S2593 Where’s the Pressure? A Case of Nodular Regenerative Hyperplasia and Non-Cirrhotic Portal Hypertension

BACKGROUND/AIMSNodular regenerative hyperplasia of the liver is a histological lesion usually associated with systemic diseases, haematological malignancies, or drugs. Its prognosis depends on portal hypertension, which usually is well tolerated and requires medical management only.PATIENTSThree unrelated families, in which two sibling adult male patients presented with nodular regenerative hyperplasia of the liver, were studied.METHODSComplete clinical charts and liver biopsy specimens were available for all patients. In addition, explanted livers were available for examination for the two transplanted patients.RESULTSThere was no evidence of any of the various clinical situations known to be associated with nodular regenerative hyperplasia of the liver. Portal hypertension was severe, requiring surgical treatment in two cases. Renal lesions were present in three patients. In two patients, progressive evolution to liver atrophy and hepatic failure, associated with renal failure, led to combined liver and renal transplantation.CONCLUSIONSThis report describes the existence of familial cases of nodular regenerative hyperplasia of the liver, occurring without underlying or associated systemic disease, characterised by a poor clinical course and often associated with progressive renal failure.

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Portal Hypertension, Nodular Regenerative Hyperplasia of the Liver, and Obstructive Portal Venopathy due to Metastatic Breast Cancer

Case Reports in Pathology ◽

10.1155/2013/826284 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Andrew T. Turk ◽

Matthias J. Szabolcs ◽

Jay H. Lefkowitch

Keyword(s):

Breast Cancer ◽

Portal Hypertension ◽

Breast Carcinoma ◽

Portal Vein ◽

Metastatic Breast ◽

Nodular Regenerative Hyperplasia ◽

Metastatic Breast Carcinoma ◽

Noncirrhotic Portal Hypertension ◽

Diagnostic Difficulties ◽

Recurrent Breast

Nodular regenerative hyperplasia (NRH) of the liver is associated with noncirrhotic portal hypertension, rheumatologic and hematologic disorders, administration of certain drugs, and other underlying conditions. This report describes a 64-year-old man with clinically presumed cirrhosis who presented to our institution with coffee-ground emesis, esophageal varices, ascites, and encephalopathy. Eleven years earlier he had been treated for breast cancer with mastectomy and chemo-radiotherapy. He died suddenly, and the autopsy showed no evidence of cirrhosis but instead demonstrated NRH with extensive emboli of recurrent breast carcinoma within the portal vein and its intrahepatic branches. Neoplastic occlusion of the portal vein as a cause of presinusoidal noncirrhotic portal hypertension has not previously been reported for metastatic breast carcinoma. This case highlights the importance of obstructive portal venopathy in the pathogenesis of NRH as well as the diagnostic difficulties that may be encountered in determining the cause of portal hypertension.

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