Case Presentation: Hepatic carcinosarcoma (HCS) is an extremely rare kind of hepatic malignancy with elements of both carcinoma and sarcoma. Chronic lymphocytic leukemia (CLL), on the other hand, is the most common lymphoid malignancy in North America accounting for ~25% of leukemia cases in the United States. This is the first case report in medical literature describing the concurrent occurrence of these two malignancies in the liver.Our patient is a 60-year-old Hispanic male with a history of non-cirrhotic hepatitis C (diagnosed in 2011, treated with Ledipasvir/Sofosbuvir and had sustained undetectable viral load since 2016) as well as indolent Rai Stage 0 CLL with poor prognostic marker of ATM gene mutation on 11q22.3 (diagnosed in 2019 and has been under observation ever since). He presented to the hospital due to worsening diffuse abdominal discomfort associated with anorexia and 40-pound weight loss over 3 months. Physical exam revealed cachexia, jaundice and hepatosplenomegaly. Diagnostics showed a baseline WBC of 18/nL, absolute lymphocyte count of 15/nL, hemoglobin of 11.2 g/dL, normal platelet count, elevated INR of 2.2, aPTT of 40 seconds, AST of 86, normal ALT, albumin of 2.2 g/dL and normal ALP. Viral hepatitis panel was negative. CT imaging of chest, abdomen and pelvis showed multiple heterogeneous ill-defined hepatic masses involving nearly the entire right hepatic lobe measuring 12 cm in largest dimension, prominent peri-gastric lymph nodes suggestive of metastasis and a heterogeneous midline mass near the pancreas measuring 10 cm in largest dimension. Liver biopsy surprisingly exhibited concurrent HCS and CLL. The patient was not a surgical nor radiation therapy candidate due to extensive disease. He refused systemic chemotherapy and opted for comfort care. He passed away 2 months later.
Discussion: HCS has only been described in 31 cases in current literature. The exact pathophysiology is unknown. However, it is postulated to arise de novo or from a cirrhotic liver due to chronic hepatitis B or C. Our patient had non-cirrhotic hepatitis C for 5 years before he had undetectable disease after treatment. According to Goto et al, despite attaining sustained undetectable viral load in non-cirrhotic hepatitis C patients, the risk of having hepatocellular carcinoma (HCC) remains at 0.3 per 100 patient years. Unfortunately, the risk of developing HCS from chronic hepatitis C is unknown due to its rarity.CLL patients have a higher risk of developing second malignancies including solid cancers (skin cancer, Kaposi sarcoma, lung cancer, melanoma, breast cancer and prostate cancer) and hematologic cancers (diffuse large B-cell lymphoma, Hodgkin lymphoma and myeloid neoplasia). This is likely attributable to the postulated defective immune surveillance in CLL. Liver biopsy is not part of the basic work-up for CLL but it can be used to investigate acute abnormalities of the liver. According to Hampel et al, CLL infiltration of the liver does not necessarily change the management of the disease and is not a criterion to start treatment.
Conclusions: Our case demonstrates the presence of two simultaneous malignancies in the liver likely due to the compounded risk of a history of chronic albeit treated hepatitis C and poor immune surveillance from CLL. Currently, there have been no studies of cancer screening in CLL patients. However, patients are encouraged to have age-appropriate screening. In the future, it may be worthwhile to consider early cancer screening for these patients with such high-risk features.