primitive neuroectodermal tumor of the diaphragm: case report

Introduction Primitive neuroectodermal tumors PNETs) are malignant tumors that belong to the group of small round blue-cell tumors of childhood, which includes Ewing's sarcoma, rhabdomyosarcoma, small-cell osteosarcoma, neuroblastoma, and hematolymphoid neoplasms [1]. The most common sites of involvement of PNETs are the chest wall, abdomen/pelvis, and extremities [2]. Males are more commonly affected than females [1] and typically present during the second decade of life [2]. We report a case of a PNET arising from the diaphragm of a neonate. Case report A male infant weighing 3,250 g was born at 37 weeks' gestation. The baby demonstrated hypotonia and respiratory distress min- utes after birth. Further deterioration led to intubation, and the first chest X-ray Fig. 1 a) showed a presumed large right pleural effusion with shift of the heart and mediastinum to the left. A chest tube was inserted that drained 40±50 cc of straw-colored fluid. Cytology demonstrated mesothelial cells, monocytes, and red blood cells, but was negative for malignant cells. A repeat chest X-ray at 5 days of age demonstrated slight recurrence of the pleural effusion, and a pleurocentesis was performed. The infant was at that time asymptomatic and was subsequently discharged the following day with the diagnosis of idiopathic right pleural effusion. Two days after discharge the infant again developed respiratory distress secondary to a large recurrent right pleural effusion, and thoracentesis produced 100 cc of serous/yellow fluid, which was sent for cytology. The pleural fluid rapidly reaccumulated, requir- ing two more chest tube insertions. A PA chest X-ray Fig.1b) done at 17 days of age demonstrated moderate-sized right pleural effusion and a possible mass in the costophrenic sulcus. An ultra- sound performed at this time demonstrated diminished excursion of the right hemidiaphragm. A solid mass with some cystic compo- nents measuring 4.5 3.5 cm was seen in the posterior sulcus, ap- pearing to be attached to the diaphragm by a pedicle Fig. 2). No blood flow was seen in the mass. At this time the differential diagnosis included extralobar sequestration, neuroblastoma, mesen- Marie S. Smerdely Gregory Raymond Kendra L. Fisher Ravi Bhargava Primitive neuroectodermal tumor of the diaphragm: case report Received: 10 March 2000 Accepted: 11 May 2000 M. S. Smerdely ´ G. Raymond ´ R. Bhargava ) ) University of Alberta, Department of Radiology and Diagnostic Imaging, 2A2.42 Walter C Mackenzie Health Sciences Center, University of Alberta Hospital, Edmonton, Alberta, T6G 2B7, Canada Tel.: + 1-7 80-4 07 69 07 Fax: + 1-7 80-4 07 61 76) K. L Fisher Department of Radiology, University of Saskatchewan, Saskatoon, Saskatchewan, Canada Abstract We present a case of primitive neuroectodermal tumor PNET) arising from the diaphragm in a neonate. PNETs are rare malignant tumors that belong to the group of small, round, blue-cell neoplasms of childhood. To the best of our knowledge, a PNET originat- ing from the diaphragm has not been previously reported. Pediatr Radiol 2000) 30: 702±704 Ó Springer-Verlag 2000

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Page 1: Primitive neuroectodermal tumor of the diaphragm: case report

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