title: International neuroblastoma staging system stage 1 neuroblastoma: a prospective study and literature review.
abstract: OBJECTIVE
  To gain insight into the management of non-metastatic neuroblastoma by examining clinical and biologic features of International Neuroblastoma Staging System (INSS) stage 1 tumors.
  
  
  METHODS
  Patients were staged by both the INSS and the Evans staging system and were evaluated for biologic prognostic factors. Patients with INSS stage 1 received no cytotoxic therapy. The literature was reviewed for clinical and biologic data about INSS stage 1.
  
  
  RESULTS
  We evaluated 10 consecutive patients (median age, 17.5 months) with INSS stage 1; all remain disease-free (median follow-up duration, > 5 years). Tumors were in the abdomen (n = 6), chest (n = 3), or pelvis (n = 1). Neuroblastoma involved margins of resection in six tumors. Poor-prognostic biologic findings included tumor-cell diploidy (n = 2) and unfavorable Shimada histopathology (n = 2). Two patients were to receive chemotherapy for, respectively, a tumor deemed unresectable and a tumor classified as Evans stage III; second opinions resulted in surgical management alone in each case. Published reports confirm that some INSS stage 1 patients (1) are at risk for overtreatment, and (2) have poor-prognostic biologic findings yet do well.
  
  
  CONCLUSIONS
  Surgery alone suffices for INSS stage 1 neuroblastoma, even if biologic prognostic factors are unfavorable, microscopic disease remains after surgery, and tumor size is suggestive of "advanced-stage" status in other staging systems. Attempts to resect regionally confined neuroblastomas should take precedence over immediate use of cytotoxic therapy; otherwise, some patients may receive chemotherapy or radiotherapy unnecessarily.
authors.affiliation: Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.
authors.forename: B H
authors.initials: BH
authors.lastname: Kushner
authors.forename: N K
authors.initials: NK
authors.lastname: Cheung
authors.forename: M P
authors.initials: MP
authors.lastname: LaQuaglia
authors.forename: P F
authors.initials: PF
authors.lastname: Ambros
authors.forename: I M
authors.initials: IM
authors.lastname: Ambros
authors.forename: M A
authors.initials: MA
authors.lastname: Bonilla
authors.forename: M
authors.initials: M
authors.lastname: Ladanyi
authors.forename: W L
authors.initials: WL
authors.lastname: Gerald
mesh_terms: D000293:Adolescent; D000328:Adult; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000145:classification / Q000473:pathology* / Q000628:therapy; D011446:Prospective Studies