Small Cell Carcinoma of the Ovary
When six year old Emily Knerr began waking at night with leg pains, her parents and pediatrician thought it was merely growing pains. Soon the leg pains were accompanied by stomach aches and weight loss. “They did blood work and said everything was fine,” said Emily’s mom, Amanda Knerr. A month later the stomach pains became so severe that the Knerrs rushed Emily to the emergency room of a hospital near their home in Ft. Lauderdale, Fla. “We thought she had appendicitis, but her tumor had actually ruptured,” Knerr said. Emily underwent surgery to remove a large tumor which the doctors diagnosed as small cell carcinoma of the ovary. The Knerrs, who were scheduled to move from Ft. Lauderdale to Birmingham in just weeks, were faced with a dilemma. “I’m from Memphis and so I wondered if we should just take her to Saint Jude’s. I asked her doctors and they told us to keep the family together and take her to Birmingham. The doctors there were just as good,” Knerr said. Ten days later the Knerrs arrived in Birmingham and the next morning they met with pediatric oncologist Dr. Joseph Pressey at Children’s of Alabama. “Small cell carcinoma of the ovary hypercalcemic type is an extremely rare tumor type that is typically found in adolescents and young adults. To our knowledge, Emily is the second youngest ever reported to have this type of tumor,” said Pressey, who recently published a medical journal article about treatments for such tumors. Surgeons in Florida had removed most of the tumor, but by the time Emily arrived at Children’s, much of it had grown back. She underwent two more surgeries as well as chemotherapy before receiving a transplant of stem cells from her own bone marrow. “She had a pretty good response to the chemotherapy, but not a complete response. Her tumor shrunk, but didn’t go away,” Pressey said. Because Emily’s tumor was so rare, there were few studies on successful treatments; however, Emily’s doctors felt that an autologous stem cell transplant was a good option. “Basically what we were doing was giving her very high doses of chemo and then rescuing her body with her own bone marrow. There were a few case reports of having done that and we thought given how extensive her tumor was, it would be worthwhile,” Pressey said. After she recovered from complications related to the transplant, Emily underwent radiation and six months of antibody therapy. Today, she is an active 10 year-old, busy with school and ballet. She returns to Children’s once a year for scans and Pressey says he is optimistic about her future. Amanda Knerr says she is grateful for the care and support they received at Children’s. “Emily actually cried on her last day of chemo because we were leaving. They took such good care of her and we became so attached to her nurses. We still go and visit them. They are an amazing group of people and they became like our family,” Knerr said. “I give Emily’s oncologist 100 percent of the credit for saving her life,” Knerr continued. “Emily’s tumor was very, very rare and has a very poor prognosis, but Dr. Pressey kept searching for studies. He took her tumor to the Tumor Board and just took it to another level. On his own time he found obscure studies of therapies that had been tried on this tumor and had kind of a success rate. He just dug deeper so he could save her, and he did,” she said.
