Paola Castrejon was just two years old when her parents, Yoleveth and Pablo Cuevas, rushed her to the emergency room at Lurie Children’s for severe abdominal pain. That pain turned out to be the result of a ruptured tumor of her adrenal gland. Paola was eventually found to have stage three adrenocortical carcinoma, an extremely rare tumor which most often occurs in patients with a genetic predisposition to cancer. As it happened, Paola’s mother was simultaneously being treated at Northwestern Memorial Hospital for leukemia. Further investigation would reveal that Paola, her mother and three siblings all had this predisposition syndrome – Li-Fraumeni Syndrome.
“When Paola arrived at the ER, she was so, so sick,” says Amy Walz, MD, Paola’s primary oncologist. “Her tumor had ruptured, causing massive bleeding into her abdomen. She required emergency surgery to stop this hemorrhage.”
Pediatric surgeon Timothy Lautz,MD, was called into action and took Paola emergently to the operating room. “Paola’s initial surgery was more like an operation performed for trauma, rather than a typical cancer operation,” says Dr. Lautz. “Our first priority was to stop the bleeding. Only then were we able to remove the tumor, which had shattered into pieces.”
Paola recovered well from that operation and was started on an intense regimen of chemotherapy. Later she returned to the operating room for a meticulous cancer operation to remove any remaining tumor. Paola continued with nine months of chemotherapy and in October of 2017, she rang the “End of Chemo Bell,” signaling that she was cancer-free.
“Paola tolerated her cancer treatment very well given how ill she was when we first met her. She always had a smile on her face and brightened the day of all those who cared for her,” Dr. Walz says.
Today, there is a good chance that Paola’s tumor won’t reoccur; however, because of her chemotherapy regimen, Paola no longer has a functioning adrenal gland. She will need adrenal hormone replacement therapy for the rest of her life. Additionally, because of her Li-Fraumeni Syndrome, she will be monitored closely for the development of other tumors. Paola and her siblings are enrolled in The Cancer Predisposition Program at Lurie Children’s. “Our program is a partnership between genetics and oncology specialists. It brings their unique areas of expertise together to create a specialized risk assessment for cancer predisposition,” says Dr. Walz. “We take a personalized approach to medicine, adjusting each risk assessment to meet the unique needs of each patient and family.” The program provides comprehensive genetic counseling and testing services to pediatric oncology patients and families at risk of hereditary cancer syndromes, as well as, management guidance and recommendations to families, based on the results of personalized cancer risk assessments.
“Because of her ruptured tumor, Paola’s condition was complex,” says Dr. Walz. “Her treatment wouldn’t have been possible without the expertise of everyone on her team.”
Paola’s case is an excellent example of the multidisciplinary care offered at Lurie Children’s. Paola’s oncologist, surgeon, and endocrinologist have all worked together to provide the best possible care and outcome.