Clinicopathological features and surgical outcomes of patients with fibrolamellar hepatocellular carcinoma (FL-HCC) are underreported. The aim of this study is to describe clinical characteristics and surgical outcomes for patients with this rare tumor to raise awareness among clinicians and surgeons.

Retrospective review of records of a tertiary referral center and specialized liver unit was performed. Out of 3623 patients who underwent liver resection, 366 patients received surgical treatment for HCC; of them, eight (2.2%) had FL-HCC and were resected between October 2001 and December 2018.

Eight patients (3 males and 5 females) with FL-HCC (median age 26 years) underwent primary surgical treatment. All patients presented with unspecific symptoms or were diagnosed as incidental finding. No patient had cirrhosis or other underlying liver diseases. Coincidentally, three patients (37.5%) had a thromboembolic event prior to admission. The majority of patients had BCLC stage C and UICC stage IIIB/IVA; four patients (50%) presented with lymph node metastases. The median follow-up period was 33.5 months. The 1-year survival was 71.4%, and 3-year survival was 57.1%. Median survival was at 36.4 months. Five patients (62.5%) developed recurrent disease after a median disease-free survival of 9 months. Two patients (25.0%) received re-resection.

FL-HCC is a rare differential diagnosis of liver masses in young patients. Since the prognosis is limited, patients with incidental liver tumors or lesions with suspicious features in an otherwise healthy liver should be presented at a specialized hepatobiliary unit. Thromboembolism might be an early paraneoplastic symptom and needs to be elucidated further in the context of FL-HCC.

• FL-HCC
• Fibrolamellar hepatocellular carcinoma
• Hepatocellular carcinoma
• Human
• Paraneoplastic
• Surgical outcome
• Thrombocytopenia
• Thromboembolism

Fibrolamellar carcinoma (FLC) is a variant of hepatocellular carcinoma (HCC), which comprises ∼1%–9% of all HCCs. Although FLC is a variant of HCC, it is distinct from HCC in that it most often affects younger patients (10–35 years of age) with no underlying liver disease. FLC often presents with vague abdominal pain, nausea, abdominal fullness, malaise, and weight loss. Surgery is the current mainstay of treatment for FLC and remains the only potentially curative option. While FLCs are considered less responsive to chemotherapy than their classic HCC counterparts, there have been suggestions that multimodality treatments may be effective, especially in advanced cases. Further research is necessary to determine effective systemic therapies as an adjunct to surgery for FLC.

• fibrolamellar
• hepatocellular carcinoma
• hepatocyte paraffin I
• locoregional therapy

Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare variant of hepatocellular carcinoma, usually presenting in the younger population (<40 years) without underlying liver disease. Although it has a better prognosis than hepatocellular carcinoma, it has a high rate of recurrence months to years after primary resection. While sites of recurrence usually involve the liver, regional lymph nodes, peritoneum, and lung, metastasis to the pancreas is extremely rare, with only 2 other cases reported in the literature. We present the case of a 46-year-old patient with metastatic FL-HCC to the pancreas 30 years after diagnosis and 26 years since his last resected liver recurrence.

• Fibrolamellar hepatocellular carcinoma
• Liver transplantation
• Metastasis

Adenocarcinosarcoma, a neoplasm containing both carcinomatous and sarcomatous components, is a rare form of a cancer and the pathophysiology is currently poorly understood. Moreover, definitive treatment guidelines for this disease have not yet been established. Pancreatic adenocarcinosarcoma is even more rare and the prognosis is fatal. Here, we report a case of a 77-year-old male with pancreatic adenocarcinosarcoma and metastasis to the liver. The patient presented at our hospital with uncontrolled glucose levels and diabetes mellitus. The patient’s laboratory findings were unremarkable with the exception of elevated carbohydrate antigen 19-9 levels. Biopsies of the tumors in the pancreas and the liver revealed two types of tumors: pancreatic adenocarcinoma and a poorly differentiated sarcoma. To determine if KRAS mutations were present, we performed a peptide nucleic acid (PNA) clamp PCR-based assay. DNA sequencing by PNA clamp PCR identified a point mutation in codon 12 of exon 2 within KRAS from both tumor types. Because the KRAS mutation is observed in both tumor components, our findings support a monoclonal tumor origin followed by subsequent divergent differentiation into the sarcomatous and carcinomatous tumor populations. After we considered the patient’s status and the late stage of tumor detection, gemcitabine chemotherapy was administered.

• Pancreas
• Adenocarcinosarcoma
• Metastasis
• KRAS
• DNA sequencing

• Adenocarcinoma/blood
• Adenocarcinoma/chemistry
• Adenocarcinoma/drug therapy
• Adenocarcinoma/genetics
• Adenocarcinoma/secondary
• Aged
• Antimetabolites, Antineoplastic/therapeutic use
• Biopsy
• CA-19-9 Antigen/blood
• Cell Differentiation
• DNA Mutational Analysis
• Deoxycytidine/analogs & derivatives
• Deoxycytidine/therapeutic use
• Exons
• Genetic Predisposition to Disease
• Humans
• Immunohistochemistry
• Liver Neoplasms/blood
• Liver Neoplasms/chemistry
• Liver Neoplasms/drug therapy
• Liver Neoplasms/genetics
• Liver Neoplasms/secondary
• Magnetic Resonance Imaging
• Male
• Neoplasms, Complex and Mixed/blood
• Neoplasms, Complex and Mixed/chemistry
• Neoplasms, Complex and Mixed/drug therapy
• Neoplasms, Complex and Mixed/genetics
• Neoplasms, Complex and Mixed/pathology
• Pancreatic Neoplasms/blood
• Pancreatic Neoplasms/chemistry
• Pancreatic Neoplasms/drug therapy
• Pancreatic Neoplasms/genetics
• Pancreatic Neoplasms/pathology
• Phenotype
• Point Mutation
• Proto-Oncogene Proteins/genetics
• Proto-Oncogene Proteins p21(ras)
• Sarcoma/blood
• Sarcoma/chemistry
• Sarcoma/drug therapy
• Sarcoma/genetics
• Sarcoma/secondary
• Tomography, X-Ray Computed
• Up-Regulation
• ras Proteins/genetics
• Gemcitabine