Case Study: MSK’s Approach to Complex Pediatric Surgical Oncology

Case Study: MSK’s Approach to Complex Pediatric Surgical Oncology

Pediatric surgical oncologist Enrico Danzer, MD, and colorectal surgical oncologist J.Joshua Smith, MD, PhD, FACS, collaborate on pediatric cases that require complex surgeries more often performed in adults, allowing the care team to offer specialized care.

The American College of Surgeons recently designated Memorial Sloan Kettering Cancer Center (MSK) the first-ever Children’s Surgery Verification (CSV) Quality Improvement Program Level I Specialty Children’s Surgery Center in Oncology. The distinction recognizes MSK as a highly specialized hospital providing exceptional surgical care that matches children’s needs with optimal pediatric resources.

MSK surgeons have extensive experience performing surgeries to treat patients with common and rare tumors. They perform annually about 2,500 procedures on patients under the age of 18 and about 800 procedures on young adult patients with pediatric cancers across 14 surgical subspecialties.

One of the ways that set MSK’s approach apart is pairing pediatric surgeons with adult surgeons on diagnoses, treatment planning, procedures, and patient follow-ups for pediatric patients who require complex surgeries more often performed in adults. MSK Kids and MSK are under one roof, making collaboration fast and seamless, and allowing pediatric and adult subspecialty experts to join the care team as needed.

In this Q&A with pediatric surgical oncologist Enrico Danzer, MD, and colorectal surgical oncologist J. Joshua Smith, MD, PhD, FACS, we discuss the complex case of a teen with a rare rectal cancer and ulcerative colitis and discover how the surgeons worked together to provide the patient with the best chance for a positive outcome.

A Complex Case Study: Rectal Cancer With Ulcerative Colitis

The 14-year-old patient was referred to you after a rectal tumor was found on a routine colonoscopy for ulcerative colitis. How did he present?

Dr. Danzer: The patient had a rare, high-grade, small cell neuroendocrine tumor (NET) in the rectum, which is very uncommon in pediatric patients and has a high risk of recurrence. His comorbid ulcerative colitis history was a complicating factor. He was diagnosed with ulcerative colitis at the age of 4 and underwent years of routine surveillance endoscopies and treatment with a variety of immunomodulatory drugs, including methotrexate, prednisone, infliximab, and ustekinumab.

Why did you reach out to Dr. Smith to collaborate?

Dr. Danzer: Imaging showed the rectal tumor was very close to the anal verge, raising the question of whether it could be resected with a sufficient negative margin without interfering with anal sphincter function, which would likely render the patient incontinent.

Which other MSK specialists did you consult with?

Dr. Smith: The team included pediatric oncologist Julia Glade Bender, MD, an expert in treating adolescents and young adults with rare tumors; medical oncologist Nitya Raj, MD, who has expertise in treating adults with gastrointestinal NETs and colorectal cancer; and pediatric gastroenterologist Stanley Cho, MD, an expert in pediatric gastroenterology and hepatology.

What issues did you weigh when determining the optimal surgical plan?

Dr. Smith: The oncologic goals were to control local disease and reduce the chance of metastasis to the liver or the lung.

Dr. Danzer: At the same time, we aimed to reduce the risk of incontinence, sexual dysfunction, and fertility issues.

What was the treatment plan?

Dr. Danzer: The first step was treatment with neoadjuvant chemotherapy to control local disease. On Dr. Raj’s recommendation, the patient received six cycles of FOLFIRINOX, an adult chemotherapy regimen containing 5-fluorouracil, irinotecan, and oxaliplatin. We ruled out treatment with radiation therapy given the high risk of urinary and sexual dysfunction based on the tumor location.

Dr. Smith: We determined a total proctocolectomy with end ileostomy was the best solution.  In consultations with the team, we ruled out reconstruction options such as taking part of the colon and reconnecting it lower down to the rectum or even the anus, given the patient’s ulcerative colitis history and tumor location.

How did the patient and the family react to the treatment plan presented?

Dr. Smith: We had a long discussion about how this plan would give their son the safest and best possible oncologic and functional outcomes. The team recognized that total removal of the colon, rectum, and anal sphincter complex, and having an end ileostomy was a lot to consider for a 14-year-old. However, it would address his immediate and longer-term cancer risk and eliminate the need for constant surveillance endoscopy.

Dr. Danzer: Most of the conversation revolved around managing expectations. They wanted to know if chemotherapy and surgery would work, the odds of the cancer coming back, and how life would change after surgery, and the patient’s prognosis. We also discussed the risks of sexual and urinary dysfunction and potential fertility issues, which was also a lot for a 14-year-old and his parents to consider. They decided to go ahead, as they understood the long-term risk of cancer recurrence was much higher than the potential adverse effects of the treatment plan.

You used a minimally invasive laparoscopic approach with a small incision below the patient’s belly button. How did the surgery go?

Dr. Smith: Our skill sets are complementary: I perform rectal resections on adult patients and Dr. Danzer performs complex bowel and laparoscopic surgeries on pediatric patients. Combining our expertise was the best way to optimize the outcome in this complex case.

Dr. Danzer: It was very easy to combine our expertise while developing the treatment plan and in the operating room. I could not have done it without Dr. Smith, and he could not have done it without me.

How is the patient doing?

Dr. Smith: The last time I saw him, he was vibrant, back to his regular activities, and his most recent abdominal MRI and chest CT scans were all clear. From a surgical perspective, all margins were clear of tumor and the lymph nodes were all negative. The patient bounced back quite quickly and has adapted well to the stoma. The main part of his early recovery was in his head. Once he realized he was through the major surgery and would be okay, he took off – literally! Dr. Danzer and I now alternate follow-up visits every six months.

Dr. Danzer: The patient initially had some activity restrictions until the stoma healed. After that, there were no restrictions for sports or even wrestling with his siblings. He has a lot of self-esteem, which has helped him adjust to having an ileostomy bag. He has a protective cover to reduce the risk of the bag coming off during sports. His urinary and sexual functions are normal. He will need surveillance imaging for a while, but for now, there’s a good chance this approach was curative. 

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