Frequency of Certain Established Risk Factors in Soft Tissue Sarcomas in Adults: A Prospective Descriptive Study of 658 Cases

Table 2

Sarcomas associated with genetic syndromes.

Sex ratio	13 males/6 females
Age	Median: 37.5 (18–64)
	Mean: 37.5 (+/− 14)
Parameter	Number of cases	Percentage	95%-CI
Recklinghausen disease	14	73.6	56–95
Bilateral retinoblastoma	2	10.5	0–23
Familial polypadenomatosis	1	5.2	0–14
Gorlin syndrome	1	5.2	0–14
Li-Fraumeni syndrome	1	5.2	0–14
MPNST	7	36.8	18–61
Undifferentiated sarcoma	4	15.7	2–37
Leiomyosarcoma	2	10.5	6–30
Synovialosarcoma	2	10.5	6–30
Angiosarcoma	1	5.2	0–14
Fibrosarcoma	1	5.2	0–14
Aggressive fibromatosis	1	5.2	0–14
Rhabdomyosarcoma	1	5.2	0–14
Grade 1	0	0	0–0
Grade 2	3	27.2	5–49
Grade 3	8	72.8	50–99
Chest wall	8	42.1	14–56
Lower limb	5	26.3	10–50
Head and neck	3	15.8	0–30
Abdominal wall	2	10.5	0–30
Pulmonary artery	1	5.2	0–14