1. 1 23
Journal of Gastrointestinal Surgery
ISSN 1091-255X
J Gastrointest Surg
DOI 10.1007/s11605-016-3123-1
Giant Primary Yolk Sac Tumor of the Liver
Aleksandr A. Reznichenko, Lindsey
R. Klingbeil & Shimul A. Shah
2. 1 23
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3. 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 . Liver
resection
Case Presentation
A 39-year-old female with prior history of obesity and C-
section developed right upper quadrant abdominal pain. She
did not have fever, nausea and vomiting, or weight loss. Her
pain was worse with deep breath but was not affected by food.
She stopped taking birth control pills 14 years ago. Her BMI
was 36.75. On physical exam, she had a large palpable mass in
right 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 contrast
showed a giant 25 cm solid heterogeneous mass in the right
lobe of the liver (Fig. 1), and additional smaller masses in
segments 4a and 5 measuring 4 and 3.5 cm, respectively
(Fig. 2). All of the liver masses demonstrated peripheral and
septal enhancement on the arterial and portal venous phases,
which was highly suspicious for malignancy.
Patient underwent right extended hepatectomy. There was
a giant tumor in the right hepatic lobe with significant neovas-
cularization of the adjoining areas, including inferior vena
cava and diaphragm. Large specimen was removed (Fig. 3).
Final pathology showed malignant neoplasm with marked
necrosis, 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 was
discharged to home on day 7. Three weeks after the procedure,
her AFP was 851, and hCG was <1. She was referred to a
medical oncologist.
Primary YST tumors of the liver are rare. Only few cases of
liver yolk sac tumor in adults were reported. The histogenesis
of primary YST of the liver is unclear. It has been suggested
that YSTs might originate from a germ cell that escaped its
migration course from the yolk sac to the genital ridge during
embryogenesis. 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. Large
heterogeneous mass (yellow arrow) in the right hepatic lobe
* Aleksandr A. Reznichenko
reznicaa@ucmail.uc.edu
1
Division of Transplant Surgery, Department of Surgery, University of
Cincinnati, 231 Albert Sabin Way, Suite 1555,
Cincinnati, OH 45267-0519, USA
J Gastrointest Surg
DOI 10.1007/s11605-016-3123-1
Author's personal copy
4. embryogenesis in mature liver. The clinical behavior of the
YST of the liver also is unclear.1
After diagnosis, the gold
standard treatment is surgery, usually followed by adjuvant
chemotherapy, with a reported mortality rate greater than
50 %. Although the morphologic features of hepatic YST
have been described in the literature, this rare neoplasm fre-
quently poses significant diagnostic problems. The serum
AFP is uniformly elevated in HCC, hepatoblastoma, and
YST, which is making preoperative differential diagnosis on
the basis of serum markers quite difficult. Reported imaging
findings of YST range from solid to predominantly cystic
mass with heterogeneous appearances and prominent en-
hancement on contrast or post contrast CT phases, like a large
nodule 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 were
considered preoperatively in the differential diagnosis.
However, based on the patient age, CT findings, size of the
major tumor, and presence of multifocal liver masses, HCC
was primarily considered.
Our case is noticeable because of the giant (25 cm) size of
the primary liver YST. To our knowledge, this is the first
report 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 yolk
sac 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 of
the right hepatic lobe. Smaller mass (yellow arrow) in segment 5
Fig. 2 CT abdomen with contrast, coronal view, venous phase. Large
heterogeneous mass (yellow arrow) in the right hepatic lobe. Two
additional smaller lesions (red arrow) in segments 4a and 5
J Gastrointest Surg
Author's personal copy