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.[58,59,60,61] Treatment consists of complete tumor resection (ideally without biopsy). Metastases may occur, but in general, prognosis is good following surgery alone.[47,62,63]; [Level of evidence: 3iiA]; [Level of evidence: 3iiDi]; [Level of evidence: 3iiDiii]
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, although pancreatoblastoma has a high recurrence rate.[44,55]; [Level of evidence: 3iiA] 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.[68,69] Postoperative treatment with cisplatin, doxorubicin, ifosfamide, and etoposide has also produced responses in patients with pancreatoblastoma, although surgery is the mainstay of therapy.; [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 one per 1 million persons younger than 20 years in the United States annually, and fewer than 100 cases are diagnosed in children each year in the United States. From 1973 to 2006, the SEER database recorded 174 cases of colorectal cancer in patients younger than 19 years.
In children, 40% to 60% of tumors arise on the right side of the colon, in contrast to adults who have a prevalence of tumors on the left side. Most reports also suggest that children present with more advanced disease and have a worse outcome.[71,73,74,75,76,77,78,79,80,81,82,83,84,85]