23. The answer is A.
Some of these prognostic factors no longer pertain because of the devel-
opment of effective therapy via the NWSTG (trials 1 to 5) and other inter-
national trials. Histology and lymph node involvement remain the most
important prognostic factors. Survival for most children with favorable
histology Wilms’ tumor now approaches 90%. The presence of extension
or metastatic spread results in intensification of therapy.Answer 29.24. The answer is B.
Neuroblastoma, a tumor of the sympathetic nervous system, dissemi-
nates frequently to the bone, bone marrow, and lymph nodes. Clinical
symptoms depend on the location of the tumor and may include Horner
syndrome and spinal cord compression. Hematogenous dissemination is
evident in 62% of patients at diagnosis. Undifferentiated tumor (Shimada
histology), low DNA ploidy, N-myc amplification, and disseminated dis-
ease are associated with poor prognosis. Other poor prognostic markers
include low Trk A and high Trk B expression, chromosome 1p deletion,
and chromosome 17q gains. In a unique small group of infants, small pri-
mary tumors with metastatic disease restricted to the liver, skin, and bone
marrow without bone involvement (stage IV S) have favorable prognosis.
Tumors in infants often have the capacity to regress spontaneously. For
the new International Neuroblastoma Staging System (INSS), seePPO
Table 50.2.2 on page 2047.Answer 29.25. The answer is C.
Intense chemotherapy alone achieves a remission rate of 22%±4% in
disseminated neuroblastoma and can be improved to 34% ±4% with
autologous bone marrow transplant. The addition of 13-cis retinoic acid
improves the 3-year relapse-free survival rate to 46%± 6% in these
patients. Those with residual disease or positive lymph nodes have better
control with local radiation therapy (24 to 30 Gy) to involved areas. Low-
risk patients (stage I, stage II with N-myc amplification<10 or>10 with
favorable Shimada histology, stage IVS, and hyperdiploidy) often require