Synchronous primary intrapulmonary and mediastinal thymoma-A case report
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We report an extremely rare case of Synchronous primary intrapulmonary and mediastinal thymoma in a Chinese patient. We describe the histological and radiological findings, which support the possibility of multicentric thymoma. Resection of the mass in the left anterior superior mediastinum and upper lobectomy of right lung were performed, with lymph Nodes clearance, superior vena cava, left and right brachiocephalic veins resection, reconstruction of left brachiocephalic vein to right auricle and reconstruction of right brachiocephalic vein to superior vena cava.
KeywordsThymoma Superior Vena Visceral Pleura Brachiocephalic Vein Multiple Primary
Thymomas are tumors derived from thymic epithelial cells and have an incidence of 0.15 per 100000. Primary intrapulmonary thymomas are defined as thymomas arising in an intrapulmonary location without an associated mediastinal component and are very rare. Here we present a successfully resected case of synchronous primary intrapulmonary and mediastinal thymoma with vascular reconstruction.
Primary intrapulmonary thymuses are very uncommon, with 28 cases reported to date. Even rarer cases were reported for Synchronous primary intrapulmonary and mediastinal thymoma. The incidence for lung cancer in China increased by 1.63% from 1988 to 2005. Some special thoracic malignancies should be paid attention to in China. Primary intrapulmonary thymomas appear to fall into two groups: one is in the hilus of the lung, in relation to the wall of a major bronchus or attached to the pericardium, and the other is peripheral in the lung and beneath the visceral pleura. In the case of hilar type, the notion that intrapulmonary thymomas are derived from mediastinal thymomas that migrate into the lung with pinching off of the pleural behind them would be acceptable, but it fails to provide a satisfactory explanation for the occurrence of the peripheral. Marchevsky believed intrapulmonary thymomas arose from stem cells, uncommitted germinative cells capable of differentiating along a variety of lines. This theory is supported by the large number of reports of heterotopic, histologically mature tissues within the lung parenchyma, such as thyroid follicules, pancreas, adrenal, liver, neuro-glial tissue and endometrium, or tumors derived from ectopic tissue, such as melanoma, meningioma, glomus or glomangioma, choriocarcinoma, teratoma, ependymoma and, of course, thymoma, which could develop from such stem cells. Multiple thymomas remain controversial as to whether multiple thymomas involve intrathymic dissemination or represent multiple primaries, which could be explained by Marchevsky's theory. Although Bernatz et al.  reported 3 out of 138 (2.2%) thymomas to be multiple primaries, it was difficult to clarify whether the multiple thymomas in their cases involved double primary or dissemination, because they did not mention any close histological characteristics among the multiple thymomas. Since both our cases were totally encapsulated tumors and did not have any dissemination in the other portion, they were considered to be multiple primaries. Therefore our case provided better evidence to support Marchevsky's theory for the development of intrapulmonary thymomas.
The clinical course is that of a slow-growing lesion that remains asymptomatic until it reaches a size causing problems due to local growth, such as pain, bronchial obstruction or hemoptysis. As with mediastinal thymomas, they can be associated with paraneoplastic syndromes, such as myasthenia gravis or Good's syndrome. Resection appears sufficient in non-malignant tumors. In incompletely resected patients, adjuvant radiotherapy should be considered. Long-term regular clinical follow-up is warranted, because of the risk of late local recurrence.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
This study was partly supported by the grants from Key Project of National Natural Science Foundation of China(No.30430300), National 973 Program (No.2010CB529405), National 863 Program (No.2006AA02401) and S&T Support Key Program of Tianjin (09ZCZDSF04100, 09ZCZDSF04000)..
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