Management of a large retroperitoneal primitive neuroectodermal tumour: 'a multimodal approach'.
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.
Gastrointestinal Surgery Department, Jaslok Hospital, Mumbai, India.
Shailesh
S
Sable
Vidhyachandra
V
Gandhi
Aabha
A
Nagral
Sanjay
S
Nagral
D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D004317:Doxorubicin / Q000008:administration & dosage / Q000627:therapeutic use; D005047:Etoposide / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage / Q000627:therapeutic use; D008297:Male; D018242:Neuroectodermal Tumors, Primitive / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D012186:Retroperitoneal Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D014057:Tomography, X-Ray Computed; D014750:Vincristine / Q000008:administration & dosage / Q000627:therapeutic use
D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D007069:Ifosfamide