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Unusual Cancers of Childhood Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Abdominal Cancers

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The use of radiation therapy in pediatric patients with adrenocortical tumors has not been consistently investigated. Adrenocortical tumors are generally considered to be radioresistant. Furthermore, because many children with adrenocortical tumors carry germline TP53 mutations that predispose to cancer, radiation may increase the incidence of secondary tumors. One study reported three of five long-term survivors of pediatric adrenocortical tumors died of secondary sarcoma that arose within the radiation field.[33]

(Refer to the PDQ summary on adult Adrenocortical Carcinoma Treatment for more information.)

Treatment options under clinical evaluation

The following is an example of a national and/or institutional clinical trial that is currently being conducted. Information about ongoing clinical trials is available from the NCI Web site.

  • COG-ARAR0332 (Cisplatin-Based Chemotherapy and/or Surgery in Treating Young Patients With Adrenocortical Tumor): This Children's Oncology Group trial is evaluating the treatment of adrenocortical tumors with surgery and lymph node dissection. Patients with advanced disease will receive multiagent chemotherapy. Patients with stage I or stage II disease will have resection and retroperitoneal lymph node sampling (stage I) or dissection (stage II). Patients with stage III and stage IV disease will receive chemotherapy before resection. The chemotherapy regimen is cisplatin, doxorubicin, etoposide, and oral mitotane.

Carcinoma of the Stomach

Primary gastric tumors in children are rare, and carcinoma of the stomach is even more unusual.[34] In one series, gastric cancer in children younger than 18 years accounted for 0.11% of all gastric cancer cases seen over an 18-year period.[35] The frequency and death rate from stomach cancer has declined worldwide for the past 50 years with the introduction of food preservation practices such as refrigeration.[36]

The tumor must be distinguished from other conditions such as non-Hodgkin lymphoma, malignant carcinoid, leiomyosarcoma, and various benign conditions or tumors of the stomach.[34] Symptoms include vague upper abdominal pain, which can be associated with poor appetite and weight loss. Other symptoms may include nausea, vomiting, change in bowel habits, poor appetite, weakness, and Helicobacter pylori infection.[35,37] Many individuals become anemic but otherwise show no symptoms before the development of metastatic spread. Fiberoptic endoscopy can be used to visualize the tumor or to take a biopsy sample to confirm the diagnosis. Confirmation can also involve an x-ray examination of the upper gastrointestinal tract.

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