Primitive neuroectodermal tumor of chest wall in a young adult: comprehensive work-up, nine months post surgery follow-up and adjuvant chemotherapy
Amit Nandan Dhar dwivedi, Prashant K Gupta, Rohit Bhargava and Kumkum Gupta
World Journal of Surgical Medical and Radiation Oncology 2012, 1:5
Introduction: Primitive neuroectodermal tumor is a malignant small round cell tumor affecting the thoraco-pulmonary region, also known as Askin tumor.
Case report: A seventeen year old male presented with recent onset breathlessness of one and half month duration which was progressive and increased on exertion. After physical and systemic examination he was subjected to chest skiagram, contrast enhanced computed tomography (CECT) scan and magnetic resonance imaging (MRI). Skiagram of chest showed a well circumscribed mass in upper zone of right lung. Fine needle aspiration cytology (FNAC) showed malignant small round cell tumor. Clinical, radiological and cytological findings led to the diagnosis of round cell tumor-primitive neuroectodermal tumor of chest wall. The patient underwent radical surgery with adjuvant chemotherapy. The patient was followed up for nine months. During this period he was clinically and radiologically disease free.
Conclusions: Primitive neuroectodermal tumor is malignant small round cell tumor affecting thoraco-pulmonary region. A standardized multimodality treatment is more beneficial. Long term survival can be achieved with both aggressive local and adjuvant multidrug chemotherapy