Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1

In this study, we review the institution’s experience in treating malignant peripheral nerve sheath tumors (MPNSTs). A secondary aim was to compare outcomes between MPNSTs with and without neurofibromatosis type 1 (NF1).

Methods

Ninety-two patients with MPNSTs, over a period of 20 years, were reviewed. A retrospective chart review was performed. The median age was 43.5 years (range, 3–84 years) and 55.4% were female; 41 patients (44.6%) had NF1-associated tumors.

Results

Mean tumor sizes were 15.8 ± 8.2 cm and 10.8 ± 6.3 cm for patients with and without NF1, respectively. Combined two- and five-year overall survival was 48.5% and 29%. Multivariate analysis confirmed the association of tumor size greater than 10 cm (hazard ratio (HR) 2.99; 95% confidence interval (CI) 1.14–7.85; p = 0.0258) and presence of NF1 (HR 3.41; 95%CI 1.88–6.19; p < 0.001) with a decreased overall survival.

Conclusion

Tumor size and NF1 status were the most important predictors of overall survival in our population.

nerve sheath tumors; neurofibromatosis 1; survival

Characteristics	NF1-associated	Sporadic	p-value
Gender
Male	23 (56.1%)	18 (35.3%)	0.046
Female	18 (43.9%)	33 (64.7%)	NS
Age at diagnosis, yr, (median)	6–68 (36.2)	3– 84 (48.9)	0.001
Presentation
Mass	29 (70.8%)	37 (72.5%)	NS
Pain	11 (26.8%)	8 (15.7%)
Ulceration	1 (2.4%)	3 (5.9%)
Hemorrhage	-	2 (3.9%)
Skin macula	-	1 (2%)
Tumor size
< 5 cm	3 (7.3%)	11 (21.6%)	0.003
5–10 cm	7 (17.1%)	16 (31.4%)
> 10 cm	30 (73.2%)	18 (35.3%)
Missing	1 (2.4%)	6 (11.8%)
Tumor location
Extremity	24 (58.5%)	33 (64.7%)	NS
Trunk	13 (31.7%)	11 (21.6%)
Head and neck	4 (9.8%)	7 (13.7%)
AJCC grade
IA	1 (2.4%)	3 (5.9%)	NS
IB	1 (2.4%)	4 (7.8%)
IIA	2 (5%)	6 (11.8%)
IIB	1 (2.4%)	3 (5.9%)
III	30 (73.2%)	35 (68.6%)
IV	6 (14.6%)	-
Treatment goal
Curative	16 (39.1%)	40 (78.4%)	0.01
Palliative	25 (60.9%)	11 (21.6%)	0.01
Follow-up, mo, mean	24.8	46.5	0.0001

Prognostic factor	OS (mean) (mo)	p-value	DFS (mean) (mo)	p-value
NF1-status
With	47.4	<0.0001	52.5	0.17
Without	104.6	<0.0001	76.5	0.17
Age
< 45 yr	90.2	0.42	78.4	0.54
> 45 yr	82.8	0.42	60.7	0.54
Gender
Male	69.6	0.12	53.2	0.11
Female	91.6	0.12	81.6	0.11
Tumor location
Trunk	66.4	0.16	68.7	0.53
Extremity	107		51.5
Head and neck	37.1		36.2
Tumor size
< 5 cm	75.8	0.005	75.1	0.041
5–10 cm	94.5		75.5
> 10 cm	56.2		60.8
Treatment goal
Palliative	13.8	0.0001	-	-
Curative	134.3	0.0001	-	-

Variable	HR	95%CI	p-value
Model 1
NF1	3.33	1.91–5.8	0.0000214
Model 2
NF1	3.41	1.88–6.19	0.0000547
Tumor size > 10 cm	2.99	1.14–7.85	0.0258
Model 3
NF1	2.4	1.25–4.6	0.0084
Tumor size > 10 cm	2.21	0.82–5.92	0.12
Curative treatment	0.21	0.11–0.42	0.00000523

Academia Brasileira de Neurologia - ABNEURO R. Vergueiro, 1353 sl.1404 - Ed. Top Towers Offices Torre Norte, 04101-000 São Paulo SP Brazil, Tel.: +55 11 5084-9463 | +55 11 5083-3876 - São Paulo - SP - Brazil
E-mail: revista.arquivos@abneuro.org

Acompanhe os números deste periódico no seu leitor de RSS

[1] Correspondence: Roberto André Torres de Vasconcelos; Rua Equador, 831; 20220-410 Rio de Janeiro RJ; Brasil; E-mail: ortovasconcelos@ig.com.br