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INTRODUCTION

Choriocarcinoma is a germ cell tumor belonging to the spectrum of gestational trophoblastic diseases [1,2]. It is characterized by wide and rapid hematogenous metastases, usually to the lungs and vagi-na [3]. It mostly occurs in the gonads and less frequently in extrag-

onadal locations, including the mediastinum, retroperitoneum, stomach, or liver as a primary tumor. Primary choriocarcinoma of the mediastinum or stomach is more frequently reported than pri-mary hepatic choriocarcinoma. To our knowledge, nine case reports of primary hepatic choriocarcinoma have been published in inter-national journals [4-8]. In this case report, we describe a patient with primary liver choriocarcinoma and lung metastases.

CASE REPORT

A 59-year-old woman was referred to our clinic with a history of upper right quadrant pain lasting 1 month. The patient’s medical history was unremarkable. Computed tomography (CT) revealed a large hypervascular mass in the right liver (Fig. 1). Magnetic reso-nance imaging revealed large masses with tiny daughter nodules in the right lobe of the liver, characteristic of angiosarcoma (Fig. 2). Cervicovaginal smear was negative for intraepithelial lesions and malignancy. Blood tests on admission revealed a low hemoglobin level (6.5 g/dL).

Case Report

Korean Journal of Clinical Oncology 2015;11:33-36http://dx.doi.org/10.14216/kjco.15007pISSN 1738-8082 ∙ eISSN 2288-4084

여성환자에서 발생한 일차성 간내 융모암 Makhmud Malikov1*,a, 신 은2*, 조재영1, 한호성1, 윤유석1, 최영록1, 장재율1, 최한림1, 장재성1, 권성욱1, 김혜령2

서울대학교 의과대학 분당서울대학교병원 1외과, 2병리과

Primary hepatic choriocarcinoma in a female patientMakhmud Malikov1*,a, Eun Shin2*, Jai Young Cho1, Ho-Seong Han1, You-Seok Yoon1, Young Rok Choi1, Jae Yool Jang1, Hanlim Choi1, Jae Seong Jang1, Seong Uk Kwon1, Haeryoung Kim2

Departments of 1Surgery and 2Pathology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea

Choriocarcinoma is a germ cell tumor belonging to the spectrum of gestational trophoblastic diseases, and occurs most commonly in the go-nads. Primary hepatic choriocarcinoma is very rare, especially in females. A 59-year-old woman was referred to our clinic with a history of upper right quadrant pain lasting 1 month. Computed tomography and magnetic resonance imaging revealed a large hypervascular mass in the right liver, characteristic of angiosarcoma. The patient underwent right hemihepatectomy without unexpected events. Histopathology re-vealed the tumor as a choriocarcinoma, with numerous syncytiotrophoblasts. Immunohistochemistry was positive for β-human chorionic go-nadotropin and CK-19, with a Ki-67 labeling index of 90%. The tumor showed focal positivity for CD31 and human placental lactogen. The patient started chemotherapy (vincristine and cyclophosphamide) on postoperative day 16. The patient’s clinical condition improved and she was discharged on postoperative day 17. Primary hepatic choriocarcinoma in female adults is a rare aggressive tumor, and further studies are needed to improve its diagnosis and treatment.

Keywords: Hepatic choriocarcinoma, Choriocarcinoma, hepatic

Received: Jun 2, 2015 Accepted: Jun 25, 2015*These two authors contributed equally to this study. aCurrent affiliation: Republic Research Center of Emergency Medicine, Tashkent, Uzbekistan

Correspondence to: Jai Young ChoDepartment of Surgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, 82 Gumi-ro 173beon-gil, Bundang-gu, Seongnam 463-707, KoreaTel: +82-31-787-7098, Fax: +82-31-787-40578E-mail: jychogs@gmail.com

Copyright © Korean Society of Clinical OncologyThis is an Open Access article distributed under the terms of the Creative Commons Attri-bution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

34 Korean Journal of Clinical Oncology

The patient underwent right hepatectomy (Fig. 3). At surgery, the tumor was attached to the peritoneum but was easily dissected without leaving residual tumor tissue. The patient recovered with-out immediate postoperative complications. However, on postoper-ative day 6, the patient developed vaginal spotting. Pancreaticobili-ary CT revealed no significant abnormality, but several pulmonary nodules were found, probably representing pulmonary metastasis. Chest CT revealed multiple round nodules in both lungs, most likely metastases. The nodules were well defined, of variable size, and were randomly distributed. On postoperative day 12, positron-emis-sion tomography confirmed the presence of multiple metastatic nodules in both lungs. Histopathology revealed the tumor as a choriocarcinoma con-taining numerous syncytiotrophoblasts (Fig. 4). Immunohistochem-istry was positive for β-human chorionic gonadotropin (hCG; Fig. 5) and CK-19, with a Ki-67 labeling index of 90%. The tumor was also focally positive for CD31 and human placental lactogen. The patient

started chemotherapy (vincristine and cyclophosphamide) on post-operative day 16. Serum and urine hCG levels (checked after we re-ceived the pathologic report) were very high (1,637 mIU/mL and 3,070 mIU/mL, respectively). In contrast, α-fetoprotein, carcinoem-bryonic antigen, and carbohydrate antigen 19-9 levels were within normal ranges. The patient’s clinical condition improved, and the patient was discharged on postoperative day 17. On postoperative day 29, her serum hCG level had fallen to 210 mIU/mL, but was still relatively high. The patient is currently being followed up at our outpatient department.

DISCUSSION

Primary hepatic choriocarcinoma is a rare malignant tumor with propensity for rapid metastasis. Most choriocarcinomas located in the liver are of an infantile type, indicative of metastasis from oc-cult placental choriocarcinoma [9,10]. All of the patients in previous reports were males [4,6,7]. Case reports of primary hepatic chorio-carcinoma in females are difficult to find. A review of the literature and our experience with the present case confirmed the difficulty of diagnosing hepatic choriocarcino-

Fig. 1. Computed tomography image showing a large hypervascular mass in the right liver.

Fig. 2. Magnetic resonance image showing multiple masses with tiny daughter nodules in the right lobe of the liver, characteristic of angiosarcoma.

Fig. 3. Gross image of the specimen obtained after right hepatec-tomy.

Makhmud Malikov et al. • Primary hepatic choriocarcinoma in a female patient

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ma. Percutaneous needle biopsy is technically challenging and is not recommended because of the rarity of the tumor and the risk of post-biopsy bleeding [7]. Detection of overproduction of the tumor marker hCG can help with diagnosis, but hCG levels are not rou-tinely measured in the diagnosis of liver tumors. In our patient, hCG levels were only measured postoperatively after histopathology had confirmed the presence of choriocarcinoma. For these reasons, the diagnosis of primary hepatic choriocarcinoma can only be con-firmed after histopathologic testing of the resected tumor or during autopsy. Another major issue is the differential diagnosis of primary he-patic choriocarcinoma from metastasis from other sites, especially from the testis or ovary. This is confounded by the fact that primary gonadal tumors can spontaneously regress, such that only distant metastasis can be detected in the patient [11-13]. In all of the pre-

A

C

B

D

Fig. 4. Histological features. (A) Hemorrhagic and necrotic tumor is located in the hepatic parenchyma (H&E, ×40). (B) The tumor has a di-morphic plexiform pattern, consisting of cytotrophoblasts and syncytiotrophoblasts (H&E, ×100). (C) The syncitiotrophoblastic cells are mul-tinucleated with nuclear pleomorphism and hyperchromasia (H&E, ×200). (D) The tumor has invaded the vascular lumen (H&E, ×200).

Fig. 5. Immunohistochemical staining. The syncytiotrophoblasts are immunoreactive for β-human chorionic gonadotropin (×200).

36 Korean Journal of Clinical Oncology

viously reported cases, the diagnosis of primary hepatic choriocar-cinoma was confirmed after examination of serial pathologic sec-tions of the gonads during autopsy to exclude the possibility of a tumor or a tumor scar in these tissues. This method is considered a specific criterion for the diagnosis of extragonadal choriocarcinoma [14]. Another potential diagnostic method is celiac angiography, which can detect hypervascular hepatic masses with aneurismal dilatation of the peripheral hepatic arteries in the arterial phase and persistent vascular lakes in the venous phase [15]. The treatment of choice for patients without metastasis is surgi-cal resection followed by chemotherapy. In an earlier study, the re-sponse to chemotherapy was poor, and the median survival time was only 2–8 months [7]. Our patient was discharged on postoper-ative day 17. She is currently undergoing chemotherapy in our out-patient department. The patient is still alive and asymptomatic 7 months after surgery. In conclusion, primary hepatic choriocarcinoma is an aggressive tumor in adults that is extremely rare, especially in females. Further studies are needed to improve the diagnosis and treatment of this tumor.

CONFLICT OF INTEREST

No potential conflict of interest relevant to this article was reported.

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