Fibrolamellar hepatocellular carcinoma presenting as cholestatic jaundice: An unusual presentation of a rare disease

Fibrolamellar hepatocellular carcinoma is a rare primary hepatic tumor that usually occurs in youth. The common presenting features are vague abdominal pain, nausea, vomiting and weight loss. We present a case report of a young male who presented with cholestatic jaundice and on evaluation was diagnosed to have fibrolamellar hepatocellular carcinoma. He underwent successful surgical resection of the tumor. In young individuals presenting with unexplained cholestasis, fibrolamellar hepatocellular carcinoma should be considered.

Clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 cases

OBJECTIVES: Fibrolamellar hepatocellular carcinoma is a rare primary malignant liver tumor that differs from conventional hepatocellular carcinoma in several aspects. The aim of this study was to describe the clinical, surgical and histopathological features of fibrolamellar hepatocellular carcinoma and to analyze the factors associated with survival. METHODS: We identified 21 patients with histopathologically diagnosed fibrolamellar hepatocellular carcinoma over a 22-year period. Clinical information was collected from medical records and biopsies, and surgical specimens were reviewed. RESULTS: The median age at diagnosis was 20 years. Most patients were female (67%) and did not have associated chronic liver disease. Most patients had a single nodule, and the median tumor size was 120 mm. Vascular invasion was present in 31% of patients, and extra-hepatic metastases were present in 53%. Fourteen patients underwent surgery as the first-line therapy, three received chemotherapy, and four received palliative care. Eighteen patients had “pure fibrolamellar hepatocellular carcinoma,” whereas three had a distinct area of conventional hepatocellular carcinoma and were classified as having “mixed fibrolamellar hepatocellular carcinoma.” The median overall survival was 36 months. The presence of “mixed fibrolamellar hepatocellular carcinoma” and macrovascular invasion were predictors of poor survival. Vascular invasion was associated with an increased risk of recurrence in patients who underwent surgery. CONCLUSION: Fibrolamellar hepatocellular carcinoma was more common in young female patients without chronic liver disease. Surgery was the first therapeutic option to achieve disease control, even in advanced cases. Vascular invasion was a risk factor for tumor recurrence. The presence of macrovascular invasion and areas of conventional hepatocellular carcinoma were directly related to poor survival. .

Hepatocarcinoma fibrolamelar un tumor de adultos jóvenes poco frecuente: Reporte de un caso / Case Report of Fibrolamellar Hepatocarcinoma, a Rare Tumor of Young Adults

El hepatocarcinoma fibrolamelar (CHC-FL) es una rara variante del carcinoma hepatocelular (CHC), se presenta con mayor frecuencia en adultos jóvenes, sin distinción de sexo, se desarrolla en su manera más característica sobre un hígado previamente sano. La manifestación clásica está dada por la triada de masa palpable en hipocondrio derecho, dolor y pérdida de peso. Ante la ausencia de historia de enfermedad hepática previa, es usual que se encuentre como una neoplasia avanzada de gran tamaño, la cual tiene hallazgos característicos en la tomografía o en la RM, que permiten en un alto porcentaje de casos hacer el diagnóstico solo con las imágenes. En este tipo de neoplasias, el mejor tratamiento es la resección quirúrgica, que proporciona una sobrevida a 5 años de 58-82%, pero con unas tasas de recaídas muy elevadas, que puede variar según las series entre 33-100% de los casos. Presentamos el caso de un paciente joven, sin historia de enfermedad hepática, en el que se diagnosticó un CHC-FL mediante el uso de resonancia magnética con medio de contraste órgano-específico y que es tratado con resección quirúrgica radical.

Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare variant of hepatocellular carcinoma (HCC) which occurs most often in young adults without regard to sex. It develops in its characteristic way on a previously healthy liver. The classical presentation is a palpable mass in the right upper quadrant, pain, and weight loss. In the absence of a prior history of liver disease, it is usual to find a large advanced neoplasia which has characteristic findings in a CT scan or MRI. A high percentage of cases can be diagnosed solely with images. The best treatment for this type of tumor is surgical resection which provides a 5-year survival rate of 58% to 82% but with very high rates of relapse. In published studies rates of relapse vary between 33% and 100%. We report the case of a young patient with no history of liver disease in which fibrolamellar hepatocellular carcinoma was diagnosed using contrast organ-specific MRI. The tumor was treated with radical surgical resection.

Diagnóstico, tratamento e acompanhamento do carcinoma hepatocelular variante fibrolamelar / Diagnosis, treatment and monitoring of hepatocellular carcinoma variant fibrolamellar

Objetivos: descrever um caso de carcinoma hepatocelular variante fibrolamelar, uma patologia pouco comum e com tratamento de escolha ainda controverso. Descrição do caso: aqui relatamos o caso de um paciente masculino de 45 anos, com diagnóstico de carcinoma hepatocelular variante fibrolamelar que foi submetido à hepatectomia parcial com ressecção tumoral, e encontra-se momentaneamente livre de doença após o seguimento de um ano. Conclusão: a variante fibrolamelar do carcinoma hepatocelular acomete geralmente em pacientes jovens, com fígado não-cirrótico, apresentando crescimento tumoral indolente e com melhores taxas de cura em comparação à forma clássica do carcinoma hepatocelular.

Aim: to describe a case of hepatocellular carcinoma fibrolamellar variant, an unusual pathology and treatment of choice remains controversial. Case description: here we report the case of a male patient of 45 years diagnosed with hepatocellular carcinoma variant fibrolamellar who underwent hepatectomy with tumor resection and is momentarily free of disease after one year follow up. Conclusion: fibrolamellar variant of hepatocellular carcinoma usually affects young patients with non-cirrhotic liver and has indolent tumor growth and better cure rates in comparison to the classic form of hepatocellular carcinoma.

A case of fibrolamellar hepatocellular carcinoma in an old-age woman / 대한내과학회지

Fibrolamellar hepatocellular carcinoma (FLHCC) is an entiry distinct from ordinary hepatocellular carcinoma (HCC) and is very rare in oriental countries. In contrast to the ordinary type of HCC, FLHCC is predominantly a disease of young adults and shows no sex predilection. In addition, it is rarely associated with positive hepatitis-B surface antigen, chronic liver disease, and a high level of serum alpha-fetoprotein (AFP). FLHCC has a high resectability rate than that of ordinary HCC, because of its occurrence in adolescents and the lack of underlying disease. Here, we report a case of FLHCC observed in a 78 year old woman. She had a dyspepsia for one month and was older than average-age patient with FLHCC, but had many characteristics of FLHCC. The tumor was removed by right anterior and left medial segmentectomy, and diagnosed as FLHCC.