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. 2014 Aug 25;5(5):463–467. doi: 10.1111/1759-7714.12120

Primary choriocarcinoma in mediastinum with multiple lung metastases in a male patient: A case report and a review of the literature

Song Zhang 1,*, Hui Gao 2,*, Xin-an Wang 3, Bin Liang 1, Dao-wei Li 1, Yang Shao 1, Shu-juan Jiang 1,✉
PMCID: PMC4704359  PMID: 26767039

Abstract

Primary choriocarcinoma is a rare malignant tumor, particularly in men. The tumor, mostly found in the gastrointestinal system and mediastinum, often metastasizes early with poor therapeutic effects and prognosis. Herein, we present a male patient with primary mediastinum choriocarcinoma and widespread lung metastases. The disease progressed rapidly with little therapeutic effect from chemotherapy. The patient died of this disease 75 days after initial symptom presentations. Literature review found only 41 cases of primary choriocarcinoma reported in the mediastinum. This case highlights the importance of keeping primary choriocarcinoma in the differentials for mediastinum tumors in young men. Sex hormone testing is helpful to confirm diagnosis. Early biopsy should be performed to confirm pathologic diagnose, and early surgery and chemotherapy should be considered to improve the cure rate of this disease.

Keywords: Gestational trophoblastic neoplasm, lung metastases, primary mediastinal choriocarcinoma

Case report

A 20 year old male presented with paroxysmal cough, expectoration of phlegm, minor fresh red hemoptysis, fever, and chest distress and pain for two weeks on 25 May 2011. Contrast-enhanced computed tomography (CT) showed a huge borderless mass in the anterior mediastinum, with low density in the central and marked enhancement in the surrounding area; diffuse oval nodules with peripheral enhancement in bilateral lungs; and enlargement of multiple mediastinal lymph nodes (Fig 1).

Figure 1.

Figure 1

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Contrast-enhanced chest computed tomography scan (24 May 2011): huge mass in the anterior mediastinum, with low density of central part and marked enhancement of the surrounding part, unclear border; diffuse distribution of nodules in oval shape with peripheral enhancement in both lungs; and multiple enlargement of mediastinal lymph nodes.

The patient was lassitude with mild cyanosis. No superficial lymph node enlargement was found. Clear harsh breath sounds were heard in both lungs, with mild moist rales in the right lower lung. No abnormal signs were observed in the heart, abdomen, genital system or neural system. Auxiliary examination results showed: Blood-Rt: white blood cell: 17.32 × 109/L; neutrophil percentage: 75.8%; hemoglobin: 139g/L; Urine-Rt: red blood cell: 5.5/heterogeneous packet flows; protein: 1 + ; tumor markers: CA12-5 56.03 U/mL (normal value: 0–39 U/mL), CYFRA21-1 50.77 ng/mL (normal value: 1–3.3 ng/mL), NSE 19.93 ng/mL (normal value: 0–16.3 ng/mL) were all elevated significantly, and the carcinoembryonic antigen (CEA) level was normal; coagulation tests (5 items): D-dimer: 666 ug/L, FIB: 4.57g/L; and the biochemical test for liver function was normal. The abdominal ultrasound and CT scan were normal. The preliminary diagnosis was a mediastinal tumor with multiple lung metastases. B-ultrasound examination was performed to exclude the primary tumor from other sites, including bilateral testes, and all were negative. Ultrasound guided percutaneous needle biopsy of the mediastinal mass was performed and the cytologic examination showed few marked dyskaryotic cells, but only hemorrhagic necrosis tissue with some keratinized material was seen in histology (Figs 2, 3).

Figure 2.

Figure 2

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Cytology examination by biopsy of the mediastinal mass (31 May 2011): few marked dyskaryotic cells.

Figure 3.

Figure 3

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Pathological examination on needle biopsy of mediastinum mass (31 May 2011): hemorrhagic necrosis accompanied with a little keratinized material, which was consistent with the characteristics of chorioepithelioma that easily develop necrosis (hematoxylin and eosin stain, four times).

The second ultrasound guided percutaneous needle biopsy from both the mediastinal mass and a metastatic lesion in the right lung was performed on 7 June 2011 and a clear pathological diagnosis was obtained as mediastinum chorionic epithelioma with lung metastasis. The immunohistochemical staining showed that human chorionic gonadotropin (HCG) and cytokeratin (CK) were positive in endothelial cells and alphafetoprotein (AFP) and CEA were negative (Figs 4, 5).

Figure 4.

Figure 4

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Pathological examination on needle biopsy of mediastinum and lung mass (7 June 2011): mediastinum chorionic epithelioma, with extensive necrosis, tumor detected in lung; immunohistochemistry: human chorionic gonadotropin (HCG)(+), alphafetoprotein (AFP)(−), carcinoembryonic antigen (CEA)(−), cytokeratin (CK) endothelial cell (+) (hematoxylin and eosin stain, four times).

Figure 5.

Figure 5

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Pathological examination on needle biopsy of mediastinum and lung mass (7 June 2011): mediastinum chorionic epithelioma, with extensive necrosis, tumor detected in lung; immunohistochemistry: human chorionic gonadotropin (HCG)(+), alphafetoprotein (AFP)(−), carcinoembryonic antigen (CEA)(−), cytokeratin (CK) endothelial cell (+). (hematoxylin and eosin stain, 10 times).

Further laboratory testing showed significantly elevated serum HCG (>5,00 000 mIU/mL, normal 0–3), estradiol (>4300 pg/mL), prolactin (65.64 ng/mL, normal value: 4.04–15.2) and AFP (104.9 ng/Ml,normal value: 0–20) levels. After communication with the patient's family and consultation with obstetrician-gynecologists, chemotherapy with the EMA-CO regimen (etoposide, methotrexate, actinomycin D, cyclophosphamide, vincristine/oncovine) was given.1 This patient was classified as having high-risk choriocarcinoma (stage III, score 10) based on the International Federation of Gynecology and Obstetrics (FIGO) staging and modified World Health Organization (WHO) risk-factor scoring system for gestational trophoblastic neoplasia (GTN),2. The EMA-CO regimen is the primary therapy for high-risk GTN including choriocarcinoma. During chemotherapy, the patient deteriorated with progressively decreased pulse oxygen saturation (60 %∼30 %), significant respiratory distress and wheezing, severe cough, and massive hemoptysis. The patient's family members decided to cease treatment on day four of chemotherapy, and the patient was discharged voluntarily. After 70 days of symptom presentations, the patient died on 21 July 2011.

Discussion

Choriocarcinoma, a highly malignant tumor from syncytiotrophoblast, can excreteβ-HCG. It usually occurs in women, mainly as a gestational trophoblastic neoplasm, and rarely occurs in men as a nonseminomatous testicular tumor. Choriocarcinoma can be divided into two types: (i) gestational choriocarcinoma, also known as secondary choriocarcinoma, which occurs after hydatidiform mole (50% of cases), abortion (30%), and normal pregnancy (20%), and good outcomes can be achieved after chemotherapy; (ii) non-gestational choriocarcinoma, also known as primary choriocarcinoma, is a very rare chorioepithelioma that can occur in men, and post-menopausal women, with primary sites mostly located in the reproductive system and occasionally in other locations. This type usually develops distant metastasis in the early stage with the lung as the common site and with a very poor prognosis. In the case presented, primary mediastinal choriocarcinoma was diagnosed via pathologic confirmation, as obvious lesions were found only in the mediastinum and lung, and the patient had an elevated serum β-HCG level.

Since the first report of primary mediastinal choriocarcinoma by Arendt in 1931,3 there have been only 43 subsequent reports.4–28All published cases of primary mediastinal choriocarcinoma were collected by a PubMed search with the key word “primary mediastinal choriocarcinoma,” and the search results were checked for eligibility. All case reports of primary mediastinal choriocarcinoma published in English or providing an informative English abstract were included. The term “primary mediastinal choriocarcinoma” published in the British Medical journal summarized 20 cases of primary mediastinal choriocarcinoma reported from 1931 to 1969,3 19 of which were in men; Fanger and MacAndrew reported the only female case;25 and 23 cases from 1969 to 2014 were found and included,4–23,27,28three of which were in women.11,20,22

Because the lung and mediastinum are frequent sites of metastatic choriocarcinoma, a diagnosis of the primary tumor should be made carefully. In this case, although multiple nodules were found in both lungs, we diagnosed the patient with primary choriocarcinoma because there was no apparent evidence of a primary genital tumor, and no lesions were found in midline structures or in other visceral organs by CT scan or abdominal echography. Nevertheless, the possibility of a metastasis from invisible lesions in other locations could not be ruled out completely because the request for an autopsy was denied.

The disease mostly presents an irritable dry cough, hemoptysis, chest pain, fever, dyspnea, and emptysis, and is usually diagnosed in young patients aged between 20 to 30 years old (range 13–67 years old). Space occupying lesions, often complicated with multiple metastases in the lung, can be revealed in the superior mediastinum by chest X-ray or CT scan, and mammary gland development by B-ultrasound. Patients with primary choriocarcinoma have significantly increased serum and urine β-HCG levels, which can serve as an important diagnosis and prognosis index with high specificity and sensitivity.16 Although AFP can be secreted by a β-HCG-producing tumor, it has low sensitivity and specificity for a diagnosis of choriocarcinoma. In contrast to gestational choriocarcinoma, primary mediastinal choriocarcinoma is rapidly fatal in the great majority of cases. In almost all cases previously reported, the tumors were found to be invading adjacent structures and organs at the time of diagnosis.26 The patient presented here was given salvage therapy with the preferred chemotherapy regimen (EMA-CO) for high-risk choriocarcinoma, but this was discontinued as a result of intolerance to the treatment caused by disease progression and respiratory failure.

We need to raise the profile of this disease. The diagnosis of primary mediastinal choriocarcinoma must be considered in young male patients with hemoptysis, dry cough, chest pain, progressive dyspnea, and radiologic findings of airspace consolidation with mediastinal disease. Sex hormone testing and biopsy should be performed earlier to determine the diagnosis, and an early combination of surgery and chemotherapy should be considered to improve the survival rate of this disease.

Disclosure

No authors report any conflict of interest.

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