What is the prognosis of primitive neuroectodermal tumor of the thoracopulmonary region (PNET)?

Updated: Dec 25, 2019
  • Author: Joseph F Tomashefski, Jr, MD; more...
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Answer

The prognosis of Askin tumor is generally poor. Current treatment usually includes, when possible, neoadjuvant chemotherapy followed by complete excision of the primary tumor to achieve clear surgical margins. [88] Postoperative doxorubicin-based chemotherapy is also a current standard approach. [89] Postoperative radiotherapy is deployed when surgical margins are positive. [88, 90]

In the original report by Askin et al, the mean survival was 8 months following diagnosis. [19] In another series, the 2-year survival rate was 38% and the 6-year survival rate was 14%. [18] Rare instances of extended survival have been reported. [91] Early detection of a relatively small primary lesion was thought to have been a factor in prolonged survival in one patient. [91]

Complete tumor resection with negative margins followed by chemotherapy may also achieve a longer disease-free survival. [89] Laskar et al, reporting on a large series of 104 consecutive patients with Askin tumor, identified pleural effusion, age (≥18 years), and poor response to induction chemotherapy as indicators of inferior survival. [92] After a median follow-up of 28 months, these authors found disease-free and overall survivals of 36% and 45%, respectively. Median time to relapse was 25 months, and the median survival was 76 months. [92] Fifty-six percent of patients who presented with metastatic disease died within 1 month of diagnosis, with a 2-year survival rate of 14%.


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