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J Thorac Cardiovasc Surg 2003;125:193-196
© 2003 The American Association for Thoracic Surgery
Brief Communications |
From the Department of Thoracic Surgerya and the Department of Oncology and Biomedical Science,b University of Torino, Italy.
Received for publication March 15, 2002. Accepted for publication April 30, 2002. Address for reprints: Anna Arslanian, MD, University of Torino, San Giovanni Battista Hospital, Department of Thoracic Surgery, Via Genova, 3 10126 Torino, Italy (E-mail: slani{at}interfree.it).
Choriocarcinoma is a malignant proliferation of the Langerhans cell and of syncytial cells of trophoblastic origin that is normally situated in the female genital tract after a gestational event such as molar pregnancy, term pregnancy, abortion, or ectopic pregnancy.
1 Rarely it occurs in either sex as a midline lesion in the retroperitoneum, mediastinum, and the region of the pineal gland. Less frequently it is found in the bladder, liver, stomach, and colon, where it is seen in combination with adenocarcinoma. 2-4 In men, choriocarcinoma occurs in the testis in combination with such tumors as seminoma, teratoma, and embryonal carcinoma. 1 The literature contains few cases of lung localization of choriocarcinoma. The prognosis of this tumor is extremely poor, despite surgical and chemotherapeutic treatment. 5 We report a surgically treated case of choriocarcinoma in a 37-year-old woman who came to our attention because of a lung tumor with radiologic characteristics similar to those of a solitary fibrous tumor of the pleura.
Clinical summary
During surgical evaluation for cholecystectomy for cholelithiasis in a 37-year-old woman, the chest radiograph showed an opacity in the right inferior pulmonary lobe (Figure 1). Computed tomographic scans demonstrated a well-demarcated mass similar to solitary fibrous tumor of the pleura (Figure 2). On physical examination, the woman appeared healthy with stable vital signs. The patient referred in her history to an operation for vesicular mole that had occurred 7 years previously.
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Chemotherapy was started 2 months after thoracic surgery and consisted of bleomycin, etoposide, and cisplatin. Before the start of chemotherapy, the level of ß-HCG was measured; it was 8 U/L 40 days after surgery. At 1-year follow-up the patient is alive in good condition. The ß-HCG level is actually normal.
Discussion
Choriocarcinoma is a germ cell tumor containing syncytiotrophoblastic cells and secreting human ß-HCG. 4-6 Primary choriocarcinoma of the lung is extremely uncommon, with roughly 20 cases reported in the literature. In contrast to other extragonadal germ cell tumors, choriocarcinoma appears more frequently in women and elderly men. The origin of these tumors at this site is unknown, but there are several theories. The tumors could represent metastases from a primary gonadal neoplasm undergoing spontaneous regression. 1,6 It is also possible that they are derived from a progression to malignancy of trophoblastic emboli from a previous vesicular mole, term pregnancy, abortion, or ectopic pregnancy months or years before. 3,6 Another hypothesis is that there was a differentiation or metaplasia to trophoblast from a primary lung cancer. Finally, the tumor could have been generated from remaining germ cells after an anomalous migration during the embryonic phase. 6
Pulmonary choriocarcinoma can appear in different ways. Recurrent hemoptysis seems to be the most frequent symptom; in addition to this, it has been reported as chest pain, persistent cough, and signs of feminization in men with gynecomastia, loss of libido, and testicular atrophy. Distant metastases are commonly present in the lung, brain, and kidney. 3,6
Two typical characteristics of this tumor represent the choriocarcinoma syndrome: the bleeding tendency of the primary and metastatic sites and the constant elevation of serum ß-HCG levels. 6 ß-HCG is useful for diagnostic, prognostic, and follow-up purposes. 2
A positive ß-HCG test result in patients with hemoptysis and progressive dyspnea could be diagnostic for a pulmonary choriocarcinoma and may be helpful for a early diagnosis. 5,6 The diagnostic criteria would include lack of a previous gynecologic malignancy, solitary or predominant lung lesion with the exclusion of a gonadal primary site, raised serum ß-HCG titers that become normal after surgery or chemotherapy, and pathologic confirmation of the disease.
Hematologic analysis shows also leukocytosis with neutrophilia and elevated sedimentation rate. 5 Although urinary ß-HCG has considerable diagnostic and prognostic value, a negative test result does not exclude presence of these neoplasms.
The radiologic presentation may be nodular image, diffuse shadowing, or miliary or micronodular forms with or without interstitial infiltrates. 5 Diagnosis is often delayed, and pleural effusion may be present.
Bronchoscopic examination yields negative results for this tumor. 5 Percutaneous needle biopsy often shows a largely necrotic nonBsmall cell carcinoma interpreted as squamous cell type from the deeply eosinophilic cytoplasm, suggesting keratinization in the few viable anaplastic cells. Electron microscopic features of tonofilaments are present on ultrastructural analysis, also in keeping with squamous differentiation. Immunohistochemical staining shows cytotrophoblastic cells reactive for ß-HCG. Coexistence with adenocarcinoma of the lung has been reported in the literature. 1,3
The surgical option is often selected on the basis of presumed squamous cell carcinoma of the lung in the absence of distant and regional metastases, and the correct diagnoses represent a surprise at histologic diagnosis. 2 So, as in this case, ß-HCG was not measured before surgery. The natural course of choriocarcinoma is rapidly fatal in the great majority of cases. 5 We think that surgical excision according to the oncologic criteria for bronchopulmonary cancer is advisable, however, because there are no established therapeutic guidelines and this tumor evolved rapidly with bleeding, chest pain, and dyspnea. More or less 30 days after surgery, the ß-HCG level returned to normal. During follow-up an elevation of ß-HCG would be indicative of micrometastasis. 2
Chemotherapy is indicated for this tumor independently of surgery and consists of bleomycin, etoposide, and cisplatin. 2 Nongestational choriocarcinoma, unlike that arising from a gestational tumor, is unresponsive to irradiation. The reason is unknown. 1
Frequent signs and symptoms
General syndrome
Radiologic evidence
Appearance of the tumor may be diffuse or defined.
Hematologic analysis
Positive results are seen on ß-HCG test. Leukocytosis is present, with neutrophilia and elevated sedimentation rate.
Urinalysis
ß-HCG may be present in the urine.
References
This article has been cited by other articles:
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  N. Girard, M. Barbareschi, J.-F. Cordier, and B. Murer What is a rare tumour and how should it be dealt with clinically? Pathology of the Lung, June 30, 2010; 85 - 133. [Abstract] [Fulltext] [PDF]
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