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Germ Cell Tumors

CASE 3. Primary Yolk Sac Tumor of the Liver

Institut Gustave Roussy, Villejuif; Hopital Henry Mondor, Creteil; Hopital d'Instruction des Armées, Percy, Clamart, France

A 28-year-old white female presented with a month's history of abdominal pain. She had no previous medical history and was not using oral contraceptives. She reported having lost 3 kg through dieting. She had neither fever nor gastrointestinal symptoms. Physical examination showed abdominal distension and the liver edge was palpable four finger breadths below the right costal margin. She also had bilateral pedal edema. Laboratory tests revealed normal blood cell counts, but abnormal liver function, with elevated AST and ALT two times the normal level and bilirubin three times the normal level. Serum alpha-fetoprotein (AFP) was 54,000 ng/mL, lactate dehydrogenase was 900 U/L (normal is 650 U/L), and the human chorionic gonadotropin level was normal. Serology testing was negative for viral hepatitis. Ultrasound and computed tomography (CT) depicted massive enlargement of the liver with a 137-mm large mass in the right lobe (Fig 1). There was no other tumor site. Hepatocellular carcinoma was considered the most likely diagnosis and a right hepatectomy was performed. Surgery revealed compression of the inferior vena cava by the tumor. The nontumorous liver appeared normal. Complete resection of the tumor was carried out and a tumor thrombus in the suprahepatic vein was also removed. The right lobe of the liver measured 230 mm x 170 mm. It had lost its normal capsule and appeared yellowish, homogenous, and microcystic. The tumor cells had pleomorphic nuclei with numerous mitoses, and a solid architecture. There were tumor cells around the blood vessels and areas of ischemic necrosis. Large cystic areas were observed. Periodic acid Schiff–positive globules were present within and around the tumor cells (Fig 2A, 2B) which also showed immunoreactivity for cytokeratin 8, 18, and 19, alpha-1-antitrypsine and AFP but not for cytokeratin 7, vimentin, human chorionic gonadotropin, or placental alkaline phosphatase. Antihepatocytic expression was found in 30% of the cells and expression of Mib1, a proliferation marker, was high in 40% of the counted cells. There were no hepatocellular carcinoma or hepatoblastoma features. There were numerous areas of vascular invasion in suprahepatic and portal vessels. The background liver was normal.

View larger version (121K): [in this window] [in a new window]   Fig 1.

View larger version (69K): [in this window] [in a new window]   Fig 2.

Because postoperative clinical and radiologic assessment showed no evidence of an ovarian or other extrahepatic tumor, the diagnosis of a primary YST of the liver was made. Three weeks after surgery, AFP was 3,600 ng/mL with no evidence of tumor on CT scan. The patient was treated with four cycles of bleomycin, etoposide, and cisplatin chemotherapy.³ AFP returned to 15 ng/mL (normal is 15). There was no evidence of tumor either on CT scan or at positron emission tomography. However, within 3 months, AFP increased to 134 ng/mL and pulmonary metastases appeared. She responded only transiently to salvage treatment with vinblastine, ifosfamide, and cisplatin and a third-line germ cell tumor salvage regimen with paclitaxel, epirubicin, and high-dose chemotherapy.

Eight patient cases of primary YST of the liver have been reported previously.^4–11 The first report was in 1975.⁴ Of these eight previously reported patient cases, six involved women with a median age of 27 years (range, 24 to 29 years).^5–10 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.^4,6 Alternatively the embryonic cell theory favors the persistence of pluripotential embryonic cells that escaped the influence of the differentiation process during embryogenesis in mature liver.⁷ The clinical behavior of the tumor also is unclear. One patient was reported to have responded to chemotherapy.⁸ However, all other reported patients died as a result of disease despite the use of an aggressive therapeutic strategy.^5,6,9 Our patient responded only moderately to chemotherapy. The clinical behavior of this tumor seemed similar to that of a hepatoid ovarian YST, which also is of germ cell origin. To our knowledge, the similarity between these two types of rare YST, both arising in a young women, has never before been addressed. In conclusion, better characterization of the biologic and pathologic features of this tumor is still needed as well as knowledge of its clinical behavior. This would justify registration in a rare tumor registry and a centralized pathological review.

Authors' Disclosures of Potential Conflicts of Interest

The authors indicated no potential conflicts of interest.

REFERENCES

1. Vicente D, Bernabe R, Mazo J, et al: Hepatoblastoma in an adult. Med Clin (Barc) 116:758, 2001

2. Wittekind C: Pathology of liver tumors. Zentralbl Chir 125:587–591, 2000[Medline]

3. Williams SD, Birch R, Einhorn LH, et al: Treatment of disseminated germ cell tumors with cisplatin, bleomycin and either vinblastine or etoposide. N Engl J Med 316:1435–1440, 1987[Abstract]

4. Hart WR: Primary endodermal sinus (yolk sac) tumor of the liver: First reported case. Cancer 35:1453–1458, 1975[CrossRef][Medline]

5. Narita T, Moriyama Y, Ito Y: Endodermal sinus (yolk sac) tumor of the liver: A case report and review of the literature. J Pathol 155:41–47, 1988[Medline]

6. Natori T, Teshima S, Kikuchi Y, et al: Primary yolk sac tumor of the liver, an autopsy case with ultra structural and immunohistochemical studies. Acta Pathol Jpn 33:555–564, 1983[Medline]

7. Villaschi S, Balistreri P: Endodermal sinus tumor of the liver. Histopathology 18:86–88, 1991[Medline]

8. Wheelan JS, Stebbings W, Owen RA, et al: Successful treatment of primary endodermal sinus tumor of the liver. Cancer 70:2260–2262, 1992[Medline]

9. Higuichi T, Kikuchi M: Yolk sac tumor of the liver treated with trans-catheter arterial embolization. Am J Gastroenterol 88:1125–1126, 1993[Medline]

10. Wong NA, D'Costa H, Barry RE, et al: Primary yolk sac tumor of the liver in adulthood. J Clin Pathol 51:939–940, 1998[Abstract]

11. Devouassoux-Shisheboran M, Schammel DP, Tavassoli FA: Ovarian hepatoid yolk sac tumours: Morphological, immunohistochemical and ultrastructural features. Histopathology 34:462–469, 1999[Medline]

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