FCF is please to announce that it has funded a new study directed by Roshni Dasgupta, MD of the Cincinnati Children’s Hospital Medical Center (CCHMC). Dr. Dasgupta is a leader in surgical oncology and the chair of the Pediatric Surgical Oncology Research Collaborative (PSORC). PSORC includes 41 member institutions and has a mission “to improve surgical outcomes for children, adolescents and young adults with cancer through multi-institutional collaborative investigation.”
She and the other co-investigators – Soo-Jin Cho, MD, PhD of the University of California, San Francisco (UCSF); Dolores López-Terrada, MD, PhD of the Texas Children’s Hospital; and Kimberly Riehle, MD of the University of Washington – will lead a project under the PSORC umbrella to address questions about the true incidence, accuracy of diagnosis, and optimal treatments for rare pediatric liver tumors, including fibrolamellar carcinoma (FLC).
Several years ago, PSORC initiated a project to understand the incidence of rare pediatric liver tumors and how best to treat them. The consortium collected and analyzed records of 262 patients under age 20 diagnosed with liver cancers, assembled from different hospitals. This represents the richest available source of data on such pediatric liver cancers. They found that 119 (45%) of the tumors had been classified as FLC, 110 (42%) as conventional HCC, and the remainder mainly as “hepatocellular neoplasms-not otherwise specified” (HcN-NOS) (12%). That work, published in May 2022, can be read here. However, the classification of each tumor in the original effort was based on the diagnosis originally made at the treating hospital. Because evidence suggests that FLC is frequently misdiagnosed, the PSORC team requested funding from FCF to carry out a centralized pathology review of the 262 tumors to confirm the actual number of FLC cases versus other types of liver cancers. They also proposed to carry out molecular analyses of the tumors using methods that were not readily available in hospital labs when the bulk of the specimens were initially assessed by pathologists.
The expanded study – “How fibrolamellar carcinoma differs from hepatocellular carcinoma in children – Creation of a multi-institutional database including central pathologic review” – will offer a refined view of clinical correlations made using the initial unverified classifications of tumors in the PSORC collection. A longer-term goal, beyond accurate diagnosis of FLC, will be to determine whether molecular analysis can offer individualized information for FLC patients about disease prognosis and optimal treatment regimens.