Rare Gastrointestinal Cancers Aren’t So Rare to Johns Hopkins Team

A multimodality approach ensures the best care possible for patients with gastrointestinal stromal tumors, small-bowel neuroendocrine tumors and intra-abdominal sarcomas.

Published in Surgery - Spring 2019

Certain gastrointestinal tumors — especially those found in the stomach and peritoneal cavity — are rare and complex, requiring a highly skilled team of specialists who collaborate to individualize multimodal treatment plans. At Johns Hopkins, experts in gastrointestinal oncology, surgery, gastroenterology, genetics and interventional radiology offer a mix of surgical options (traditional and minimally invasive), medical therapies and testing to tackle these diagnoses with precision.

Fabian Johnston, chief of gastrointestinal surgical oncology at Johns Hopkins, explains the value of this team approach regarding gastrointestinal stromal tumors (GISTs), which can occur anywhere along the gastrointestinal tract but are most common in the stomach or small intestine. “Although stromal tumors are typically benign, they can act differently in younger patients,” says Johnston. “So after we remove those tumors, our pathologists will do a molecular profile on them. And if the profile tells us the person is at increased risk of recurrence, we set up for an alternative plan to follow the patient with the oncologists.” These collaborations help inform a comprehensive regimen of care.

For small-bowel neuroendocrine tumors, Johnston and his fellow surgical oncologists may lean on interventional radiologists, medical oncologists and hepatobiliary surgeons when needed. “These carcinoid tumors could be anywhere,” says Johnston, noting that small-bowel neuroendocrine tumors occur in only about one of every 100,000 Americans. “It’s such an uncommon malignancy, and we are fortunate to have a group of amazing specialists [to] collaborate on the patient’s care.”

Intra-abdominal sarcoma resection is commonly performed by the Johns Hopkins gastrointestinal surgical oncology team. “Sarcomas make up about 1% of all tumors and, within that small number, there are over 50 different subtypes,” Johnston says. A sarcoma multidisciplinary group at Johns Hopkins includes both adult and pediatric medical oncologists, and orthopaedic oncologists. This multidisciplinary perspective ensures the best treatment for each case. “Patients come here for the care this whole team provides before, during and after surgery,” says Johnston.

Nonsurgical Treatment Options

Surgery is not always indicated for gastrointestinal tumors. In these cases, expert gastroenterologists such as Saowanee Ngamruengphong, who specializes in nonsurgical full thickness resection of tumors located deep in the wall of the gastrointestinal tract, lead the charge.

“When we have difficult cases, we work with our colleagues to come up with what’s best for the patient,” says Ngamruengphong. “At Johns Hopkins, our surgeons and our pathologists and oncologists — we all work together.”

“Several endoscopists in the Division of Gastroenterology are trained not only to diagnose diseases, but to resect tumors,” Johnston says. “The goal is always to get patients the therapy that’s right for them. If cancer can be removed without surgery, that’s great for the patient.”

Treating More Complex, Extensive Disease with HIPEC

If the disease has spread to the abdominal or peritoneal cavity, Johnston — who also serves as director of the Johns Hopkins Peritoneal Surface Malignancy Program, which includes fellow gastrointestinal surgical oncologist Jonathan Greer — offers cytoreductive surgery and hyperthermic intraperitoneal chemotherapy, or HIPEC. In this approach, all of the visible disease is removed, followed by heated intra-abdominal chemotherapy.

HIPEC helps attack tumors in ways that intravenous chemotherapy can’t perform alone, sometimes working in concert to improve the patient’s care. 

Heating the drugs, Johnston says, “agitates the walls of the cancer cells, making it easier for the chemotherapy to penetrate the tumor and to perform its cytotoxic abilities on the cancer.”

Johns Hopkins is one of only a few centers in the United States to offer HIPEC.

To discuss a patient case or to make a referral for gastrointestinal surgery, call 443-997-1508.