International neuroblastoma staging system stage 1 neuroblastoma: a prospective study and literature review.
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.
Kushner
B H
BH
Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.
Cheung
N K
NK
LaQuaglia
M P
MP
Ambros
P F
PF
Ambros
I M
IM
Bonilla
M A
MA
Ladanyi
M
M
Gerald
W L
WL
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