title: Primitive neuroectodermal tumors of kidney: Our experience in a tertiary care center.
abstract: OBJECTIVE
  Primitive neuroectodermal tumors (PNET) are rare highly aggressive neoplasms. The diagnosis is made by histopathology with the support of immunohistochemistry (IHC) and cytogenetics. The aggressive multimodality treatment is recommended for the management of these tumors. The purpose of our study is to review our experiences in the diagnoses and treatment of PNET of the kidneys.
  
  
  METHODS
  We retrospectively reviewed the data of all the patients, who were treated for the PNET of the kidneys at our institute between April and March 2011 and compared with the available literature.
  
  
  RESULTS
  A total of eight patients were treated for PNET of the kidney. Out of the eight patients, four were males and four females. Nearly 50% of our patients had inferior vena caval thrombus at the time of presentation. The diagnosis was made on histopathology supported by IHC. Out of the eight patients, one patient had intraoperative death due to massive pulmonary thromboembolism and another died on the 7th post-operative day due to disseminated intravascular coagulation and multiorgan failure. Rest six patients were treated with post-operative chemotherapy or a combination of chemotherapy and radiotherapy. For these six patients, overall median survival was 45 months with a 3 year disease-free survival of 66% and 5 year survival of 44%.
  
  
  CONCLUSIONS
  PNET of the kidneys are rare peripheral neuroectodermal tumors with an aggressive clinical course. These tumors carry a very poor prognosis. An aggressive treatment approach using a combination of surgery, chemotherapy and radiotherapy is recommended for a reasonable survival in these tumors.
authors.affiliation: Department of Urology, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: A
authors.initials: A
authors.lastname: Seth
authors.forename: S K
authors.initials: SK
authors.lastname: Mahapatra
authors.forename: B
authors.initials: B
authors.lastname: Nayak
authors.forename: A K
authors.initials: AK
authors.lastname: Saini
authors.forename: B
authors.initials: B
authors.lastname: Biswas
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D059248:Chemoradiotherapy; D017024:Chemotherapy, Adjuvant; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D009392:Nephrectomy; D018242:Neuroectodermal Tumors, Primitive / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D012189:Retrospective Studies; D062606:Tertiary Care Centers; D055815:Young Adult