(Chest. 2002;121:654-656.)
© 2002
American College of Chest Physicians
Choriocarcinoma in the Pulmonary Artery Treated With Emergency Pulmonary Embolectomy*
Shun-ichi Watanabe, MD;
Shinji Shimokawa, MD;
Koh-ichi Sakasegawa, MD;
Hiroshi Masuda, MD;
Ryuzo Sakata, MD and
Michiyo Higashi, MD
*
From the Second Department of Surgery (Dr. Higashi) and the Second Department of Pathology (Drs. Watanabe, Shimokawa, Sakasegawa, Masuda, and Sakata), Kagoshima University Faculty of Medicine, Kagoshima, Japan.
Correspondence to: Shun-ichi Watanabe, MD, The Second Department of Surgery, Kagoshima University Faculty of Medicine, 8–35-1 Sakuragaoka, Kagoshima, 890-8520 Japan
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Abstract
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A 42-year-old woman with choriocarcinoma required emergency
pulmonary embolectomy under cardiopulmonary bypass. After diagnosis of
choriocarcinoma was confirmed by examination of tumor emboli specimens,
the patient was treated and had complete remission by chemotherapy over
a 6-month period. Although rare, choriocarcinoma should be considered
in the differential diagnosis of fertile women presenting with
pulmonary embolism.
Key Words: cardiopulmonary bypass • choriocarcinoma • emergency • pulmonary artery • pulmonary embolectomy • pulmonary embolism
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Introduction
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Due
to their rarity, tumors of the pulmonary arteries are often incorrectly
diagnosed as more common diseases, such as pulmonary thromboembolism,
and are thus seldom diagnosed during a patient’s lifetime. We report a
patient with a choriocarcinoma mimicking pulmonary embolism, who
required emergency pulmonary embolectomy and had a complete remission
with adequate chemotherapy thereafter.
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Case Report
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A 42-year-old woman presented with exertional dyspnea in August
1999. The patient had three children, the youngest being 4 years old,
and she had two previous spontaneous abortions. The chest radiographs
showed a bilateral diffuse infiltrative shadow. She was treated for
interstitial pneumonia; however, her symptoms became worse. On December
16, pulmonary thromboembolism was suspected and she was urgently
admitted to our hospital. On hospital admission, she was orthopneic and
could not be placed in a left decubitus position. Arterial blood gas
levels under oxygen mask inhalation (5 L/min) were as follows: pH,
7.49; PO2, 44 mm Hg; and
PCO2, 26 mm Hg. The chest enhanced CT
showed a filling defect from the main pulmonary artery (MPA) to the
right pulmonary artery (RPA) [Fig 1
]
. On the same day, the patient underwent emergency pulmonary embolectomy
under cardiopulmonary bypass. Opening from the MPA to the bilateral
pulmonary arteries, a whitish, soft tumor embolus with a papillary
surface was found occluding the MPA and extending into the RPA. The
tumor embolus was removed as much as possible. Pulmonary arterial
pressure dropped from 80 to 50 mm Hg immediately after the operation.
However, the patient required ventilator support for subsequent
respiratory failure owing to residual tumor emboli.
Microscopically, the pulmonary tumor emboli specimens showed necrotic
tissue in the central area and proliferation of atypical cells in the
peripheral areas (Fig 2
, top). The cells had hyperchromatic nuclei and eosinophilic
cytoplasm with indistinct cell borders forming "syncytiotrophoblastic
cells" (Fig 2
, bottom). After a diagnosis of
choriocarcinoma was made, serum human chorionic gonadotropin (hCG)
level was measured and a gynecologic examination was performed. As
serum hCG values were > 70,000 mIU/mL, chemotherapy was initiated
with actinomycin D, 0.1 mg/m2 for 5 days. Then,
etoposide, 80 mg/m2 on days 1 to 5, and
carboplatin at a dose set to the area under the concentration-time
curve of 4.0 on day 1 were administered during two courses. After
her performance status improved by a level of 2, a high-risk
chemotherapy regimen was initiated: etoposide, 100
mg/m2 on days 1 to 5; methotrexate, 100
mg/m2 via IV bolus on day 1 and 200
mg/m2 via IV infusion over 12 h on day 1;
actinomycin D, 500 µg via IV bolus on day 2; cyclophosphamide, 600 mg
via IV infusion on day 8; and vincristine, 1.0
mg/m2 on day 8. This treatment regimen was
continued until three consecutive normal hCG levels were reached. After
a 6-month period of chemotherapy, the residual lung tumor decreased in
size and lung fields of the chest radiographs became clear. On July 20,
2000, the patient was discharged from our hospital in complete
remission.
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Figure 2.. Top: Microscopically, the pulmonary
tumor emboli specimens showed necrotic tissue in the central area and
proliferation of atypical cells in the peripheral areas
(hematoxylin-eosin, original x 40). Bottom: The cells
had hyperchromatic nuclei and eosinophilic cytoplasm with indistinct
cell borders forming "syncytiotrophoblastic cells". These cells
were localized on the peripheral side (hematoxylin-eosin,
original x 200).
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Comment
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Bagshawe and Brooks1
were the first to suggest that
pulmonary embolism and pulmonary hypertension could be due to a
choriocarcinoma of the pulmonary arteries. Choriocarcinoma is the most
aggressive form of gestational trophoblastic disease. Most cases occur
following a complete hydatidiform mole. The natural history of
untreated choriocarcinoma is characterized by the development of early
hematogenous metastases.2
Some of the vagaries of this
tumor have attracted attention, and failure to find a primary growth in
the uterus, even on careful microscopic examination, is not infrequent.
To the best of our knowledge, only a few cases of primary
choriocarcinoma in a patient’s lifetime have been
reported.3
4
5
6
However, this is the first patient who
underwent emergency pulmonary embolectomy and was treated with
subsequent chemotherapy after a confirming pathologic diagnosis. The
patient had been monitored for interstitial pneumonia for > 5 months,
and pulmonary thromboembolism was finally diagnosed. Diagnosis of
choriocarcinoma in the pulmonary artery is very difficult in the early
stage.1
In the present case, a pulmonary tumor thrombus
was examined histologically and diagnosed as choriocarcinoma for the
first time.
Many medical textbooks omit choriocarcinoma as a cause of pulmonary
emboli and pulmonary artery hypertension. However, Seckl and
coworkers4
reported that although rare, choriocarcinoma
should be considered in the differential diagnosis of fertile women
presenting with pulmonary embolism or pulmonary artery hypertension.
Furthermore, Savage et al6
emphasized the importance of
considering choriocarcinoma and measuring serum hCG in the
investigation of fertile women presenting with pulmonary emboli or
pulmonary artery hypertension. In our case, choriocarcinoma was not
diagnosed in an early stage. However, chemotherapy was initiated as
soon as the diagnosis was made and the patient responded positively,
with serum hCG falling to within normal levels. Unfortunately, because
the hospitalization was long, the cost of treatment became expensive.
The problem of the limited health-care resources is a further
consideration. Our results indicate that although rare, choriocarcinoma
should be considered in the differential diagnosis of fertile women
presenting with pulmonary embolism, because complete remission can be
achieved with appropriate chemotherapy.
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Footnotes
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Abbreviations: hCG = human chorionic gonadotropin;
MPA = main pulmonary artery; RPA = right pulmonary artery
Received for publication April 3, 2001.
Accepted for publication July 12, 2001.
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References
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Bagshawe, KD, Brooks, WDW (1959) Subacute pulmonary hypertension due to chorioepithelioma. Lancet 28,653-658[CrossRef]
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Rosai, J (1996) Female reproductive system. Rosai, J eds. In: Ackerman’s surgical pathology 8th ed. ,1319-1554 Mosby St. Louis, MO.
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Graham, JP, Rotman, HH (1976) Tumor emboli presenting as pulmonary hypertension: a diagnostic dilemma. Chest 69,229-230[Abstract]
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Seckl, MJ, Rustin, GJS, Newlands, ES, et al (1991) Pulmonary embolism, pulmonary hypertension, and choriocarcinoma. Lancet 338,1313-1315[CrossRef][ISI][Medline]
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Trubenbach, J, Pereira, PL, Huppert, PE, et al (1997) Primary choriocarcinoma of the pulmonary artery mimicking pulmonary embolism. Br J Radiol 70,843-845[Abstract]
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Savage, P, Roddie, M, Seckl, MJ (1998) A 28-year-old woman with a pulmonary embolus. Lancet 352,30[CrossRef][ISI][Medline]
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Abstract
Introduction
