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Year : 2014  |  Volume : 2  |  Issue : 2  |  Page : 81-83

Primitive neuroectodermal tumor presenting with superior vena cava syndrome

1 Department of Pediatrics, PGIMER, Dr. RML Hospital, New Delhi, India
2 Department of Pathology, PGIMER, Dr. RML Hospital, New Delhi, India

Correspondence Address:
Devki Nandan
MD (Pediatrics), D 3/3210, DDA SFS Flats, Vasant Kunj, New Delhi 110 070
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2320-8775.135119

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Peripheral primitive neuroectodermal tumor (PNET) of the chest wall is considered akin to Ewing's sarcoma in terms of histology, immunohistology and cytogenetics. These tumors usually occur in children and young adults. These tumors usually present with cough, fever, chest pain and hemoptysis. Initial presentation as superior vena cava syndrome (SVCS) is rarely seen. Complete blockage of venous drainage in SVCS may lead to fatal outcome. These tumors are highly aggressive with a high incidence of local recurrence and distant metastases. Combination therapy comprising of chemotherapy, radiotherapy and surgery can be lifesaving in these patients. We present a case of primary mediastinal PNET in a 12-year-old girl. To the best of our knowledge, this is the first case of PNET presenting with SVCS in children.

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