Ewing’s sarcoma family of tumors in Finland during 1990–2009: A population-based study

Figures & data

Table I. Patient characteristics.

	No. patients
	Local	Metastatic	All
All	57	19	76
Age (median)	16.1	18.6	17.8
Sex
Male	35	15	50
Female	22	4	26
Site of tumor as axial or distal
Distal	29	9	38
Axial	28	10	38
Site of tumor as bone or soft tissue*
Bone	38	11	49
Soft tissue	19	8	27
Largest diameter of the primary tumor in cm**
< 8 cm	26	9	35
> 8 cm	31	8	39
Year of diagnosis
1990–1999	27	6	33
2000–2009	30	13	43
Molecular genetic analysis†
Positive	26	9	35
Negative	9	1	10

* Multifocal disease was classified as bone tumor if bone lesions were present.

** Data not available for two patients.

† Molecular genetic analysis was not performed in 31 patients.

Figure 1. Five-year disease-free survival of patients with localized disease according to a) surgical local control and b) age at diagnosis.

Table II. The five-year EFS and DSS of patients with localized ESFTs.

	No. patients	EFS		DSS
		%	p	%	p
Sex
Male	35	64%	0.370	72%	0.750
Female	22	54%		67%
Age at diagnosis*
0–18 years	30	66%	0.154	75%	0.051*
> 18 years	27	53%		64%
Site of primary tumor
Axial	28	50%	0.046	54%	0.016
Peripheral	29	70%		85%
Bone or soft tissue tumor
Bone	38	59%	0.886	67%	0.597
Soft tissue	19	63%		78%
Size
0–8 cm	26	69%	0.425	76%	0.202
> 8 cm	31	53%		65%
Neoadjuvant chemotherapy**
No	7	65%	0.516	80%	0.201
Yes	47	60%		69%
Response to neoadjuvant therapy†
Poor	11	72%	0.941	68%	0.347
Good	27	70%		81%
High dose chemotherapy††
No	43	59%	0.817	71%	0.662
Yes	13	67%		67%
Surgical resection with at least marginal margins††
No	11	46%	0.040	46%	0.033
Yes	45	65%		79%
Time of diagnosis
1990–1999	27	56%	0.465	63%	0.210
2000–2009	30	66%		79%

*In Cox regression analysis the risk for death due to disease increases 1.03-fold for every year of age at diagnosis (95% CI 1.00–1.06, p = 0.031)

**N = 54, two patients had no local treatment and one died in <3 months

^†Those who received neoadjuvant therapy and were operated and the data was available.

^††The patient who died in less than 3 months was not included.

CI, confidence interval; DSS, disease-specific survival; EFS, event-free survival; ESFT, Ewing’s sarcoma family of tumors.

Table III. The local treatment of the primary tumor and five-year local relapse-free survival (LRFS).

	No. patients	5-year LRFS	p	5-year LRFS	p
Local treatment
No surgical resection or intralesional margins	11			53%
No local treatment	2	50%
RT only	6	44%
Intralesional resection and postoperative RT	3	67%
Surgical resection with at least marginal margins	45			83%	0.021**
with no RT	30	87%
with preoperative RT	4	38%
with postoperative RT	11	90%	0.050*
Site
Axial	28	70%
Peripheral	29	82%	0.193
Bone or soft tissue tumor
Bone tumor	38	75%
Soft tissue tumor	19	82%	0.814
Size of primary tumor
0–8 cm	26	92%
> 8 cm	31	66%	0.066
Neoadjuvant chemotherapy
No	9	65%
Yes	47	80%	0.356

RT, radiation therapy.

*The borderline p = 0.050 applies to the differences between the six subgroups of local treatments.

**The p = 0.021 applies to the difference between the two main groups “No surgical resection or intralesional margins” and “Surgical resection with at least marginal margins”.

Figure 2. Five-year disease-free survival according to presence of metastases at diagnosis.

Table IV. The five-year DSS of patients with metastatic ESFT.

	No. pts.	5-year DSS	p
Sex
Male	15	36%	0.742
Female	4	25%
Age at diagnosis
0–18 years	9	53%	0.089
> 18 years	10	13%
Site of primary tumor
Axial	10	30%	0.301
Peripheral	9	31%
Bone or soft tissue tumor
Bone	11	55%	0.121
Soft tissue	8	0%
Size (n = 17)
0–8 cm	9	53%	0.194
> 8 cm	8	17%
Neoadjuvant chemotherapy (n = 14)*
No	3	0%	0.008
Yes	11	58%
Response to neoadjuvant therapy (n = 7)**
Poor	4	50%	0.919
Good	3	67%
HD-chemotherapy (n = 17)†
No	8	0%	< 0.001
Yes	9	74%
Total lung irradiation†
No	14	25%	0.074
Yes	3	100%
Surgical resection with at least marginal margins (n = 17)†
No	10	20%	0.052
Yes	7	64%
Time of diagnosis
1990–1999	6	17%	0.492
2000–2009	13	46%

* N = 14, two patients died in <3 months and three did not receive local treatment for primary treatment.

*Those who received neoadjuvant therapy and were operated and the data was available.

^† The two patients who died in less than 3 months were not included.

DSS, disease-specific survival; ESFT, Ewing’s sarcoma family of tumors.