Carcinoma of the Adrenal Cortex
        Treatment options under clinical evaluation
Carcinoma of the Stomach
Cancer of the Pancreas
Colorectal Carcinoma
Carcinoid Tumors
Gastrointestinal Stromal Cell Tumor

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 cell tumors. The prognosis, diagnosis, classification, and treatment of these abdominal cancers are discussed below.  [Note: Refer to the Renal Cell Carcinoma section in the PDQ summary on Wilms Tumor and Other Childhood Kidney Tumors for more information.]

Adrenocortical tumors are classified as carcinomas and adenomas.[1-5] 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.[6] 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.[1,7] Children with Beckwith-Wiedemann syndrome [8] or hemihypertrophy [9] 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.

These tumors spread locally to the lymph nodes and can also involve the kidneys, lungs, bones, and brain.[10] 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[11] or chemotherapy using cisplatin, 5-fluorouracil (5-FU), and etoposide.[4,12] 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.[13,14] 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.[3,15] 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.[16] Adrenal tumors can present as incidental findings (incidentalomas), and these tumors should be thoroughly evaluated.[17] (Refer to the PDQ summary on adult Adrenocortical Carcinoma Treatment for more information.)

• ARAR0332: 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 (I) or dissection (II). Patients with stage III and stage IV disease will receive chemotherapy before resection. The chemotherapy is cisplatin, doxorubicin, etoposide, and oral mitotane.

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.[18] 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. Symptoms include vague upper abdominal pain, which can be associated with poor appetite, and weight loss. Many individuals become anemic but otherwise show no symptoms before the development of metastatic spread. Other symptoms may include nausea, vomiting, change in bowel habits, poor appetite, weakness, and Helicobacter pylori infection.[19] 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.

Treatment should include surgical excision with wide margins. For individuals who cannot have a complete surgical resection, radiation therapy may be used along with chemotherapeutic agents such as 5-FU and irinotecan.[20] Other agents that may be of value are the nitrosoureas with or without cisplatin, etoposide, doxorubicin, or mitomycin C.

Prognosis depends on the extent of the disease at the time of diagnosis and the success of treatment that is appropriate for the clinical situation.[21] Because of the rarity of stomach cancer in the pediatric age group, little information exists regarding the treatment outcomes of children. (Refer to the PDQ summary on adult Gastric Cancer Treatment for more information.)

Pancreatic tumors are rare in children and adolescents.[22] Tumors included in this general category can arise at any site within the pancreas. Cancers of the pancreas may be classified as adenocarcinomas, squamous cell carcinomas, acinic cell carcinomas, liposarcomas, lymphomas, papillary-cystic carcinomas, pancreatoblastomas, malignant insulinomas, glucagonomas, and gastrinomas.[23-25] Several cases of primitive neuroectodermal tumor of the pancreas have been reported in children and young adults.[26] Most pancreatic tumors do not secrete hormones, though some tumors secrete insulin, which can lead to symptoms of weakness, fatigue, hypoglycemia, and coma.[23,27] If the tumor interferes with the normal function of the islet cells, patients may have watery diarrhea or abnormalities of salt balance. Both carcinoma of the pancreas and pancreatoblastoma can produce active hormones and can be associated with abdominal mass, wasting, and pain.[28-30] At times, there is obstruction of the head of the pancreas, which is associated with jaundice and gastrointestinal bleeding. Elevation of alpha-fetoprotein has been seen in pancreatoblastoma.[31,32] Pancreatoblastoma is reported to be associated with Beckwith-Wiedemann syndrome and Cushing syndrome.[33,34]

Solid pseudopapillary neoplasm of the pancreas is a rare tumor of borderline malignancy that has been reported in children but more commonly occurs in young women.[35,36] Treatment consists of complete tumor resection (ideally without biopsy). Metastases may occur, but in general, prognosis is good following surgery alone.[37,38]

Diagnosis of pancreatic tumors is usually established by biopsy, using laparotomy or a minimally invasive surgery (e.g., laparoscopy). A diagnosis can be achieved only after ruling out various benign and cancerous lesions. Treatment includes various surgical procedures to remove the pancreas and duodenum or removal of part of the pancreas. Complete resection is usually possible and long-term survival is likely, though pancreatoblastoma has a high recurrence rate.[24,31] For pediatric patients, the effectiveness of radiation therapy is not known. Chemotherapy may be useful for treatment of localized or metastatic pancreatic carcinoma. The combination of cisplatin and doxorubicin has produced responses in pancreatoblastoma prior to tumor resection.[39,40] Postoperative treatment with cisplatin, doxorubicin, ifosfamide, and etoposide has also produced responses in patients with pancreatoblastoma.[41][Level of evidence: 3iiiA] Other agents that may be of value include 5-FU, streptozotocin, mitomycin C, carboplatin, gemcitabine, and irinotecan. Response rates and survival rates generally are not good. (Refer to the PDQ summary on adult Pancreatic Cancer Treatment for more information.)

Carcinoma of the large bowel is rare in the pediatric age group; it is seen in only one per 1 million persons younger than 20 years in the United States annually.[42] Forty percent to 60% of the tumors arise on the right side in children in contrast to adults who have a prevalence of tumors on the left side.[43] Colon cancer in children is often associated with a family cancer syndrome.[44,45] There is an increasing risk of colorectal carcinoma in members of families with a family history of intestinal polyposis, which can lead to the development of multiple adenomatous polyps.[46] Juvenile polyps are not associated with an increased incidence or risk of cancer.

Familial polyposis is inherited as a dominant trait, which confers a high degree of risk. Early diagnosis and surgical removal of the colon eliminate the risk of developing carcinomas of the large bowel.[47] Some colorectal carcinomas in young people, however, may be associated with a mutation of the adenomatous polyposis coli (APC) gene, which also is associated with an increased risk of brain tumors and hepatoblastoma.[48] The familial APC syndrome is caused by mutation of a gene on chromosome 5q, which normally suppresses proliferation of cells lining the intestine and later development of polyps.[49] Another tumor suppressor gene on chromosome 18 is associated with progression of polyps to malignant form. Multiple colon carcinomas have also been associated with progression of polyps to a malignant form. Multiple colon carcinomas have been associated with neurofibromatosis type I and several other rarer syndromes.[50]

The histologic types of colorectal cancer include adenocarcinomas, mucinous or colloid adenocarcinomas, signet ring adenocarcinomas, and scirrhous tumors. Most tumors in the pediatric age group are mucin-producing carcinomas,[51][Level of evidence: 3iii][52][Level of evidence: 3iiA] whereas only about 15% of adult lesions are of this histology. The tumors of younger patients with this histologic variant may be less responsive to chemotherapy. These tumors arise from the surface of the bowel, usually at the site of an adenomatous polyp. The tumor may extend into the muscle layer surrounding the bowel, or the tumor may perforate the bowel entirely and seed through the spaces around the bowel, including intra-abdominal fat, lymph nodes, liver, ovaries, and the surface of other loops of bowel. A high incidence of metastasis involving the pelvis, ovaries, or both may be present in girls.[53]

Colorectal carcinoma usually presents with symptoms related to the site of the tumor.[51][Level of evidence: 3iii] Changes in bowel habits are associated with tumors of the rectum or lower colon. Tumors of the right colon may cause more subtle symptoms but are often associated with an abdominal mass, weight loss, decreased appetite, and blood in the stool. Any tumor that causes complete obstruction of the large bowel can cause bowel perforation and spread of the tumor cells within the abdominal cavity.

Because of its rarity, colorectal carcinoma is rarely diagnosed in a pediatric patient; however, vague gastrointestinal symptoms should alert the physician to investigate this possibility. Diagnostic studies that may be of value include examination of the stool for blood, studies of liver and kidney function, measurement of carcinoembryonic antigen, and various medical imaging studies, including direct examination using colonoscopy to detect polyps in the large bowel. Other conventional radiographic studies include barium enema followed by computed tomography of the chest and bone scans.[53,54]

Most patients present with evidence of metastatic disease,[51][Level of evidence: 3iii] either as gross tumor or as microscopic deposits in lymph nodes, on the surface of the bowel, or on intra-abdominal organs.[55] Complete surgical excision should be the primary aim of the surgeon, but in most instances this is impossible; removal of large portions of tumor provides little benefit for the individuals with extensive metastatic disease. Most patients with microscopic metastatic disease generally develop gross metastatic disease, and few individuals with metastatic disease at diagnosis become long-term survivors.

Current therapy includes the use of radiation for rectal and lower-colon tumors, in conjunction with chemotherapy using 5-FU with leucovorin.[56] Other agents that may be of value include irinotecan.[51][Level of evidence: 3iiiA] No significant benefit has been determined for interferon-alpha given in conjunction with 5-FU/leucovorin.[57] (Refer to the PDQ summaries on adult Colon Cancer Treatment and Rectal Cancer Treatment for more information.)

These tumors, like bronchial adenomas, may be benign or malignant and can involve the lining of the lung or the large or small bowel.[58-62] Most lung lesions are benign; however, some metastasize.[63] Most carcinoid tumors of the appendix are discovered incidentally at the time of appendectomy, and are small, localized tumors; simple appendectomy is the therapy of choice.[64] For larger (>2 cm) tumors or tumors that have spread to local nodes, cecectomy or rarely, right hemicolectomy, is the usual treatment. It has become accepted practice to remove the entire right colon in patients with large carcinoid tumors of the appendix (>2 cm in diameter) or with tumors that have spread to the nodes; however, this practice remains controversial.[65] Treatment of metastatic carcinoid tumors of the large bowel or stomach becomes more complicated and requires treatment similar to that given for colorectal carcinoma. The carcinoid syndrome of excessive excretion of somatostatin is characterized by flushing, labile blood pressure, and metastatic spread of the tumor to the liver.[63] Symptoms may be lessened by giving somatostatin analogs, which are available in short-acting and long-acting forms.[66] (Refer to the PDQ summary on adult Gastrointestinal Carcinoid Tumors for more information.)

Gastrointestinal stromal cell tumor (GIST) is a mesenchymal tumor of the intestinal tract that typically occurs in adults older than 40 years. These tumors are rare in children. Only 1.4% of all patients with GISTs are children and young adults.[67] Many of these tumors were previously diagnosed as leiomyomas, leiomyosarcomas, and leiomyoblastomas. Younger patients with GISTs are usually female and commonly present in the second decade of life with anemia-related gastrointestinal bleeding. In children, most tumors are in the stomach and may be localized or multifocal. Carney triad is associated with gastric GIST in a small number of children and in an association with extra-adrenal paraganglioma and pulmonary chondroma. Some patients may present with GIST as the first manifestation of Carney triad and for this reason, a diagnosis of Carney triad should be considered in pediatric patients presenting with GIST.[68] A familial variant of pediatric GIST has not been established. The association of KIT mutations in adults with GIST is as high as 90%; imatinib mesylate has been found to be effective therapy in these patients. GIST in adolescents and young adults has a heterogeneous presentation; some patients have tumors with mutations in KIT or PDGFA, which suggests that these tumors may respond to imatinib mesylate.[69,70] Pediatric GIST is probably biologically different from adult GIST, since KIT and PDGFA mutations are rarely detected in pediatric GIST tumors.[71] Clinical response to targeted therapies such as imatinib mesylate has not been proven. Complete surgical resection of localized disease should be the initial treatment in pediatric GIST. No chemotherapy regimen has been effective in the treatment of GIST, and in the absence of mutations in KIT, imatinib mesylate is not recommended as adjuvant therapy. The clinical course of GIST is variable, with an excellent prognosis in children with localized disease and an indolent, slowly progressive course in children with liver and lymph node metastatic disease. Because of the variable expression of mutations in KIT or PDGFA, all patients with GISTs should have their tumors examined for these mutations.[67,69,70]

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