anyone know much about calangio carcinoma?

Question

my aunt has it and i was just wondering shes only 33

Answer 1

Background: Cholangiocarcinomas (CCCs) are malignancies of the biliary duct system that may originate in the liver and extrahepatic bile ducts, which terminate at the ampulla of Vater. CCCs are encountered in 3 geographic regions: intrahepatic, extrahepatic (ie, perihilar), and distal extrahepatic. Perihilar tumors are the most common, and intrahepatic tumors are the least common. Perihilar tumors, also called Klatskin tumors (after Klatskin's description of them in 1965), occur at the bifurcation of right and left hepatic ducts. Distal extrahepatic tumors are located from the upper border of the pancreas to the ampulla. More than 95% of these tumors are ductal adenocarcinomas; many patients present with unresectable or metastatic disease.


Pathophysiology: Cholangiocarcinoma is a tumor that arises from the intrahepatic or extrahepatic biliary epithelium. More than 90% are adenocarcinomas, and the remainder are squamous cell tumors. The etiology of most bile duct cancers remains undetermined. Long-standing inflammation, as with primary sclerosing cholangitis (PSC) or chronic parasitic infection, has been suggested to play a role by inducing hyperplasia, cellular proliferation, and, ultimately, malignant transformation. Cholangiocarcinomas tend to grow slowly and to infiltrate the walls of the ducts, dissecting along tissue planes. Local extension occurs into the liver, porta hepatis, and regional lymph nodes of the celiac and pancreaticoduodenal chains. Life-threatening infection (cholangitis) may occur that requires immediate antibiotic intervention and aggressive biliary drainage.Medical Care:

Stenting may relieve pruritus and improve quality of life.
Palliative plastic or metal stents can be placed by ERCP or PTC to relieve biliary obstruction. They usually are used if the tumor is unresectable or if the patient is not a surgical candidate. Debate exists about whether preoperative stenting is warranted, but most surgeons believe that preoperative biliary decompression does not alter the outcome of surgery.

Plastic stents usually occlude in 3 months and require replacement.

Metal stents are more expensive but expand to a larger diameter and tend to stay patent longer. Adequate biliary drainage can be achieved in a high percentage of cases.
Photodynamic therapy (PDT) is a new experimental local cancer therapy already in use for other GI malignancies.

PDT is a 2-step process: the first step is intravenous (IV) administration of a photosensitizer; the second step is activation by light illumination at an appropriate wavelength.

PDT is effective in restoring biliary drainage and improving quality of life in patients with nonresectable disseminated cholangiocarcinomas (CCCs). Survival times may be longer than those reported previously. A recent prospective, multicenter study showed a significant survival benefit in the PDT treatment group (Ortner, 2003). An additional multicenter study is being planned.
Most often, chemotherapy is given in low doses to act as a radiation sensitizer during a 4- to 5-week course of external beam radiotherapy. Primary chemotherapy has been evaluated as well including gemcitabine and cisplatin as first-line chemotherapy in inoperable biliary tract carcinoma.

Adjuvant and preoperative radiation therapy has been used to reduce tumors in an effort to make them resectable.
This therapy has been performed with and without concurrent chemotherapy as a radiation sensitizer.

The value of adjuvant radiotherapy has been to improve local control, with variable effect on overall survival rate after complete resection. Several series have shown an increase in median survival duration with postoperative radiation, from 8 months with surgery alone to more than 19 months.

Special radiation techniques have been used, such as intraluminal brachytherapy and external beam therapy during surgery (ie, intraoperative radiotherapy [IORT]). See Image 3 for treatment planning technique.
Primary radiotherapy without surgery, with or without chemotherapy, has provided a survival advantage and significant palliation over stent placement or bypass surgery alone in patients with medially inoperable or unresectable tumors.

Chemotherapy agents used without radiotherapy or surgery do not appear to provide any local control or meaningful survival benefit.

The most used agent is 5-fluorouracil, but many agents have been tested in phase I/II trials. Partial responses, lasting from weeks to months, have been observed in 10-35% of trials (Thongprasert, 2005).

For palliative treatment, celiac-plexus block via regional injection of alcohol or other sclerosing agent can relieve pain in the mid back associated with retroperitoneal tumor growth.
Surgical Care: Complete surgical resection is the only therapy to afford a chance of cure. Unfortunately, only 10% of patients present with early stage disease and are considered for curative resection. Intrahepatic and Klatskin tumors require liver resection, which may not be an option for older patients with comorbid conditions. In a recent series report, 15% of patients with proximal lesions were candidates for complete resections, with higher rates in patients with mid ductal (33%) or distal tumors (56%). Survival rate for patients with proximal tumors can be 40% if negative margins are obtained.

Orthotopic liver transplantation is considered for some patients with proximal tumors who are not candidates for resection because of the extent of tumor spread in the liver. The largest series reports a 53% 5-year survival rate and a 38% complete pathologic response rate with preoperative radiation therapy and chemotherapy. Liver transplantation may have a survival benefit over palliative treatments, especially for patients with tumors in the initial stages. A more recent study has demonstrated a 5-year survival rate greater than 80% in select patients.
Distal tumors are resected via Whipple procedure; periampullary region tumors have a uniformly better prognosis, with a long-term survival rate of 30-40%.
Patterns of treatment failure after curative surgery show disappointingly high rates of tumor bed and regional nodal recurrence. This finding may be due in part to the narrow pathologic margins; however, regional node failure rate is approximately 50% and distal metastases rate 30-40%. Failures are correlated with TNM stage.
Palliative procedures are required if internal stenting cannot be accomplished and/or external stenting is not desirable or cannot be obtained; surgical bypass, particularly for tumors in common bile duct, should be performed.
Consultations: Gastroenterologists, interventional radiologists, and transplant/biliary surgeons play a key role in diagnosis and management. Radiation oncology and medical oncology specialists are part of the multidisciplinary team taking part in the treatment of both patients with curatively resected tumors and those with unresectable tumors. Radiation oncologists have taken a more significant role in therapy for CCC since the early 1980s.

Answer 2

Yes , Calangio is an uncle of mine who lives in Canada. A really nice person!

Answer 3

It is a cellular dysfunction that can be reversed with glyconutrient supplements.
Info on my page.

Answer 4

www.nlm.nih.gov/medlineplus/ency/article/000291.htm