Malignant Involvement of the Porta Hepatis Lymph Nodes

Introduction

Porta hepatis is the fissure beneath the left portion of the right lobe of the liver. It transmits the lymphatic system, common bile ducts, hepatic arteries, veins, and nerves. The cancer of the lymphatic system is called lymphomas. The cancer of lymph nodes in porta hepatis has never been reported in non-transplant patients. Therefore, primary lymphomas in porta hepatis are a rare entity. Malignant (a condition in which tumors tend to progress aggressively and spread to other body parts) involvement of the porta hepatis lymph nodes is generally seen in immunocompromised patients associated with organ transplantation. Imaging techniques play an important role in diagnosing primary malignant lymphomas of porta hepatis.

What Is Primary Malignant Lymphomas of Porta Hepatis?

Porta hepatis is a deep fissure of five centimeters located beneath the left portion of the right lobe of the liver. The function of porta hepatis is to transmit the lymphatic system, common bile ducts, hepatic arteries, veins, and nerves. Lymphoma is a cancer of the lymphatic system. The malignant involvement of porta hepatis lymph nodes is seen in biliary tract diseases. The biliary system consists of the liver, gallbladder, and bile duct. The biliary tract is responsible for the transportation of the bile. The obstruction in the drainage of the lymphatic system in the biliary tract can cause primary lymphomas.

What Are the Causes of Primary Malignant Lymphomas of Porta Hepatis?

Primary malignant lymphomas of porta hepatis are a rare entity. The exact cause of primary malignant lymphomas of porta hepatis is unknown but is prevalent in patients who have undergone transplants. The liver consists of lymphoid tissue, and some host factors (such as immunocompromised conditions) trigger the development of malignant lymphoma. In addition, malignant lymphomas in porta hepatis can arise from benign and malignant biliary tract tumors.

The following are the causes of primary malignant lymphomas of porta hepatis:

• Cholangiocarcinoma (CCA) - This is an uncommon tumor that can occur anywhere in the biliary tree. It arises from the liver's right and left hepatic ducts in 50 % to 60 % of cases. Tumors of the biliary tree can invade the lymphatic system. CCA can be associated with gallstones and primary and secondary sclerosis cholangitis.

• Liver Transplantation - Primary malignant lymphomas of porta hepatis are seen in the liver transplant patient. The incidence of malignant lymphoma is about 27 % in patients with Cyclosporine immunosuppressants associated with transplant. The chances of primary malignant lymphoma increase after post-transplant surgeries.

• Benign Gallbladder Tumors - The gallbladder tumors are uncommon, often asymptomatic, and usually an unexpected find on the operation. It is associated with cholesterolosis of the gallbladder, a condition in which lipid deposition is found in the layers of the gallbladder.

• Cholecystitis - The chronic gallbladder inflammation associated with a gallstone is common among the population.

• Choledocholithiasis - The formation of stones in the common bile duct can occur in a few patients with gallstones. It can develop with a common bile duct after cholecystectomy (gallbladder removal). Common bile duct stones obstruct bile transportation and may be complicated by cholangitis, bacterial infection, and liver abscess. It obstructs the drainage of the lymphatic system resulting in malignant lymphomas of porta hepatis.

• Cholangitis - Acute cholangitis is caused by bacterial infection of the bile duct and occurs in patients with other biliary problems such as choledocholithiasis and biliary tumors.

• Choledochal Cysts - Cysts (abnormal sacs filled with fluid) can develop anywhere in the biliary tree, known as choledochal cysts. It causes diffuse dilatation of the common bile duct.

• Adenocarcinoma - The condition in which neoplasia (abnormal growth of the cells) of epithelial cells occurs. Adenocarcinoma of the liver can cause malignant lymphoma of porta hepatis.

What Are the Symptoms of Primary Malignant Lymphomas of Porta Hepatis?

The following are the symptoms of primary malignant lymphomas of porta hepatis:

• Yellowing of the eyes and skin.

• Abdominal pain.

• Weight loss.

• Fever.

• Itching.

• Dark urine.

• Chills.

• Lethargy.

• Weakness.

• Confusion.

• Shortness of breath.

• Profuse sweating.

• Headache.

• Hepatomegaly (liver enlargement).

• Mild splenomegaly (spleen enlargement).

Signs:

• Dark urine.

• Pale stools.

• Alteration in vitals.

How Are the Primary Malignant Lymphomas of Porta Hepatis Diagnosed?

The patient's history of prior infection and surgeries, such as gastric cancer surgeries, excision of cysts from the gallbladder, or history of liver transplantation, helps diagnose the cause of primary malignant lymphomas of porta hepatis. The following are the ways to diagnose primary malignant lymphomas of porta hepatis:

• Physical Examination - The liver and gallbladder mass may be palpable in the abdomen. The signs of scars on the body are noticed on physical examination.

• Blood Tests - The elevated level of bilirubin confirms jaundice. A routine blood culture helps in finding the infection if any.

• Liver Function Test (LFT) - The LFT shows signs of cholestasis. The elevated level of transaminases indicates biliary diseases.

• X-Ray - The liver and gallbladder tumor mass can be detected with the help of an X-ray. The course and the location of the tumor can be detected by X-ray.

• Ultrasound - Ultrasound of the abdomen is the most convenient method of locating the obstruction of the common bile duct.

• Computed Tomography Scan (CT Scan) - A CT scan can help detect the infection's course and location.

• Magnetic Resonance Imaging (MRI) - MRI is the investigation of choice for primary malignant lymphomas of porta hepatis. MRI provides additional information regarding post-surgical tumors. It helps in obtaining images of the lymphatic system. MRI is useful in understanding the extent of tumors.

• Cholangiography - This technique helps in obtaining images of the biliary system. It helps to detect stones in the biliary duct or any obstruction in the biliary tree.

• Liver Biopsy - Liver biopsy shows histological changes in liver cells, which is advisable in patients with large tumors.

When Should the Doctor Be Consulted?

If individuals experience severe abdominal pain and recurrent attacks of biliary pain, they should immediately contact a doctor. Primary malignant lymphomas need urgent medical attention, and the patient should be hospitalized. The illness's severity depends on the tumor size and extent.

Conclusion

Primary lymphomas of porta hepatis are extremely rare cases. It is seen in patients who have undergone liver transplants. According to studies, no cases were reported in non-transplant patients. Imaging techniques are important in treating primary malignant lymphomas of porta hepatis. Primary lymphomas of porta hepatis can be life-threatening and require urgent medical intervention. However, a cocktail of chemotherapy provides complete remission in 80% of the patients, with a 5-year survival rate from the date of diagnosis in 87 % of the cases.