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Novel treatment (new drug/intervention; established drug/procedure in new situation)
Management of a large retroperitoneal primitive neuroectodermal tumour: ‘a multimodal approach’
  1. Shailesh Sable1,
  2. Vidhyachandra Gandhi1,
  3. Aabha Nagral2,
  4. Sanjay Nagral1
  1. 1Gastrointestinal Surgery Department, Jaslok Hospital, Mumbai, India
  2. 2Medical Gastroenterology, Jaslok Hospital, Mumbai, India
  1. Correspondence to Dr Aabha Nagral, aabhanagral{at}gmail.com

Summary

Retroperitoneal primitive neuroectodermal tumour (PNET) is a rare disease having poor prognosis. Treatment mainly consists of en block resection of the tumour to achieve RO resection, however multimodal approach has also been used with improved survival. The authors report a 40-year-old male with large retroperitoneal PNET adherent to aorta and compressing the inferior vena cava. He was subjected to P6 protocol chemotherapy (cyclophosphamide, adriamycin, vincristine, ifosfamide and etoposide) in view of borderline operability. Postchemotherapy contrast enhanced CT revealed significant reduction in size of the lesion. He underwent complete resection of the tumour followed by concurrent chemoradiation. He remains asymptomatic on follow-up over a period of 2 years.

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  • Competing interests None.

  • Patient consent Obtained.

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