Pseudocirrhosis is a condition of the liver that closely resembles true cirrhosis in its appearance and some symptoms, but it arises from different underlying mechanisms. Distinguishing pseudocirrhosis from true cirrhosis is important because their causes, progression, and management strategies can differ significantly.
Understanding Pseudocirrhosis
Pseudocirrhosis refers to changes in the liver’s morphology that mimic the nodularity and irregular surface typical of cirrhosis, yet without the extensive, irreversible scarring and architectural distortion characteristic of true cirrhosis. In true cirrhosis, chronic inflammation and widespread fibrosis destroy the liver’s normal structure, forming regenerative nodules and bridging fibrosis. Pseudocirrhosis often involves hepatic capsular retraction, liver nodularity, and changes in liver volume, such as segmental volume loss and caudate lobe enlargement, visible on imaging. These changes can create a physical appearance and some symptoms similar to cirrhosis, particularly those related to increased pressure in the portal vein system, known as portal hypertension.
Pseudocirrhosis can arise from different pathological processes, such as the liver’s response to certain treatments or the presence of metastatic disease. Nodular regenerative hyperplasia, where regenerative nodules form and can compress liver tissue, has been associated with pseudocirrhosis. Desmoplastic reactions, which involve the development of fibrous tissue near areas of tumor infiltration, are also linked.
Underlying Causes
Pseudocirrhosis primarily develops in the setting of metastatic malignancy, particularly when the liver is involved. The most common association is with metastatic breast cancer, especially after chemotherapy. It has also been reported in other cancers that spread to the liver, including pancreatic, colorectal, gastric, esophageal, lung, ovarian, and medullary thyroid cancers.
The mechanisms leading to pseudocirrhosis are multifactorial. One cause is the response of hepatic tumors to chemotherapy, which can result in scarring and retraction of the liver capsule as the tumors shrink, leading to a nodular contour. Another mechanism involves extensive fibrosis, representing a desmoplastic response to an infiltrating tumor, even before chemotherapy. Nodular regenerative hyperplasia can also occur in response to chemotherapy-induced hepatic injury, contributing to the cirrhotic-like appearance.
Recognizing the Signs
Individuals with pseudocirrhosis may experience signs and symptoms that frequently overlap with those of true cirrhosis. These manifestations are a consequence of the liver’s reaction to the underlying cause rather than extensive, widespread scarring. A common symptom is ascites, the accumulation of fluid in the abdomen, reported in about 50% of cases. This fluid buildup often necessitates interventions like drainage.
Symptoms related to portal hypertension are also common. These can include splenomegaly, an enlarged spleen, and the development of esophageal varices, which are enlarged veins in the esophagus that carry a risk of bleeding. Other general symptoms that may occur include fatigue, unexplained weight loss, and jaundice, the yellowing of the skin and eyes. These symptoms reflect the liver’s compromised function and altered blood flow within it.
Diagnosis and Management
Diagnosing pseudocirrhosis involves a combination of clinical assessment and specific medical tests, aiming to differentiate it from true cirrhosis. A detailed medical history, especially concerning any prior cancer diagnoses and treatments, is important. Physical examination may reveal signs of fluid retention, such as abdominal distension or leg swelling, and jaundice. Blood tests, including liver function tests, help evaluate the liver’s performance.
Imaging studies are particularly useful in diagnosing pseudocirrhosis. Computed tomography (CT), ultrasound, and magnetic resonance imaging (MRI) scans of the abdomen are frequently used to visualize the liver. These scans can show characteristic changes like nodular liver surface contour, capsular retraction, loss of liver volume in certain segments, and enlargement of the caudate lobe, all mimicking true cirrhosis. While liver biopsy is sometimes performed, its role in pseudocirrhosis diagnosis may differ from true cirrhosis, as it helps confirm the absence of bridging fibrosis or may show nodular regenerative hyperplasia or desmoplastic fibrosis.
Management of pseudocirrhosis primarily focuses on addressing the underlying cause and alleviating symptoms. For patients with cancer, adjusting or continuing chemotherapy regimens to manage the primary malignancy is often central, especially if pseudocirrhosis develops due to chemotherapy. Symptomatic management includes diuretics to reduce fluid buildup from ascites and medications to manage portal hypertension and its complications, such as variceal bleeding. The prognosis for individuals with pseudocirrhosis is closely linked to the progression of their underlying condition, particularly the metastatic cancer. Early recognition and appropriate management are important for improving patient outcomes and quality of life.