Further Outpatient Care
Most patients with cholangiocarcinoma require follow-up care for acute and late adverse effects of therapy. Aggressive follow-up care also is necessary to treat symptoms from tumor recurrence and persistence. Patients with the best prognosis may be seen every 2-3 months with periodic laboratory and imaging studies (eg, CT scan).
Patients treated palliatively should enter hospice programs rapidly, as median survival duration is only 2-8 months.
Complications
Complications include the following:
-
Infection of the biliary tree (ie, cholangitis) may result from cholangiocarcinoma and subsequent obstruction of the duct.
-
Cirrhosis develops in 10-20% of patients with cholangiocarcinoma. This may be secondary biliary cirrhosis resulting from neoplastic obstruction of the bile ducts or related to underlying fibrosis from primary sclerosing cholangitis.
-
Other complications are usually the result of diagnostic and therapeutic procedures.
Prognosis
Patients with perihilar tumors that are completely resected may achieve long-term survival. Prognosis is poorest for patients with intrahepatic tumors.
Patients with distal extrahepatic tumors may have the best hope for survival if tumors are excised completely; tumors at this site are the most likely to be resectable. These patients may experience a 5-year survival rate as high as 40%. The median survival duration in patients who undergo resection and postoperative chemoradiation may be as high as 17-27.5 months. A study by Polistina et al found that chemoradiation given by stereotactic body radiotherapy plus gemcitabine offers high local control rates and is a promising treatment. [50]
An intermediate prognosis (ie, median survival duration of 7-17 mo) is achieved for patients who are unable to undergo resection but can tolerate adjuvant chemoradiation or possibly photodynamic therapy.
The poorest prognosis is for the patient with unresectable disease, with or without overt metastatic disease, who can tolerate only palliative stent placement.
In a study of surgically resected hilar cholangiocarcinoma specimens, the presence of necrosis was associated with a worse prognosis. Necrosis was evident in 19 of 47 tumor samples. Compared with patients whose tumors showed no necrosis, those whose tumors showed necrosis had significantly lower 5-year recurrence-free survival (37.9% vs. 25.7%) and 5-year overall survival (42.6% vs.12.4%). [51]
A study by Ghafoori et al found that patients with locally advanced extrahepatic cholangiocarcinoma have poor survival with rare long-term survival. Most patients treated with external beam radiation therapy (EBRT) had local control at the time of death, which suggests that symptoms related to the local tumor effect may be controlled using radiation therapy. The authors concluded that novel approaches are indicated in the therapy for this condition. [52]
A study of prognostic scores in 219 patients with unresectable perihilar cholangiocarcinoma concluded that the modified Glasgow Prognostic Score (mGPS) [53] and the neutrophil-to-lymphocyte ratio (NLR) [54] each have prognostic value, but the platelet-to-lymphocyte ratio and Prognostic Nutritional Index do not. In addition, the combination of mGPS and NLR stratified survival well: mean survival time was 12.8 months in patients with an mGPS of 0 and an NLR of 1 or 2, but was only 3.0 months in patients with an mGPS of 1 or 2 and an NLR of 2. [55]
-
Bismuth classification for perihilar cholangiocarcinoma. Shaded areas represent tumor location.
-
Tight stricture of a common hepatic duct in a patient presenting with jaundice. Cytologic studies confirmed cholangiocarcinoma.
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Three-dimensional treatment planning uses CT scan slices to reconstruct the patient as a volume. Shown here is the display for planning external-beam radiotherapy to the cholangiocarcinoma (green structure). A biliary catheter (red tube) runs through the tumor volume and was used to deliver brachytherapy, which was given in addition to external-beam radiotherapy. Such technology has assisted greatly in the delivery of high doses to the tumor, while sparing vital normal structures, such as the kidney and spinal cord.
Tables
T - Primary tumor |
|||
TX |
Primary tumor cannot be assessed |
||
T0 |
No evidence of primary tumor |
||
Tis |
Carcinoma in situ (intraductal tumor) |
||
T1 |
Solitary tumor without vascular invasion, ≤5 cm or > 5 cm |
||
T1a |
Solitary tumor ≤5 cm without vascular invasion |
||
T1b |
Solitary tumor > 5 cm without vascular invasion |
||
T2 |
Solitary tumor with intrahepatic vascular invasion, or multiple tumors with or without vascular invasion |
||
T3 |
Tumor perforating the visceral peritoneum |
||
T4 |
Tumor involving local extrahepatic structures by direct invasion |
||
N – Regional lymph nodes |
|||
NX |
Regional lymph nodes cannot be assessed |
||
N0 |
No regional lymph node metastasis |
||
N1 |
Regional lymph node metastasis present |
||
M – Distant metastasis |
|||
M0 |
No distant metastasis |
||
M1 |
Distant metastasis present |
||
T - Primary tumor |
|||
TX |
Primary tumor cannot be assessed |
||
T0 |
No evidence of primary tumor |
||
Tis |
Carcinoma in situ – high-grade dysplasia |
||
T1 |
Tumor confined to the bile duct, with extension up to the muscle layer or fibrous tissue |
||
T2 |
Tumor invades beyond the wall of the bile duct to surrounding adipose tissue, or tumor invades adjacent hepatic parenchyma |
||
T2a |
Tumor invades beyond the wall of the bile duct to surrounding adipose tissue |
||
T2b |
Tumor invades adjacent hepatic parenchyma |
||
T3 |
Tumor invades unilateral branches of the portal vein or hepatic artery |
||
T4 |
Tumor invades main portal vein or its branches bilaterally, or the common hepatic artery; or unilateral second-order biliary radicals bilaterally with contralateral portal vein or hepatic artery involvement |
||
N – Regional lymph nodes |
|||
NX |
Regional lymph nodes cannot be assessed |
||
N0 |
No regional lymph node metastasis |
||
N1 |
One to three positive lymph nodes typically involving the hilar, cystic duct, common bile duct, hepatic artery, posterior pancreatoduodenal, and portal vein lymph nodes |
||
N2 |
Four or more positive lymph nodes from the sites described for N1 |
||
M – Distant metastasis |
|||
M0 |
No distant metastasis |
||
M1 |
Distant metastasis |
||
T - Primary tumor |
|
TX |
Primary tumor cannot be assessed |
T0 |
No evidence of primary tumor |
Tis |
Carcinoma in situ – high-grade dysplasia |
T1 |
Tumor invades the bile duct wall with a depth less than 5 m |
T2 |
Tumor invades the bile duct wall with a depth of 5–12 m |
T3 |
Tumor invades the bile duct wall with a depth greater than 12 mm |
T4 |
Tumor involves the celiac axis, superior mesenteric artery, and/or common hepatic artery |
N – Regional lymph nodes |
|
NX |
Regional lymph nodes cannot be assessed |
N0 |
No regional lymph node metastasis |
N1 |
Metastasis in 1-3 regional lymph nodes |
N2 |
Metastasis in ≥4 regional lymph nodes |
M – Distant metastasis |
|
M0 |
No distant metastasis |
M1 |
Distant metastasis |
Intrahepatic bile duct tumor |
|||
Stage |
T |
N |
M |
0 |
Tis |
N0 |
M0 |
IA |
T1a |
N0 |
M0 |
IB |
T1b |
N0 |
M0 |
II |
T2 |
N0 |
M0 |
IIIA |
T3 |
N0 |
M0 |
IIIB |
T4 |
N0 |
M0 |
|
Any T |
N1 |
M0 |
IV |
Any T |
Any N |
M1 |
Perihilar bile duct tumor |
|||
Stage |
T |
N |
M |
0 |
Tis |
N0 |
M0 |
I |
T1 |
N0 |
M0 |
II |
T2a-b |
N0 |
M0 |
IIIA |
T3 |
N0 |
M0 |
IIIB |
T4 |
N0 |
M0 |
IIIC |
Any T |
N1 |
M0 |
IVA |
T4 |
N2 |
M0 |
IVB |
Any T |
Any N |
M1 |
Distal bile duct tumor |
|||
Stage |
T |
N |
M |
0 |
Tis |
N0 |
M0 |
I |
T1 |
N0 |
M0 |
IIA |
T1 |
N1 |
M0 |
|
T2 |
N0 |
M0 |
IIB |
T2 |
N1 |
M0 |
|
T3 |
N0 |
M0 |
|
T3 |
N1 |
M0 |
IIIA |
T1-3 |
N2 |
M0 |
IIIB |
T4 |
N0-2 |
M0 |
IV |
Any T |
Any N |
M1 |
