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J Thorac Cardiovasc Surg 2002;124:833-836
© 2002 The American Association for Thoracic Surgery

Brief Communications

Primitive neuroectodermal tumor: Report of two cases and review of the literature

R. Hage, MDa, V. A. M. Duurkens, MDa, C. A. Seldenrijk, MD, PhDb, A. Brutel de la Rivière, MD, PhDc, H. A. van Swieten, MD, PhDc, J. M. M. van den Bosch, MD, PhDa Nieuwegein, The Netherlands

From the Heart Lung Centre Utrecht (HLCU), St Antonius Hospital, Departments of Pulmonology,a Pathology,b and Thoracic Surgery,c Nieuwegein, The Netherlands.

Received for publication Jan 4, 2002. Accepted for publication March 4, 2002. Address for reprints: J. M. M. van den Bosch, MD, St Antonius Hospital, Department of Pulmonology, PO Box 2500, Niuewegein, The Netherlands 3430 EM (E-mail: j.vandenbosch@antonius.net).

The first 300 words of the full text of this article appear below.

The malignant small round-cell tumors of the thoracopulmonary region were first described by Askin in 1979.Go 1 These tumors are characterized by neuroectodermal derivation and anatomic distribution. Currently, Ewing sarcoma (ES), primitive neuroectodermal tumors (PNETs), and malignant small round-cell tumors of the thoracopulmonary region are suggested to be different manifestations of a single tumor family. However, these tumors are rare and often occur in childhood or adolescence. Only a few series have been described in the past. We review 2 cases of patients who were referred to our clinic, followed by a review of literature.

Clinical summaries

Patient 1
A 13-year-old boy presented with a right-sided palpable painful mass on his chest for 1 month and thoracic pain for 5 months.

There was no pain elsewhere, nor was there any dyspnea or constitutional symptoms, such as fever and weight loss. He had no smoking history. At physical examination, there was a slightly prominent lateral chest wall on the right, without other signs of pathology. A chest x-ray film showed an abnormal consolidation in the right lateral thorax on the level of the eighth dorsal rib and discrete pleural effusion in the right hemithorax (Figure 1).


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  		Fig. 1. Chest radiograph showing a large solid mass involving the right thoracic wall.

 
Thoracoscopy showed a normal right upper lobe and an adhesion of the middle lobe to the anterolateral chest wall with thickening of the visceral pleura. The parietal pleura under this adhesion was irregular and red colored. There was some hemorrhagic fluid in the right thorax, showing no clear malignancy on posteroanterior examination. Bone scintigraphy results were positive for the right seventh rib from the anterolateral to the dorsal position, without other hot spots. Thoracotomy was performed for incisional biopsy of the tumor.

Histologically, the tumor had a solid predominant lobular growth pattern (Figure 2, A). . . . [Full Text of this Article]






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