How are metastatic pediatric colorectal tumors treated?

Updated: Jun 06, 2020
  • Author: Jaime Shalkow, MD, FACS; Chief Editor: Cameron K Tebbi, MD  more...
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Metastatic disease occurs in half of patients with CRC. Metastasis can spread through hematogenous, lymphatic, transcoelomic, endoluminal, or contiguity routes, and it may occur in lymph nodes, liver, lung, peritoneum, brain, and bone. Synchronous presentation at diagnosis occurs in 21% of patients and is associated with worse survival than metachronous disease.

Although most cases of metastatic CRC are incurable, combination chemotherapy with improved surgical techniques and radiation therapy plays a role in prolonging survival and reducing cancer-related symptoms in the context of extensive metastatic disease. [2]  

The staging work-up for metastatic disease includes CT of the chest, abdomen, and pelvis and consideration of positron emission tomography (PET)/CT for selected patients with indeterminate findings. Routine laboratory work-up, measurement of carcinoembryonic antigen (CEA) level, and determination of RAS and BRAF status at the primary tumor should be performed. Biopsy of metastases may be useful in the setting of first recurrence or in the case of indeterminate imaging findings.

A multidisciplinary evaluation is of critical importance for most patients with metastatic CRC, because the choice and order of therapies differ depending on presentation, number of sites and location of metastases, and potential for surgical resection. [2]

Research is focused on antiangiogenic agents, antibodies against EGFR (eg, cetuximab), and sequential lines of therapy. Median survival for patients with stage IV disease now exceeds 2.5 years.

Immune checkpoint inhibitors are a novel class of antineoplastic drugs that promote T-cell activation to overcome immune evasion of cancer cells. Other novel agents include monoclonal antibodies to block the programmed cell death protein. This class of therapeutics has shown promising activity for CRC with microsatellite instability, but not for microsatellite stable tumors.

Patients with type 2 diabetes who are being treated with metformin and in whom CRC develops have lower morbidity and better tumor-free survival than patients who are not receiving metformin. [68]

Tumor debulking offers little survival benefit for patients with extensive metastatic disease. [44]  However, in the case of a limited burden of cancer spread, surgical resection of metastatic deposits may result in a longer disease-free interval or even a cure in a minority of cases. [3]  

Metastasis limited to the liver, lung, or peritoneum may be operable. Resection of peritoneal disease is often performed in conjunction with heated intraperitoneal chemotherapy (HIPEC), although the added value of this therapy compared with surgery alone remains uncertain.

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