Tosun Hacı Bayram, Serbest Sancar, Turk Bilge Aydın, Gumustas Seyit Ali, Uludag Abuzer
Department of Orthopaedics and Traumatology, Faculty of Medicine, Adıyaman University, Adıyaman, Turkey.
Department of Orthopaedics and Traumatology, Faculty of Medicine, Kırıkkale University, Kırıkkale, Turkey.
Int Med Case Rep J. 2015 Oct 28;8:267-71. doi: 10.2147/IMCRJ.S92015. eCollection 2015.
Malignant peripheral nerve sheath tumors (MPNSTs) are rare sarcomas of children and adolescents, and they are aggressive tumors with a high rate of local recurrence. We present a 15-year-old boy with neurofibromatosis type 1 (NF1), who had a giant MPNST on the right thigh taking into account the available literature. Diagnosis of MPNST may be delayed in NF1 patients due to confusion with a neurofibroma and/or a plexiform neurofibroma. Malignancy should be considered, especially in cases with big masses, with heterogeneous involvement, or in the presence of cysts or necrotic nodules. The aim of surgical treatment is complete surgical excision.
恶性外周神经鞘瘤（MPNSTs）是儿童和青少年中罕见的肉瘤，是具有高局部复发率的侵袭性肿瘤。考虑到现有文献，我们报告了一名15岁患有1型神经纤维瘤病（NF1）的男孩，其右大腿有一个巨大的MPNST。由于与神经纤维瘤和/或丛状神经纤维瘤混淆，NF1患者中MPNST的诊断可能会延迟。应考虑恶性情况，特别是在肿块较大、累及不均一或存在囊肿或坏死结节的情况下。手术治疗的目的是完整的手术切除。
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