Devita, Hellman, and Rosenberg's Cancer

LWBK1006-29 LWW-Govindan-Review December 9, 2011 15:36

Chapter 29•Cancers of Childhood 411

which HCC presents is 12 years. Both demonstrate a male predominance.

Patients with hepatoblastoma frequently have additional abnormalities,

such as hemihypertrophy, congenital absence of the kidney or adrenal

gland, and umbilical hernia. AFP levels are increased in 90% of patients

with hepatoblastoma and in 78% of adult patients with HCC. HCC is

associated with hepatitis B, biliary atresia, galactosemia, Fanconi anemia,

and alpha 1-antitrypsin deficiency. Malignant tumors of the liver are rarely

calcified.

Answer 29.44. The answer is A.

Beckwith-Wiedemann syndrome is associated with hereditary predispo-

sition for developing Wilm’s tumor, hepatoblastoma, adrenal carcinoma

and RMS.

Answer 29.45. The answer is C.

Intracranial metastasis is not a common feature of malignant hepatic

tumors. The grouping system used in therapeutic studies segregates

patients according to resectability and lymph node or hematogenous

metastases. Laboratory tests are not useful for a differential diagnosis

of malignant hepatic tumors in children. Pulmonary metastases are iden-

tified on a plain chest radiograph in approximately 10% of patients with

hepatic tumors. The PRETEXT staging system by the European liver study

group divides the liver into four sectors and characterizes tumors by the

sectors involved; it was devised to facilitate the assessment of neoadju-

vant chemotherapy in rendering tumors resectable and appears to have

prognostic value.

Answer 29.46. The answer is D.

Resection is the primary treatment of hepatoblastoma and HCC. Long-

term survival is rare in the absence of successful resection. Hepatoblas-

toma is generally unifocal, and approximately one half of all hepatoblas-

tomas are resectable at initial presentation. HCC has an invasive pattern

of spread across anatomic planes and is unresponsive to current forms of

chemotherapy. Complete resection of HCC is frequently difficult because

of multifocality and invasiveness. Only 30% of HCC tumors can be fully

resected at diagnosis.

Answer 29.47. The answer is B.

Only 5% to 10% of germ cell tumors are extragonadal in adults, whereas

60% are extragonadal in children. Forty percent of all childhood germ cell

tumors involve the sacrococcygeal region. Germ cell tumors are relatively

rare in children and account for 3% of pediatric malignancies.

Answer 29.48. The answer is D.

All the others are varieties of germ cell tumors, each with characteristic

histologic features. High AFP levels are common in pediatric patients with

testicular, ovarian, presacral, and vaginal primary yolk sac tumors. AFP

is a glycoprotein produced in the liver, gastrointestinal tract, and yolk sac