CLINICAL
AND
LABORATORY OBSERVATIONS
A Sporadic Case of Advanced Metastatic Cholangiocarcinoma in a Child: A Case Report and Review of Literature Connor Hall, BS,*w Viviane Mamlok, MD,z and Ihsan Al-Khalil, MDy
Summary: Cholangiocarcinoma (CC) is a malignancy of the biliary tract that commonly presents in the seventh decade of life, but is extremely rare in children. We report on a sporadic case of malignant CC in an 11-year-old female. Originally presenting with dyspnea, chest x-ray identified multiple pulmonary metastases, and various imaging modalities revealed diffuse disease spread throughout the liver and lungs. Following pathologic diagnosis of CC, aggressive treatment was initiated with a dramatic initial response to chemotherapy, however, as often occurs with advanced disease in adults, this patient ultimately succumbed to the disease. Key Words: cholangiocarcinoma, liver tumor, metastasis, dyspnea, pediatric
(J Pediatr Hematol Oncol 2015;37:e333–e335)
C
holangiocarcinoma (CC) is a malignancy of the biliary tract representing 3% of all gastrointestinal tract cancers and is the second most common primary hepatic tumor across all ages.1 The typical age at diagnosis of CC is in the seventh decade of life and rarely occurs before the age of 402; however, there are reports of CC developing in patients younger than 20 years of age. Typically, these pediatric cases present with a known underlying risk factor. Most frequently, CC in the pediatric population is associated with congenital biliary dilatation (CBD) which is prevalent in Asian populations.3,4 In addition, other factors linked to pediatric CC include small duct sclerosing cholangitis,5 chronic cholestasis caused by mutations in ABCB11, a bile export pump,6 and following liver transplant.7 Other welldocumented risk factors associated with adult CC, but not reported in pediatric patients include age, primary sclerosing cholangitis, chronic intraductal gallstones, bile duct adenoma and biliary papillomatosis, choleductal (bile duct) cysts and Caroli disease (intrahepatic biliary cysts), exposure to thorotrast, liver flukes (Opisthorcis viverrini and Clonorchis sinensis), and chronic typhoid carriage.8 In addition, other probable risk factors include cirrhosis, obesity, diabetes, fatty liver disease, alcohol, smoking, inflammatory bowel disease, and toxins (dioxins, nitrosomaines, and vinyl chloride).2,8 Despite our knowledge of known and probable risk factors for CC, they are only Received for publication April 30, 2014; accepted February 26, 2015. From the *Cancer Center; Departments of wPharmacology and Neuroscience; zPathology; and yPediatrics, School of Medicine, Texas Tech University Health Sciences Center, Lubbock, TX. The authors declare no conflict of interest. Reprints: Ihsan Al-Khalil, MD, Department of Pediatrics, School of Medicine, Texas Tech University Health Sciences Center, 3601 4th Street STOP 9903, Lubbock, TX 79430-9903 (e-mail: Ihsan. [email protected]). Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
J Pediatr Hematol Oncol
Volume 37, Number 5, July 2015
noted in 70% of all cases. At presentation, our patient had advanced metastatic disease present in the liver, lungs, and lymph nodes. Prognosis for CC patients with advanced metastatic disease is very poor with
AND
LABORATORY OBSERVATIONS
A Sporadic Case of Advanced Metastatic Cholangiocarcinoma in a Child: A Case Report and Review of Literature Connor Hall, BS,*w Viviane Mamlok, MD,z and Ihsan Al-Khalil, MDy
Summary: Cholangiocarcinoma (CC) is a malignancy of the biliary tract that commonly presents in the seventh decade of life, but is extremely rare in children. We report on a sporadic case of malignant CC in an 11-year-old female. Originally presenting with dyspnea, chest x-ray identified multiple pulmonary metastases, and various imaging modalities revealed diffuse disease spread throughout the liver and lungs. Following pathologic diagnosis of CC, aggressive treatment was initiated with a dramatic initial response to chemotherapy, however, as often occurs with advanced disease in adults, this patient ultimately succumbed to the disease. Key Words: cholangiocarcinoma, liver tumor, metastasis, dyspnea, pediatric
(J Pediatr Hematol Oncol 2015;37:e333–e335)
C
holangiocarcinoma (CC) is a malignancy of the biliary tract representing 3% of all gastrointestinal tract cancers and is the second most common primary hepatic tumor across all ages.1 The typical age at diagnosis of CC is in the seventh decade of life and rarely occurs before the age of 402; however, there are reports of CC developing in patients younger than 20 years of age. Typically, these pediatric cases present with a known underlying risk factor. Most frequently, CC in the pediatric population is associated with congenital biliary dilatation (CBD) which is prevalent in Asian populations.3,4 In addition, other factors linked to pediatric CC include small duct sclerosing cholangitis,5 chronic cholestasis caused by mutations in ABCB11, a bile export pump,6 and following liver transplant.7 Other welldocumented risk factors associated with adult CC, but not reported in pediatric patients include age, primary sclerosing cholangitis, chronic intraductal gallstones, bile duct adenoma and biliary papillomatosis, choleductal (bile duct) cysts and Caroli disease (intrahepatic biliary cysts), exposure to thorotrast, liver flukes (Opisthorcis viverrini and Clonorchis sinensis), and chronic typhoid carriage.8 In addition, other probable risk factors include cirrhosis, obesity, diabetes, fatty liver disease, alcohol, smoking, inflammatory bowel disease, and toxins (dioxins, nitrosomaines, and vinyl chloride).2,8 Despite our knowledge of known and probable risk factors for CC, they are only Received for publication April 30, 2014; accepted February 26, 2015. From the *Cancer Center; Departments of wPharmacology and Neuroscience; zPathology; and yPediatrics, School of Medicine, Texas Tech University Health Sciences Center, Lubbock, TX. The authors declare no conflict of interest. Reprints: Ihsan Al-Khalil, MD, Department of Pediatrics, School of Medicine, Texas Tech University Health Sciences Center, 3601 4th Street STOP 9903, Lubbock, TX 79430-9903 (e-mail: Ihsan. [email protected]). Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
J Pediatr Hematol Oncol
Volume 37, Number 5, July 2015
noted in 70% of all cases. At presentation, our patient had advanced metastatic disease present in the liver, lungs, and lymph nodes. Prognosis for CC patients with advanced metastatic disease is very poor with
