A Sporadic Case of Advanced Metastatic Cholangiocarcinoma in a Child

Abstract

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.

Cholangiocarcinoma Among Children and Adolescents

“…There were reports of 22 individual cases of pediatric CCA published between 1946 and January 2016; the cases are summarized in Tables 2 and 3(21–41). The median age of diagnosis was 15 years (range 3–18 years).…”

Cholangiocarcinoma in a Child with Progressive Abdominal Distension and Secondary Hypercalcemia

“…The overall incidence rate is 0.0036 per 100,000 [ 14 ]. The youngest patient who had been reported was an 11-year-old male patient demonstrating cholangiocarcinoma associated with congenital biliary dilatation [ 10 ] presented with sporadic advanced metastatic stage at initial diagnosis [ 15 ]. Childhood cholangiocarcinoma patients are highly associated with congenital malformation of the bile duct system [ 10 ] or primary sclerosing cholangitis [ 11 , 12 ], particularly in patients under age 20.…”

Review on Pediatric Malignant Focal Liver Lesions with Imaging Evaluation: Part I

“…In advanced stages of CCA with metastatic lesions, a PET/CT scan from the skull to thigh may show numerous hypermetabolic and nonmetabolic pulmonary lesions as well as multiple hypermetabolic hepatic lesions [131].…”