Ann Thorac Surg 2008;85:656-658. doi:10.1016/j.athoracsur.2007.08.030
© 2008 The Society of Thoracic Surgeons
Case Reports
An Unusual Successfully Treated Case of Pulmonary Yolk Sac Tumor
Chih-Hao Chen, MD,
Tze-Chien Chen, MD,
Wen-Chien Huang, MD*,
Tung-Ying Chen, MD,
Hung-Chang Liu, MD
Department of Thoracic Surgery, Mackay Memorial Hospital, Taipei City, Taiwan
Accepted for publication August 15, 2007.
* Address correspondence to Dr Huang, Department of Thoracic Surgery, Mackay Memorial Hospital, No. 92, Section 2, Chung Shan North Rd, Taipei City, 10449, Taiwan (Email: musclenet2003{at}yahoo.com.tw).
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Abstract
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Extragonadal germ cell tumors are relatively rare, particularly malignant yolk sac tumors arising in the lung parenchyma. We report a case of a huge malignant yolk sac tumor in the right middle lobe. The patient was successfully treated with neoadjuvant chemotherapy followed by complete resection of the tumor.
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Introduction
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Malignant germ cell tumors usually arise in the gonads. Extragonadal tumors typically occur in the anterior mediastinum or less frequently in other midline structures. Malignant germ cell tumors, particularly yolk sac tumors, are very rarely found in the lungs.
A 19-year-old woman had a history of bilateral mature ovarian cystic teratomas for which she had undergone bilateral salpingo-oophorectomy at age 16. Her chest x-ray films at that time were normal. She presented 3 years later with a cough, chest discomfort, and weight loss. The breath sounds were decreased in the right lower lung field. Chest roentgenograms showed a huge mass in the right hemithorax. A chest computed tomographic scan showed a 20 x 20 cm intrathoracic mass with heterogenous contrast enhancement occupying nearly two-thirds of the right hemithorax. The mediastinum was deviated to the left side of the thorax by the mass. The mass invaded the chest wall, pericardium, esophagus, inferior vena cava, and diaphragm (Fig 1A). Initially we could not determine if the mass was pulmonary or mediastinal in origin, although we suspected that the previous cystic teratoma had become malignant and metastasized. However, serum levels of beta-human chorionic gonadotropin and cancer antigen-125 were within the normal range. On the other hand, the serum alpha-fetoprotein was abnormally elevated, suggesting a tumor other than a teratoma. Bronchoscopic brushing cytology, punch biopsy, and percutaneous needle biopsy had been tried, but there was no definite diagnosis obtained. Only necrotic tissues could be seen in the specimen. Therefore, a mini-thoracotomy was performed to biopsy the mass. Pathology sections showed a malignant yolk sac tumor with immunohistochemistry demonstrating strong positive staining for alpha-fetoprotein in the tumor cells. Stains for thyroid transcription factor-1 and human chorionic gonadotropin were negative. Because of the huge size of the tumor, the patient was given neoadjuvant chemotherapy with bleomycin, etoposide, and cisplatin monthly for 6 months. Although there was no further shrinkage in the tumor by 5 months after chemotherapy was begun (Fig 1B), the alpha-fetoprotein levels (Fig 2) and tumor size gradually decreased. Therefore, a standard right posterolateral thoracotomy was performed and the pleural space was entered along the sixth intercostal space. A large, firm tumor with a thin capsule was seen originating from the right middle lobe and invading the right lower lobe. The planes between the chest wall, inferior vena cava, and mediastinum were easily dissected, although adhesions to the diaphragm made dissection somewhat difficult there. We performed a right middle lobectomy and wedge resection of the right lower lobe after which there was no obvious residual tumor in the pleural cavity. On gross appearance, the tumor was ovoid and measured 12 x 10 cm (Fig 3). Microscopically, the specimen revealed large areas of necrosis, fibrosis, and inflammation without any viable tumor cells seen. The patient tolerated the procedure well without complications. Six months after surgery she had no evidence of tumor recurrence.
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Fig 1. Chest computed tomographic scan. (A) Before adjuvant chemotherapy, a huge mass invaded the lateral chest wall and mediastinum, which was deviated to the left. (B) Five months after beginning chemotherapy, the tumor is greatly shrunk, the mediastinum returns to its normal position, and there is clear separation between the tumor and the mediastinum. The tumor is relatively hypodense, suggesting large-scale necrosis.
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Fig 2. Levels of serum alpha-fetoprotein declined over the course of neoadjuvant chemotherapy. (AFP = alpha-fetoprotein; BEP = bleomycin, etoposide, and cisplatin.)
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Fig 3. Gross appearance of the pulmonary yolk sac tumor. (A) Resected tumor with part of the right lower lobe (black arrow) and the right middle lobe (green arrow), the site of origin. (B) Cut surface showing the thin capsule with marked necrosis and hemorrhage of the contents.
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Comment
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We found only two other case reports in the English literature of a primary pulmonary malignant yolk sac tumor [1, 2]. Of course, with such a rare disease there are no standard therapy guidelines. Inoue and colleagues [1] described a 31-year-old Japanese man with a pulmonary yolk sac tumor treated with a right upper lobe segmentectomy and postoperative adjuvant chemotherapy. Pelosi and colleagues [2] described another patient with elevated levels of serum alkaline phosphatase. This marker was not elevated in our patient, but her alpha-fetoprotein was elevated and it proved helpful in following the results of her neoadjuvant chemotherapy. Of course, a biopsy was required for an exact tissue diagnosis. The main concern in her case was the bulkiness of the tumor without clear tissue planes between the mass and vital organs, which led us to administer neoadjuvant treatment before undertaking surgery.
Yolk sac tumors are exceedingly sensitive to chemotherapy, and excellent results have been obtained with bleomycin, etoposide, and cisplatin [3]. Therefore, we administered these agents to the patient and followed her alpha-fetoprotein levels and tumor size. When there was no further decrease in the tumor, surgery was performed and complete resection was achieved. Based on this experience, we believe that neoadjuvant chemotherapy is worth considering for patients who have a huge pulmonary yolk sac tumor, followed by resection.
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References
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- Inoue H, Iwasaki M, Ogawa J, et al. Pure yolk-sac tumor of the lung Thorac Cardiovasc Surg 1993;41:249-251.[Medline]
- Pelosi G, Petrella F, Sandri MT, Spaggiari L, Galetta D, Viale G. A primary pure yolk sac tumor of the lung exhibiting CDX-2 immunoreactivity and increased serum levels of alkaline phosphatase intestinal isoenzyme Int J Surg Pathol 2006;14:247-251.[Abstract/Free Full Text]
- Williams S, Blessing JA, Liao SY, Ball H, Hanjani P. Adjuvant therapy of ovarian germ cell tumors with cisplatin, etoposide, and bleomycin: a trial of the Gynecologic Oncology Group J Clin Oncol 1994;12:701-706.[Abstract]
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Abstract
Introduction
