Pancreatic Case Study

CR underwent a battery of blood, stool and imaging tests that revealed the teen had a pancreatic solid pseudopapillary tumor, an uncommon condition typically discovered in females in the third decade of life. Fortunately, this condition has a 90% surgical cure rate if treated early. A biopsy confirmed CR’s tumor was benign, but we determined it needed to be removed ASAP to relieve CR’s symptoms and prevent further growth.

Surgical tumor removal

CT scan of CR, a 13-year-old female. Axial and coronal images from the initial contrast enhanced CT of the abdomen demonstrates a large heterogeneous mass centered in the right upper quadrant displacing the duodenum likely arising from the pancreatic head. Surrounding vessels are patent, but there is mild bile duct dilation. A week after diagnosis, CR underwent a partial pancreatectomy at CHOP to remove the tumor and part of the pancreas. The procedure was complex because of the tumor’s large size, its location and that it was partly fused to the surface of the pancreas near its neck. The procedure took nearly 8 hours and when complete, about 75% of CR’s pancreas had been removed, along with her gall bladder and a portion of her liver. As anticipated, the surgery resulted in pancreatic insufficiency. CR would most likely require lifelong medication to support her body’s digestion.

CR recovered in the hospital for 4 weeks before discharge, then returned to school and typical activities a few weeks later. Follow-up appointments with gastroenterology and nutrition ensured CR was consuming the correct combination of foods to fuel her body and activities; and that she was receiving enough pancreatic enzyme replacement therapy to help digest foods so they could be absorbed. This, in particular, applied to CR’s ability to digest and absorb dietary fat, fat-soluble vitamins and other nutrients.

Value of ongoing surveillance

CR, a 17-year-old female on surveillance imaging had an MRI of the abdomen. Axial T2-weighted images show two hyperintense round lesions in the liver representing metastatic lesions not seen previously. CR returned to CHOP for biannual checkups for more than four years. She continued pancreatic enzyme replacement therapy with meals and snacks, and reported intermittent epigastric abdominal pain, which ranged from moderate to severe in intensity. Epigastric abdominal pain could be caused by several factors, including anatomical issues, infectious issues, inflammatory issues and motility disorders, among others. In this case, post-surgical anatomical abnormalities such as adhesions, maldigestion and malabsorption seemed more likely than other typical causes of abdominal pain in the general population. While tumor recurrence rates were low (less than 10%), we felt ongoing surveillance remained important. We pursued follow-up blood tests, stool tests and an ultrasound.

We subsequently discovered potential hepatobiliary tumor markers and identified new concerning lesions – this time on her liver and duodenum. A biopsy confirmed the cells were the same type that were surgically removed from her pancreas years before – tumor cells. Though the cells tested benign, given how large (and how quickly) her previous tumor had grown, we determined surgical resection of two liver nodules and a resection of the retroperitoneal nodule was required.

CR’s second surgery was successfully performed a week later. This time, her recovery was quicker — 5 days in the hospital and 6 days at home. Two weeks after surgery, CR felt well enough to attend her senior prom.

Outcome

CR, a 21-year-old female, recently had surveillance imaging after resection of the liver lesions. Axial T1-weighted image of the abdomen shows no recurrent mass in the surgical bed but there is atrophy of the pancreas with fatty replacement in the distal body and tail (curved arrow). Normally, the pancreas should be larger and brighter in signal. Four years post-op from her second surgery, CR is doing well. She continues pancreatic enzyme replacement therapy with meals and snacks. She has a good appetite and maintains a healthy weight for her height. She returns to CHOP every six months for ongoing surveillance for possible tumor recurrence, to monitor her digestive/exocrine pancreatic insufficiency, and to adjust her medication and vitamin supplementation levels as needed. Recently, CR underwent an abdominal ultrasound, an MRI with and without contrast, and a DEXA bone density scan. Scans showed normal growth and no evidence of metastasis or disease recurrence.

Discussion

Though pancreatic tumors are rare in pediatric patients, they do still occur and require a skilled and knowledgeable team to correctly identify the condition and determine the best treatment for the individual patient. Fat malabsorption increases the risk of essential fatty acid deficiency and fat-soluble vitamin deficiency, which — while often subtle and asymptomatic — can increase the risk for long term consequences. Therefore, surveillance, early identification and appropriate treatment remain of paramount importance.

Specialists at CHOP’s Pancreatic Disorders Program provide comprehensive, multidisciplinary care for children with acute, recurrent and chronic pancreatitis, as well as evaluation and management of digestive/ exocrine pancreatic insufficiency and its consequences.

Our team is actively involved in research to develop innovative, new tests and treatments for pancreatic disorders more safely and effectively. Two recent examples:

• a dietary supplement that enhances fat and fat-soluble vitamin absorption even without the use of pancreatic enzyme replacement therapy
• a novel malabsorption blood test

Our program is one of only a few in the nation accredited by the National Pancreas Foundation as both a Center for Clinical Excellence and Academic Excellence for the treatment of pediatric pancreatitis. Key collaborations with other departments at CHOP and with industry leaders like the North American Society for Pediatric Gastroenterology, Hepatlogy and Nutrition (NASPGHAN), the INternational Study Group of Pediatric Pancreatitis: In Search for a CuRE (INSPPIRE) and the National Pancreas Foundation allow us to share our learnings and build upon the latest research to help more pediatric patients with pancreatic disorders.

Asim Maqbool, MD, is a pediatric gastroenterologist and Director of the Pancreatic Disorders Program at CHOP. Sudha A, Anupindi, MD, FSAR, is a pediatric radiologist and Chief of the Division of Body Imaging at CHOP. Pablo Laje, MD, is an attending pediatric surgeon in the Division of General, Thoracic and Fetal Surgery at CHOP.