Unusual presentation of a distal cholangiocarcinoma

Abstract

Key Clinical Message

We report a case of a 51‐year‐old male presented with abdominal pain and itching, whereas jaundice occurred afterwards. Diagnosis was made by means of CT and better assessed by MRI and endoscopic ultrasounds. Patient was treated with an open Whipple's procedure, and after an 18‐month follow‐up he was doing well.

A 51‐year‐old male was admitted for a mild abdominal pain localized in the upper right side quadrant, without any positive Murphy's sign, associated to a drug‐resistant increasing itching, started 4 days before. Laboratory values were normal except for a light cholestasis and cytolysis (conjugated bilirubin 1.4 mg/dL [normal range 0–0.3], γGT 98U/L [0–50], AST 115U/L [0–42], ALT 137U/L [0–50]); hepatitis markers were negative. Abdominal ultrasound showed an acalculous cholecystitis associated to a choledochal dilatation without a clear evidence of obstruction. Early diagnosis was realized by a contrast‐enhanced computed tomography (CT) that highlighted a suspect solid mass of the distal choledochal duct (Fig. ) with an upper dilatation (12 mm) and multiple celiac adenopathies. No distant metastases were found. The biliary tree obstruction features were deeply investigated by means of magnetic resonance cholangiopancreatography and endoscopic ultrasound scan (Figs. and ). The patient underwent open cephalic pancreaticoduodenectomy according to Whipple's procedure, which resulted in a complete tumor removal. Postoperative course was uneventful and the patient was discharged on the 12th postoperative day. Histological examination showed a moderately differentiated (3 cm in its greatest diameter) cholangiocarcinoma (pT2N0M0, stage II), with a well differentiated intraluminal portion, no lymph nodes were involved by metastases. Adjuvant chemotherapy (3 high‐dose cycles of 5‐fluorouracil and cisplatin) and radiotherapy (50 Gy) were performed. After an 18‐month follow‐up, the patient was doing well and free from any local recurrence or distant metastases.

Contrast‐enhanced CT with bidimensional reconstruction showed a suspect intraluminal obstruction with the upper dilatation (12 mm) of the choledochal duct (arrow).

Magnetic resonance cholangiopancreatography indicated a round polypoid mass in the distal biliary tract and confirmed that choledochal duct was ectasic (arrow).

Endoscopic ultrasound scan: hypoechoic findings of the distal biliary tract tumor (arrow).

Cholangiocarcinoma is a malignancy arising from the epithelium of the bile ducts, from the hepatic parenchyma to the ampulla of Vater. Extrahepatic cholangiocarcinoma is less common than the perihilar variety and originates in the distal part of the biliary tree . Accurate preoperative localization by integrated imaging techniques is helpful for surgical management . Jaundice occurs, as first symptom, in more than 90% of all biliary tumors, and generally, it is followed by itching due to biliary salts accumulation and high stimulation of nervous fibers of dermal–epidermal junction . In the present case, we hypothesize itching related to a hypersensitivity of the skin nervous fibers. In conclusion, a prolonged itching, resistant to drugs and without jaundice, can be also considered as an early symptom of cholangiocarcinoma.

Acknowledgment

For the accurate revision of the manuscript, we wish to thank S. Palella, a native speaker, with an extensive experience on scientific papers.

Conflict of Interest

None declared.