Second malignancies in patients with Ewing Sarcoma Family of Tumors: A population-based study

Figures & data

Table I. Ewing Sarcoma Family of Tumors cases included in the analysis by sex, race, age, stage, therapy, site and period of diagnosis, stratified by having or not having a second cancer.

	Patients without second cancer		Patients with second cancer
	N	(%)	N	(%)	P
Total	1131		35
Sex
Female	439	(39)	19	(54)	0.095
Race
Black	23	(2)	1	(3)	0.87
White	1035	(92)	31	(89)
Others	73	(7)	3	(9)
Age at first diagnosis
0 to 9	162	(14)	7	(20)	0.53
10 to 19	558	(49)	16	(46)
20 to 29	228	(20)	8	(23)
30 to 39	87	(8)	1	(3)
49 to 49	47	(4)	0	(0)
50 to 59	22	(2)	1	(3)
60 +	27	(2)	2	(6)
Metastatic
Yes	313	(28)	7	(20)	0.51*
No	709	(63)	23	(66)
Unknown	109	(10)	5	(14)
Radiation
Received	615	(54)	23	(66)	0.042*
None	478	(42)	7	(20)
Unknown/others	38	(3)	5	(14)
Site
Bone	893	(79)	29	(83)	0.73
Soft tissue	238	(21)	6	(17)
Period of incidence
1973 through 1985	371	(33)	19	(54)	0.0065
1986 through 1995	333	(29)	9	(26)
1996 through 2005	427	(38)	7	(20)

Percentages may not add to 100% due to approximation.

*, excluding unknown/others.

Table II. Details of second cancer (n = 35) that were recorded in 1 166 patients with Ewing Sarcoma.

Lymphatic and hematopoietic	Solid Tumors
9663/3: Hodgkin lymphoma, nodular sclerosis, NOS (0, A, 124) & (86, D, 192)	8041/3: Small cell carcinoma, NOS (105, A, 17)
9680/3: NHL, large B-cell, diffuse (0, A, 260)	8050/3: Papillary carcinoma, NOS (156, A, 144)
9732/3: Multiple myeloma (24, D, 20)	8071/3: Squamous cell carcinoma, keratinizing, NOS (192, A, 117)
9835/3: Precursor cell lymphoblastic leukemia, NOS (18, D, 3)	8140/3: Adenocarcinoma, NOS (85, A, 53)
9860/3: Myeloid leukemia, NOS (46, D, 0)	8310/3: Clear cell adenocarcinoma, NOS (147, A, 8)
9861/3: Acute myeloid leukemia (29, D, 0)	8500/3: Infiltrating duct carcinoma, NOS (251, D, 20) & (70, A, 49)
9867/3: Acute myelomonocytic leukemia (68, A, 46)	8522/3: Infiltrating duct and lobular carcinoma (290, A, 65)
9872/3: Acute myeloid leukemia, minimal differentiation (42, D, 1)	8720/3: Malignant melanoma, NOS (303, A, 13)
9891/3: Acute monocytic leukemia (44, D, 33) & (33, A, 209)	8800/3: Sarcoma, NOS (98, A, 199)
9920/3: Therapy-related acute myeloid leukemia, NOS (39, D, 3)	8830/3: Fibrous histiocytoma, malignant (64, D, 5) & (87, A, 1)
	8832/3: Dermatofibrosarcoma, NOS (1, A, 221)
	8850/3: Liposarcoma, NOS (182, D, 105)
	8930/3: Endometrial stromal sarcoma (15, D, 13)
	9064/3: Germinoma (317, D, 22)
	9081/3: Teratocarcinoma (46, D, 157)
	9180/3: Osteosarcoma, NOS (218, A, 69) & (171, A, 209)
	9181/3: Chondroblastic osteosarcoma (57, A, 117) & (89, A, 235)
	9500/3: Neuroblastoma, NOS (72, A, 11)
	9503/3: Neuroepithelioma, NOS (0, D, 18)

All records are preceded by ICD-O-3 code and followed by the following data: months since index case, status at study cutoff (alive [A] or dead [D]), and survival time in months (from diagnosis of 2^nd cancer until death or study cut-off).

Table III. Patients with second tumor by age at diagnosis, therapy, period of diagnosis of primary tumor, time to develop second cancers, age at diagnosis of second cancer and its outcome, stratified by type of tumor.

	All 2^nd cancers		Solid tumors		Lymphatic/hematopoieticmalignancies
	Variable	N	(%)	N	(%)	N	(%)
Number	35		23		12
Age at diagnosis of Ewing Sarcoma
Median	15		14		19		0.52
Range	3 to 71		3 to 71		7 to 34
Radiation
Beam	23	(66)	17	(74)	6	(50)	0.066
None	7	(20)	5	(22)	2	(17)
Unknown/others period of incidence	5	(14)	1	(4)	4	(33)
1973 through 1985	19	(54)	16	(70)	3	(25)	0.043
1986 through 1995	9	(26)	4	(17)	5	(42)
1996 through 2005	7	(20)	3	(13)	4	(33)
Time to develop 2nd cancer
< 1 year	4	(11)	2	(9)	2	(17)	0.0032
1 to 5 years	11	(31)	3	(13)	8	(67)
5 to 10 years	10	(29)	10	(43)	0	(0)
10 + years	10	(29)	8	(35)	2	(17)
Duration from 1st to 2nd cancer
Median (months)	70		98		36		0.0007
Range	0 to 317		0 to 317		0 to 86
Age at 2nd malignancy
Median	26		29		23.5		0.33
Range	10 to 77		10 to 77		10 to 36
5-year survival (since 2nd cancer)	62% ± 8.7%		74% ± 10%		42% ± 14%		0.068

Percentages may not add to 100% due to approximation.

Figure 1. Probability of developing second solid tumors and lymphatic/hematopoietic (hematologic) malignancies following diagnosis of Ewing Sarcoma Family of Tumors in 1 166 patients, calculated using Kaplan-Meier method.

Table IV. Observed numbers and standardized incidence ratios of second malignancies.

	Observed	O/E	95% CI	Excess risk
All cancer (excluding non-melanoma skin cancer)	35	4.01*	2.80 to 5.58	33.18
All solid tumors	23	3.10*	1.96 to 4.64	19.66
Osteosarcoma	4	51.08*	13.74 to 130.77	4.95
Soft tissue sarcoma	6	35.56*	12.98 to 77.40	7.36
Breast	3	2.72	0.54 to 7.89	2.39
Lung and bronchus	2	2.76	0.31 to 9.96	1.61
Male Genital system	2	1.53	0.17 to 5.52	0.87
All Lymphatic and Hematopoietic	12	10.36*	5.35 to 18.10	13.69
ANLL	6	51.09*	18.66 to 111.21	7.43
Lymphoma	3	3.92	0.79 to 11.46	2.82
ALL	1	11.55	0.15 to 64.27	1.15

*, statistically significant (p < 0.05); O/E, observed-to-expected ratios; CI, confidence intervals; excess risk, for 10 000; ANLL, Acute Non-Lymphocytic Leukemia; ALL, Acute Lymphoblastic Leukemia.