Ultraschall Med 2013; 34 - KS_CS5_03
DOI: 10.1055/s-0033-1355000

Some unusual cystic lesions of the liver (case report)

AE Guthoff 1, MH Wehmeier 1, C Schramm 1, M Bester 2, A Quaas 3, AW Lohse 1
  • 1University Medical Center Eppendorf, Internal Medicine I, Hamburg, Germany
  • 2University Medical Center Hamburg-Eppendorf, Department of Radiology, Hamburg, Germany
  • 3University Medical Center Eppendorf, Department of Pathology, Hamburg, Germany

Purpose: A 70-year-old male with a history of a non-Hodgkin-lymphoma treated with radiochemotherapy 13 years ago presented himself with an asymptomatic elevation of liver enzymes.

Material and methods: Abdominal ultrasound showed multiple cystic lesions within the liver. Some of the cystic walls had an irregular and nodular appearance. On contrast ultrasound (Sonovue (Bracco), GE Logiq E9) additional multiple small areas appeared hypoechoic in the portal phase. On magnetic resonance imaging (MRI) some of the cysts featured albuminous inclusions. There were no signs of sclerosing cholangitis.

Results: Biopsies were taken from three nodular lesions during mini-laparoscopy. Histologically, cells with a convoluted nucleus staining positive for CD1a and S-100 protein on immunohistochemistry and concomitant eosinophilic granulocytes were seen.

Conclusion: The histology is characteristic for a Langerhans' cell histiocytosis (LCH). This is, exspecially in adults, a rare disease with uncertain prognosis reaching from spontaneous regression to rapid progression and death. In most cases LCH is a multiorgan disease with frequent involvement of bones, lungs, skin and lymph nodes. Singular liver involvement is rare and exists in 3 types: Infiltration of extrahepatic bile ducts, infiltration of intrahepatic bile ducts, both resulting in sclerosing cholangitis and nodular Langerhanscell tumors. These can range from minute granulomas up to several cm pseudotumorous nodules not connecting to bile ducts or portal areas. Early stages of LCH may respond to chemotherapy with vinblastin and steroids. Liver involvement in a multiorgan disease is regarded as a marker for poor prognosis.

Our patient decided to receive no treatment due to the lack of clinical symptoms and involvement of only one organ and is well 2 years after diagnosis.

Having the heterogeneity of this disease in mind, Langerhans' cell histiocytosis should be considered not only as a rare cause of secondary sclerosing cholangitis but also of liver lesions in adults.