Asian Cardiovasc Thorac Ann 2008;16:410-411
© 2008 Asia Publishing EXchange Ltd
Pure Yolk-Sac Tumor of the Lung
Ahmet Basoglu, MD,
Aysen T Sengul, MD,
Yasemin B Buyukkarabacak, MD,
Tulin D Yetim, MD,
Levent Yildiz, MD1
Department of Thoracic Surgery
1 Department of Pathology, Ondokuz Mayis University Medical School, Kurupelit, Turkey
For reprint information contact: Aysen Taslak Sengul, MD Tel: 90 362 312 1919 Fax: 90 362 457 6041 Email: aysentaslak{at}yahoo.com, Ondokuz Mayis University, Medical School, Kurupelit, Samsun 55139, Turkey.
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ABSTRACT
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Primary germ cell tumors of the chest often localize in the anterior mediastinal compartment. Such tumors originating from lungs and pleura are rare. Chest tomography revealed a mass in the middle lobe of the right lung in a 25-year-old man. A middle lobe medial segmentectomy was performed, and chemotherapy was applied postoperatively.
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INTRODUCTION
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Germ cell tumors have high-grade malignancy and they progress rapidly. They often originate from the testis or ovary. Extragonadal germ cell tumors are mainly located in the retroperitoneal, mediastinal, pineal, or presacral regions.1 Primary germ cell tumors originating from lungs and pleura are rare.
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CASE REPORT
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A 25-year-old man was referred to our clinic with complaints of dyspnea, cough, weight loss, and fever. On physical examination, breath sounds were found to be decreased in the right upper and middle zones. Other systemic examinations were normal. Chest radiography showed a mass in the right middle and lower zone, which had no clear border with the heart (Figure 1A
). Computed tomography revealed a 5 x 9 x 12-cm mass in the right middle lobe (Figure 1B). On fiberoptic bronchoscopy, intrabronchial obliteration was observed in the right middle lobe medial segment. Endobronchial biopsy revealed nonspecific material. A histopathological examination of material from transthoracic needle aspiration was reported as a malignant tumor, but the tumor type was not defined. Because of the unclear pathological diagnosis, an exploratory thoracotomy was performed. A 10 x 12-cm mass full of necrotic material was observed in the middle lobe medial segment. The upper and lower lobe superior segments were atelectatic because of the mass pressure. A frozen-section study revealed a germ cell tumor, but its type was unclear. The intraoperative serum concentration of alpha fetoprotein (AFP) was high (2,116 ng·mL–1), and human chorionic gonadotropin hormone was normal. A middle lobe medial segmentectomy was performed. On histopathological examination, a cellular tumoral structure was observed. The tumor was composed of large clear cytoplasmic cells that surrounded cystic vacuoles or formed large solid sheets (Figure 2A). Immunohistochemically, the tumor cells showed widespread AFP positivity (Figure 2B). A pathological study reported a germ cell tumor. Because postoperative testis and abdominal ultrasonography was normal, the patient was diagnosed with primary pulmonary germ cell tumor. He received 4 courses of chemotherapy with bleomycin, etoposide, and cisplatin. He has been alive for 16 months with a normal AFP level.
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Figure 1. (A) Chest radiograph showing a mass in the right middle and lower zone, which had no clear border with the heart. (B) Computed tomography revealing a 5 x 9 x 12-cm mass in the right middle lobe.
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Figure 2. (A) A cellular tumor structure was observed on histopathological examination. Tumor cells with wide clear cytoplasm (hematoxylin and eosin stain, original magnification x200). (B) Immunohistochemical staining of the tumor showed a positive reaction for alpha fetoprotein. Original magnification x200 (ABC staining, DAB chromogen).
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DISCUSSION
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Malign extragonadal germ cell tumors include absence of a gonadal tumor that is detectable or appears subsequently, and no nodal metastases in the paraaortic and iliac regions.1 However, only 5% 10% of all germ cell tumors are located in the mediastinum; typically, in the anterior compartment.2 Primary pulmonary locations of germ cell tumors are extremely rare, and most are mixed germ cell tumors. Teratoma cases with embryonal carcinoma and yolk-sac tumor, and mixed embryonal carcinoma with yolk-sac tumor or seminoma have been reported.3–5 We found 3 previous reports of primary pulmonary yolk-sac tumors. The first patient lived for 13 months after surgery and chemotherapy, and the second had survived for 9 months when the report was published in 2006.6,7 The 3rd case was a pure pulmonary yolk-sac tumor in a young patient whose diagnosis was made at autopsy.4
The pathogenesis of extragonadal germ cell tumor is controversial. It is generally believed to develop by the neoplastic transformation of primordial germ cells displaced at various points during their normal pathway of migration to the urogenital ridge. Most germ cell tumors originate from such midline areas as the mediastinum and retroperitoneum.5 These tumors are observed in the young, grow rapidly, have high AFP but normal human chorionic gonadotropin levels, and they are sensitive to chemotherapy.4 For diagnosis of these rarely seen primary pulmonary germ cell tumors, an exploratory thoracotomy may be necessary. It has been claimed that nonseminomatous tumor diagnosis is possible without histopathological confirmation when the AFP level is over 500 ng·mL–1. However, primary lung cancers that produce AFP have been reported. It should be noted that the AFP level can be high not only in germ cell tumors but also in lung cancers. In the management of germ cell tumors, chemotherapy is usually the first choice, thus it should be carefully differentiated from lung cancer. A correct diagnosis is necessary not only for survival but also for justification and prediction of the outcome of chemotherapy.
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REFERENCES
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ABSTRACT
INTRODUCTION
