Cancer Health Center

Note: Some citations in the text of this section are followed by a level of evidence. The PDQ editorial boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)

Abdominal cancers include adrenocortical tumors, carcinomas of the stomach, cancer of the pancreas, colorectal carcinomas, carcinoid tumors, and gastrointestinal stromal tumors. The prognosis, diagnosis, classification, and treatment of these abdominal cancers are discussed below. It must be emphasized that these cancers are seen very infrequently in patients younger than 15 years, and most of the evidence is derived from case series. (Refer to the Renal Cell Carcinoma section in the PDQ summary on Wilms Tumor and Other Childhood Kidney Tumors for more information.)

Carcinoma of the Adrenal Cortex

Adrenocortical tumors are classified as carcinomas and adenomas.[1,2,3,4,5,6] These tumors occur throughout the pediatric age group, including the fetus and newborn.[7] Adrenocortical tumors may be hormonally active or inactive. Adenomas are generally benign, whereas adrenocortical carcinomas frequently secrete hormones and may cause the patient to develop masculine traits, irrespective of the patient's gender. Pediatric patients with adrenocortical carcinoma often have Li-Fraumeni syndrome, which is an inherited condition that predisposes family members to multiple cancers, including breast cancer, rhabdomyosarcoma, and osteosarcoma.[8] A variety of p53 mutations associated with Li-Fraumeni syndrome have been observed in North American children with adrenocortical carcinoma, whereas in a southern Brazilian population, a distinctive p53 mutation predisposes to this disease.[1,9,10] Children with Beckwith-Wiedemann syndrome [11] or hemihypertrophy [12] are at an increased risk of developing carcinoma of the adrenal cortex (as well as Wilms tumor, hepatoblastoma, and other rare cancers) in the first several years of life. Other constitutional syndromes with predisposition to develop adrenocortical tumors include MEN1, familial adenomatous polyposis, and Carney complex.[10]

These tumors spread locally to the lymph nodes and can also involve the kidneys, lungs, bones, and brain.[13] Surgical removal should be attempted but may not always be possible if the tumor has spread widely. Additional treatment may include the use of an artificial hormone that blocks the masculinizing effects of the tumor [14] or chemotherapy using cisplatin, 5-fluorouracil (5-FU), and etoposide.[4,15] A retrospective analysis in Italy and Germany identified 177 patients with adrenocortical carcinoma. Recurrence-free survival was significantly prolonged by the use of adjuvant mitotane. Benefit was present with 1 to 3 grams per day of mitotane and was associated with fewer toxic side effects than doses of 3 to 5 grams per day.[16,17] The prognosis for patients who have small, completely resected tumors generally is excellent, but prognosis can be poor for patients who have large primary tumors or metastatic disease at diagnosis.[3,18] Tumor stage has been identified as a significant prognostic factor in children with adrenocortical tumors. When possible, surgical reexcision should be attempted for local tumor recurrences and for inferior vena caval tumor invasion.[19] Adrenal tumors can present as incidental findings (incidentalomas), and these tumors should be thoroughly evaluated.[20] (Refer to the PDQ summary on adult Adrenocortical Carcinoma Treatment for more information.)