Rapidly Growing Huge Lower Back Malignant Peripheral Nerve Sheath Tumour

Abstract

Malignant peripheral nerve sheath tumours (also called neurofibrosarcomas) are a rare, highly aggressive soft tissue sarcomas that arise from the peripheral nerves or cells associated with the nerve sheath, such as Schwann cells, peri-neural cells and fibroblasts. It is representing 10% of all soft tissue sarcomas in which it is considered as an extremely rare malignancy, especially in patients with neurofibromatosis type I. In the general population, it affects approximately 1 in 100,000 people. This article is reporting a 56-year-old Malay female patient who is a known case of neurofibromatosis type I for 20 years, presented with a lower back, pruritic, gradually increasing swelling during the last five months. Last month before the presentation, the lesion rapidly grows, reaching a size of (15×15 cm), accompanied by foul-smelling discharge and pain exacerbated with movement. Although no history of preceding trauma or accident, the mass bleeds within contact. In conclusion, only a few cases of giant malignant peripheral nerve sheath tumours reported in the literature describing its location and growth progression. We present a massive, extremely rapid growth of cutaneous exophytic malignant peripheral nerve sheath tumours over the lower back.

Keywords: malignant peripheral nerve sheath tumour; neurofibromatosis; neurofibrosarcomas.

Figure 1. Huge lower back malignant nerve sheath tumour with neurofibromatosis type I.

Figure 2. Longitudinal section MRI showing cutaneous pedunculated exophytic mass at the posterior pelvic region with subcutaneous fascia involvement.

Figure 3. Excised tumour mass (15x15 cm).