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Journal of Gastrointestinal Surgery ISSN 1091-255X J Gastrointest SurgDOI 10.1007/s11605-016-3123-1

Giant Primary Yolk Sac Tumor of the Liver

Aleksandr A. Reznichenko, LindseyR. Klingbeil & Shimul A. Shah

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GI IMAGE

Giant Primary Yolk Sac Tumor of the Liver

Aleksandr A. Reznichenko1 & Lindsey R. Klingbeil1 & Shimul A. Shah1

Received: 19 February 2016 /Accepted: 28 February 2016# 2016 The Society for Surgery of the Alimentary Tract

Keywords Yolk sac tumor . Hepatocellular carcinoma . Liverresection

Case Presentation

A 39-year-old female with prior history of obesity and C-section developed right upper quadrant abdominal pain. Shedid not have fever, nausea and vomiting, or weight loss. Herpain was worse with deep breath but was not affected by food.She stopped taking birth control pills 14 years ago. Her BMIwas 36.75. On physical exam, she had a large palpable mass inright upper quadrant. Her labs were prominent for Alpha-fetoprotein (AFP) 34.321 and Human chorionic gonadotropin(hCG) 22. A CT of the abdomen and pelvis with contrastshowed a giant 25 cm solid heterogeneous mass in the rightlobe of the liver (Fig. 1), and additional smaller masses insegments 4a and 5 measuring 4 and 3.5 cm, respectively(Fig. 2). All of the liver masses demonstrated peripheral andseptal enhancement on the arterial and portal venous phases,which was highly suspicious for malignancy.

Patient underwent right extended hepatectomy. There wasa giant tumor in the right hepatic lobe with significant neovas-cularization of the adjoining areas, including inferior venacava and diaphragm. Large specimen was removed (Fig. 3).

Final pathology showed malignant neoplasm with markednecrosis, consistent with yolk sac tumor (YST), all margins of

resection were negative for tumor, and there were no lympho-vascular invasion. Patient made uneventful recovery and wasdischarged to home on day 7. Three weeks after the procedure,her AFP was 851, and hCG was <1. She was referred to amedical oncologist.

Primary YST tumors of the liver are rare. Only few cases ofliver yolk sac tumor in adults were reported. The histogenesisof primary YST of the liver is unclear. It has been suggestedthat YSTs might originate from a germ cell that escaped itsmigration course from the yolk sac to the genital ridge duringembryogenesis. Alternatively, the embryonic cell theory fa-vors the persistence of pluripotential embryonic cells that es-caped the influence of the differentiation process during

Fig. 1 CT abdomen with contrast, axial view, arterial phase. Largeheterogeneous mass (yellow arrow) in the right hepatic lobe

* Aleksandr A. Reznichenkoreznicaa@ucmail.uc.edu

1 Division of Transplant Surgery, Department of Surgery, University ofCincinnati, 231 Albert Sabin Way, Suite 1555,Cincinnati, OH 45267-0519, USA

J Gastrointest SurgDOI 10.1007/s11605-016-3123-1

Author's personal copy

embryogenesis in mature liver. The clinical behavior of theYST of the liver also is unclear.1 After diagnosis, the goldstandard treatment is surgery, usually followed by adjuvantchemotherapy, with a reported mortality rate greater than50 %. Although the morphologic features of hepatic YSThave been described in the literature, this rare neoplasm fre-quently poses significant diagnostic problems. The serumAFP is uniformly elevated in HCC, hepatoblastoma, andYST, which is making preoperative differential diagnosis onthe basis of serum markers quite difficult. Reported imagingfindings of YST range from solid to predominantly cysticmass with heterogeneous appearances and prominent en-hancement on contrast or post contrast CT phases, like a largenodule of HCC. The female gender, age less than 30 years,

very high levels of serum AFP, and the presence of a predom-inantly cystic mass within a non-cirrhotic liver were consid-ered suggestive of YST.2

In this case, both hepatocellular carcinoma and YST wereconsidered preoperatively in the differential diagnosis.However, based on the patient age, CT findings, size of themajor tumor, and presence of multifocal liver masses, HCCwas primarily considered.

Our case is noticeable because of the giant (25 cm) size ofthe primary liver YST. To our knowledge, this is the firstreport of multifocal YST in the liver.

References

1. Toumi N, Chaumette-Plankaert MT, Cherqui D, de Revel T,Duvillard P, Theodore C. Germ cell tumors. Case 3. Primary yolksac tumor of the liver. J Clin Oncol. 2004 May 1; 22(9):1756–8.

2. Lenci I, Tariciotti L, Baiocchi L, Manzia TM, Toti L, Craboledda P,Callea F, Angelico M, Tisone G. Primary yolk sac tumor of the liver:incidental finding in a patient transplanted for hepatocellular carci-noma. Transpl Int. 2008 Jun; 21(6):598–601.

Fig. 3 Resected specimen. Giant mass (red arrow) occupying most ofthe right hepatic lobe. Smaller mass (yellow arrow) in segment 5

Fig. 2 CT abdomen with contrast, coronal view, venous phase. Largeheterogeneous mass (yellow arrow) in the right hepatic lobe. Twoadditional smaller lesions (red arrow) in segments 4a and 5

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