Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1

ABSTRACT

Objective

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.

RESUMO

Objetivo

Relatamos a experiência institucional no tratamento de tumores malignos da bainha de nervo periférico (TMBNP) e comparamos o prognóstico entre pacientes com e sem neurofibromatose tipo 1 (NF1).

Métodos

Foram incluídos neste estudo 92 pacientes num período de 20 anos. Foi realizada uma análise retrospectiva dos prontuários, das características do tumor e do tratamento. A idade mediana era 43,5 anos (variação 3–84 anos) e 55,4% dos pacientes eram mulheres; 41 pacientes (44,6%) tinham tumores associados à NF1.

Resultados

O diâmetro médio dos tumores era 15,8 ± 8,2cm e 10,8 ± 6,3cm para pacientes com e sem NF1, respectivamente. A sobrevida combinada em 2 e 5 anos foi de 48,5% e 29%. A análise multivariada confirmou que o tamanho do tumor acima de 10cm (hazard ratio (HR) 2.99; 95% intervalo de confiança (IC) 1.14–7.85; p = 0.0258) e a presença de NF1 (HR 3.41; 95%IC 1.88–6.19; p < 0.001) estão associados a uma pior sobrevida.

Conclusões

O tamanho do tumor e a associação com NF1 foram os preditores mais importantes de sobrevida na nossa população.

nerve sheath tumors - neurofibromatosis 1 - survival

neoplasias de bainha neural - neurofibromatose tipo 1 - sobrevivência

• References

• 1 Pisters PW, Leung DH, Woodruff J, Shi W, Brennan MF. Analysis of prognostic factors in 1,041 patients with localized soft tissue sarcomas of the extremities. J Clin Oncol. 1996;14(5):1679-89. https://doi.org/10.1200/JCO.1996.14.5.1679
• 2 Stucky CC, Johnson KN, Gray RJ, Pockaj BA, Ocal IT, Rose PS et al. Malignant peripheral nerve sheath tumors (MPNST): the Mayo Clinic experience. Ann Surg Oncol. 2012;19(3):878-85. https://doi.org/10.1245/s10434-011-1978-7
• 3 Scheithauer BW, Louis DN, Hunter S, Woodruff JM, Antonescu CR. Malignant peripheral nerve sheath tumor (MPNST). In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. WHO Classification of tumours of the central nervous system. 3rd ed. Geneva: WHO Press; 2007. p. 160-2.
• 4 Anghileri M, Miceli R, Fiore M, Mariani L, Ferrari A, Mussi C et al. Malignant peripheral nerve sheath tumors: prognostic factors and survival in a series of patients treated at a single institution. Cancer. 2006;107(5):1065-74. https://doi.org/10.1002/cncr.22098
• 5 LaFemina J, Qin LX, Moraco NH, Antonescu CR, Fields RC, Crago AM et al. Oncologic outcomes of sporadic, neurofibromatosis-associated, and radiation-induced malignant peripheral nerve sheath tumors. Ann Surg Oncol. 2013;20(1):66-72. https://doi.org/10.1245/s10434-012-2573-2
• 6 Zou C, Smith KD, Liu J, Lahat G, Myers S, Wang WL et al. Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome. Ann Surg. 2009;249(6):1014-22. https://doi.org/10.1097/SLA.0b013e3181a77e9a
• 7 Durbin AD, Ki DH, He S, Look AT. Malignant peripheral nerve sheath tumors. Adv Exp Med Biol. 2016;916:495-530. https://doi.org/10.1007/978-3-319-30654-4_22
• 8 Kolberg M, Høland M, Agesen TH, Brekke HR, Liestøl K, Hall KS et al. Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1. Neuro-oncol. 2013;15(2):135-47. https://doi.org/10.1093/neuonc/nos287
• 9 National Institutes of Health Consensus Development Conference Statement: neurofibromatosis; Bethesda, Md, USA, July 13-15, 1987. Neurofibromatosis. 1988;1(3):172-8.
• 10 Edge SB, Byrd DR, Compton CC, editors. AJCC cancer staging manual. 7th ed. New York: Springer; 2010.
• 11 Massarweh NN, Dickson PV, Anaya DA. Soft tissue sarcomas: staging principles and prognostic nomograms. J Surg Oncol. 2015;111|(5):532-9. https://doi.org/10.1002/jso.23851
• 12 Basch E, Abernethy AP, Mullins CD, Reeve BB, Smith ML, Coons SJ et al. Recommendations for incorporating patient-reported outcomes into clinical comparative effectiveness research in adult oncology. J Clin Oncol. 2012;30(34):4249-55. https://doi.org/10.1200/JCO.2012.42.5967
• 13 Davidson D, Barr RD, Riad S, Griffin AM, Chung PW, Catton CN et al. Health-related quality of life following treatment for extremity soft tissue sarcoma. J Surg Oncol. 2016;114(7):821-7. https://doi.org/10.1002/jso.24424
• 14 Furtado S, Grimer RJ, Cool P, Murray SA, Briggs T, Fulton J et al. Physical functioning, pain and quality of life after amputation for musculoskeletal tumours: a national survey. Bone Joint J. 2015;97-B(9):1284-90. https://doi.org/10.1302/0301-620X.97B9.35192
• 15 Vranceanu AM, Merker VL, Park E, Plotkin SR. Quality of life among adult patients with neurofibromatosis 1, neurofibromatosis 2 and schwannomatosis: a systematic review of the literature. J Neurooncol. 2013;114(3):257-62. https://doi.org/10.1007/s11060-013-1195-2