Tumor hepático con vías biliares normales en un paciente con síndrome de Alagille: a propósito de un caso / Liver mass containing normal bile ducts in an Alagille patient: a case report

En este artículo se informa el caso de un paciente con síndrome de Alagille, que desarrolló una lesión de crecimiento rápido en el lóbulo caudado del hígado cirrótico. Se realizó el seguimiento riguroso del tumor aunque, desde el punto de vista radiológico, no parecía ser maligno. En el estudio por resonancia magnética (RM), no se observó ningún criterio diagnóstico de carcinoma hepatocelular; no obstante, se realizó una biopsia de la lesión y del hígado debido al rápido crecimiento del tumor. Los resultados del informe anatomopatológico indicaron desarrollo normal de los conductos biliares en el tumor y escasez de conductos biliares en la segunda muestra del hígado. Describiremos este caso y propondremos una interpretación de estos resultados.

This manuscript reports a case of a patient with Alagille syndrome who developed a rapidly growing lesion in the caudate segment of his cirrhotic liver. This mass was closely monitored but did not seem malignant from a radiological point of view. An MRI showed no criteria in favour of a hepatocarcinoma, however, the rapid growth lead to a biopsy of both the lesion and the cirrhotic liver. The pathology results indicated normal development of the bile ducts in the mass and paucity of the biliary ducts in the second liver specimen. We will describe this case and propose an interpretation of these findings.

Tumor hepático con vías biliares normales en un paciente con síndrome de Alagille: a propósito de un caso / Liver mass containing normal bile ducts in an Alagille patient: a case report

En este artículo se informa el caso de un paciente con síndrome de Alagille, que desarrolló una lesión de crecimiento rápido en el lóbulo caudado del hígado cirrótico. Se realizó el seguimiento riguroso del tumor aunque, desde el punto de vista radiológico, no parecía ser maligno. En el estudio por resonancia magnética (RM), no se observó ningún criterio diagnóstico de carcinoma hepatocelular; no obstante, se realizó una biopsia de la lesión y del hígado debido al rápido crecimiento del tumor. Los resultados del informe anatomopatológico indicaron desarrollo normal de los conductos biliares en el tumor y escasez de conductos biliares en la segunda muestra del hígado. Describiremos este caso y propondremos una interpretación de estos resultados.(AU)

This manuscript reports a case of a patient with Alagille syndrome who developed a rapidly growing lesion in the caudate segment of his cirrhotic liver. This mass was closely monitored but did not seem malignant from a radiological point of view. An MRI showed no criteria in favour of a hepatocarcinoma, however, the rapid growth lead to a biopsy of both the lesion and the cirrhotic liver. The pathology results indicated normal development of the bile ducts in the mass and paucity of the biliary ducts in the second liver specimen. We will describe this case and propose an interpretation of these findings.(AU)

[Hepatocellular carcinoma originated in the caudate lobe. Surgical strategy for resection. A propos of a case]. / Hepatocarcinoma originado en el lóbulo caudado. Estrategia quirúrgica para su resección. A propósito de un caso.

BACKGROUND: Hepatocellular carcinoma originating from the caudate lobe has a worse prognosis than other hepatocellular carcinoma in another segment of the liver. An isolated caudate lobe resection of the liver represents a significant technical challenge. Caudate lobe resection can be performed along with a lobectomy or as an isolated liver resection. There are very few reports about isolated caudate lobe liver resection. We report a case of successful isolated resection of hepatocellular carcinoma in the caudate lobe with excellent long-term survival. CLINICAL CASE: A 74 years old female with 8cm mass lesion in the caudate lobe without clinical or biochemical evidence of liver cirrhosis, serum alpha-fetoprotein 3.7 U/l, and negative hepatitis serology was evaluated for surgery. Complete resection of the lesion in 270minutes with Pringle maneuver for 13minutes was satisfactorily performed. Patient was discharged ten days after surgery without complications. Patient is currently asymptomatic, without deterioration of liver function and 48 month tumor free survival after the procedure. CONCLUSION: Isolated caudate lobe resection is an uncommon but technically possible procedure. In order to achieve a successful resection, one must have a detailed knowledge of complete liver anatomy. Tumor free margins must be obtained to provide long survival for these patients who have a malignancy in this anatomic location.