Rhabdoid cholangiocarcinoma: a variant of cholangiocarcinoma with aggressive behavior.
A rhabdoid cholangiocarcinoma is a very rare variant of sarcomatous cholangiocarcinomas. Here, we report a vimentin positive cholangiocarcinoma showing rhabdoid features in the entire tumor, with a very aggressive behavior. A 41-year-old woman was admitted to our hospital due to a huge hepatic mass. The resected liver revealed a 17 x 15 cm sized solid mass with extensive necrosis and an infiltrative border. On microscopic examination, the entire tumor was composed of loosely cohesive round to polygonal cells, with rhabdoid features having abundant eosinophilic glassy cytoplasm and eccentrically located vesicular nuclei. Some tumor cells contained intracytoplasmic mucin vacuoles, but definite areas of glandular differentiation or spindle cell were not found. Immunohistochemical staining showed a diffuse strong positive reaction to pan-cytokeratin and vimentin, and focal positivity for the carcinoembryonic antigen. Other immunohistochemical stainings for cytokeratin 7, cytokeratin 20, S-100 protein, HMB-45, desmin, alpha-smooth muscle actin, c-kit, CD34, alpha-fetoprotein, anti-hepatocyte antigen, chromogranin and synaptophysin were all negative. After two months, the patient developed a local recurrence along the resection margin, and multiple hematogenous metastases to the lung and liver were seen on the follow-up CT scan.
Lim
Beom Jin
BJ
Department of Pathology, Brain Korea 21 Project for Medical Science, Institute of Gastroenterology, Yonsei University College of Medicine, 134 Shinchon-dong, Seodaemun-gu, Seoul 120-752, Korea.
Kim
Kyung Sik
KS
Lim
Joon Seok
JS
Kim
Myeong Jin
MJ
Park
Chanil
C
Park
Young Nyun
YN
D000328:Adult; D018281:Cholangiocarcinoma / Q000737:chemistry / Q000473:pathology*; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000737:chemistry / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D011379:Prognosis; D018335:Rhabdoid Tumor / Q000737:chemistry / Q000473:pathology*; D014057:Tomography, X-Ray Computed; D014746:Vimentin / Q000032:analysis
D014746:Vimentin