Carcinoma of the proximal bile duct.

Carcinoma of the proximal bile duct, while an uncommon disease, can present diagnostic and therapeutic challenges. Although frequently discovered as a small tumor, the disease is usually histologically advanced with microscopic progression along the biliary system, perineural invasion and occult involvement of regional lymphatics. Because of this behavior, treatment, while perhaps macroscopically complete, may not be curative. However, important issues need to be considered. Whenever possible, provided the patient is an acceptable operative risk, histologic confirmation of carcinoma of the bile duct should be made as other, sometimes benign, causes may mimic this disease. Preoperative assessment using contrast visualization of ductal and vascular anatomy should be undertaken to aid in intraoperative decisions regarding resectability, thereby selecting, as well as possible, patients who will benefit from potentially radical and hazardous surgical treatment. Palliation, in patients who are unresectable, should be performed either by transtumoral intubation or bypass. Adjuvant radiotherapy, while not clearly beneficial, should probably still be attempted in patients with residual disease. Chemotherapy may also be used either alone or in combination with radiation. The long term survival period is poor, but may be extended in those patients in whom complete excision of carcinoma can be accomplished.