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ISSN: 2081-0016
Medycyna Paliatywna/Palliative Medicine
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1/2014
vol. 6
 
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Malignant rhabdoid tumour of the liver: an unusual case report

Zeynep Z. Gümüş
,
Cem Çekiç
,
Şebnem Karasu
,
Serkan İpek
,
Sezgin Vatansever
,
Fatih Aslan
,
Sinan Akay
,
Belkis Ünsal

Medycyna Paliatywna 2014; 6(1): 68–70
Data publikacji online: 2014/04/01
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Malignant rhabdoid tumour (MRT) is a rare and aggressive childhood neoplasm, first described as an aggressive variant of Wilms’ tumour by Beckwith and Palmer in 1978 [1]. The first case, an infant, with a primary liver neoplasm was described as MRT of the liver in 1982 [2]. Although MRTs are generally seen in the kidneys, primary MRT of liver, retroperitoneum, central nervous system and other sites were reported. Extra-renal malignant rhabdoid tumors are more rare tumors than the former one. Up today less than 40 primary malignant rhabdoid tumors of liver were reported in the literature [3]. One third of the patients have high alpha-fetoprotein (AFP) levels, but two thirds of them have normal AFP levels. Although rare, it should be considered in the differential diagnosis of primary liver tumors with normal AFP.

A 67 year-old man was admitted to our clinic with a 2-month history of abdominal pain and jaundice. Phsysical examination showed hepatomegaly and ascites. The biochemical tests showed aspartate aminotransferase: 85 U/l, alanine aminotransferase: 57 U/l, alkaline phosphatase: 777 U/l, gama glutamil transferase: 1132 U/l and total bilirubin: 8.8 mg/dl. Inflammation markers were elevated (WBC: 18 110/ml and C-reactive protein: 8.75 mg/dl). An abdominal ultrasound showed the multiple heterogenous and hypodense solid lesions of the liver parenchyma and a percutaneous needle liver biopsy was performed. In addition to the masses in the liver and ascites, the abdominal and chest computed tomography (CT) showed a large number of lymphadenopathies on the retroperitoneal, mesenteric and celiac areas as well as a mass in the pancreas. Alpha fetoprotein was 1.5 ng/ml. An upper gastrointestinal endoscopy and a colonoscopy revealed normal findings.

On the immunohistochemical examination of the liver biopsy, the tumour cells stained positively with cytokeratin (AE1+ AE3+), vimentin, epithelial membrane antigen (EMA+); and negatively with CD34, CD99, CD117, CD37, CD45, Hep Par, cytokeratin 20, cytokeratin 7, desmin, TTF-1, glypican, S-100 and melan-a. With histopathological and immunohistochemical features, the patient diagnosed as malignant rhabdoid tumour.

During the clinical follow-up the patient was consulted to medical oncology and, we planned chemotherapy. He died on the 15th day of hospitilization due to multiorgan dysfunction, particularly liver and respiratory failure.

Malignant rhabdoid...


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