Ewing's sarcoma and primitive neuroectodermal tumor in adults: are they different from Ewing's sarcoma and primitive neuroectodermal tumor in children?
OBJECTIVE
To determine whether age at diagnosis influences the behavior of Ewing's sarcoma and primitive neuroectodermal tumor (PNET).


METHODS
We reviewed the clinical features, treatment, and outcome of 59 consecutive patients with Ewing's sarcoma and PNET treated on the Adult Sarcoma Unit at our institution from 1980 to 1995.


RESULTS
The 37 male and 22 female patients had a median age of 24 years. Lower limb was the most common primary tumor site. Fifteen patients had nonmetastatic tumor less than 100-mL volume, 27 had nonmetastatic disease greater than 100-mL volume, and 17 had evidence of metastatic disease at presentation. The origin of the primary tumor was soft tissue in 28 cases, bone in 30, and uncertain in one. The Kaplan-Meier estimate of 5-year overall survival (OS) in all patients was 38% and of progression-free survival (PFS), 27%. When patients with metastatic disease at presentation were excluded, these figures increased to 52% and 34%, respectively. Bulk of disease at presentation and response to primary treatment were statistically highly significant predictors of both PFS and OS. Age and tissue of origin of the tumor did not influence outcome.


CONCLUSIONS
The behavior of Ewing's sarcoma and PNET in adults is no different from its behavior in children. We feel the way forward in the treatment of adults with Ewing's sarcoma and PNET is for them to be included in the current multicenter trials of multidisciplinary treatment directed at children.
Verrill
M W
MW
Sarcoma Unit, Royal Marsden National Health Service Trust, London, United Kingdom.
Judson
I R
IR
Harmer
C L
CL
Fisher
C
C
Thomas
J M
JM
Wiltshaw
E
E
D000293:Adolescent; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008297:Male; D008499:Medical Records; D008875:Middle Aged; D009367:Neoplasm Staging; D017599:Neuroectodermal Tumors / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D012189:Retrospective Studies; D012512:Sarcoma, Ewing / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D016019:Survival Analysis; D016896:Treatment Outcome