Sarcomatoid carcinoma of the pancreas and congenital choledochal cyst

At the Focal Point

Sarcomatoid carcinoma of the pancreas and congenital choledochal cyst

A 72-year-old woman was admitted to our hospital with abdominal pain, fever, and weight loss. Physical examination revealed tenderness in the epigastrium. Laboratory data showed a white blood cell count of 12,600 109/L (normal range, 4800-10,800 109/L) and increases in the serum levels of amylase (379 IU/L; normal !160 IU/L); aspartate aminotransferase (78 IU/L; normal 1-21 IU/L), alanine (60 IU/L; normal 1-22 IU/L), alkaline phosphatase www.giejournal.org

(1410 IU/L; normal !207 IU/L), and gamma-glutamyl transpeptidase (447 IU/L; normal !25 IU/L). US and CT revealed dilatation of the common bile duct suggestive of a choledochal cyst (type I) and pancreatic-duct dilatation. ERCP showed a large and bulging papilla and a dilated pancreatic duct with intraductal filling defects (A). Adjacent to the pancreatic duct was a distal dilatation compatible with congenital malformation in the biliary tree (B). Once the Volume 64, No. 6 : 2006 GASTROINTESTINAL ENDOSCOPY 1005

At the Focal Point

pancreatic duct was selectively cannulated, an endoscopic papillotomy was performed. With a Dormia basket and a Fogarty balloon (Wilson-Cook, Winston-Salem, SC), multiple membranes of mucinous material were obtained from the pancreatic duct (C). Biopsy specimens revealed that the tumor had a mostly sarcomatous component with spindle-shaped cells (D; left [H&E, orig. mag. 100], right [IHQ, orig. mag. 40]). Immunohistochemistry revealed some spindle tumor and anaplastic cells were positive for vimentin and cytokeratin. The patient died 9 months later; autopsy findings included a spindle-cell type sarcomatoid tumor of the pancreatic head, with hepatic metastases and a congenital cyst of the distal biliary tree.

DISCLOSURE The authors attest that they have no commercial associations that might be a conflict of interest in relation to the submitted manuscript. Susana De la Riva, MD, PhD, Miguel A. Mun˜oz-Navas, MD, PhD, Maite Bete´s, MD, PhD, Jose C. Su´btil, MD, PhD, Cristina Carretero, MD, Division of Gastroenterology; Jesus J. Sola, MD, PhD, Division of Pathology, University Clinic, University of Navarra, Pamplona, Spain Presented, in part, at Digestive Disease Week, American Gastroenterological Association Annual Meeting, Orlando, Florida, May 16-19, 1998. doi:10.1016/j.gie.2006.06.004

Commentary Sarcomatoid carcinoma is a rare and very aggressive malignant tumor that has a mixture of carcinomatous and sarcomatous elements. Tumors also may have a deceptively bland appearance and may be associated with a marked inflammatory reaction. Sarcomatous carcinomas occur in diverse locations throughout the body, including the upper respiratory tract, genitourinary tract, upper and lower digestive tracts, and the breast and thyroid glands, among others. The most feared complication of choledochal cysts, most of which occur in the type I cyst, as in the present case, is cholangiocarcinoma. It occurs with an incidence of 9% to 28%, increases with age and in the presence of anomalous union of the common bile and pancreatic ducts, and may even occur after complete surgical excision of the cyst. Early diagnosis of the cyst and early surgery allows hope to permeate an otherwise dreadful situation. Lawrence J. Brandt, MD Associate Editor for Focal Points

Microcystic serous cystadenoma mimicking a solid pancreatic tumor

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