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.
Highlights
Malignant peripheral nerve sheath tumours are known as an aggressive and rare malignant soft tissues neoplasm [1,2,3]
Malignant peripheral nerve sheath tumours 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
Extremely rapid growth of cutaneous exophytic malignant peripheral nerve sheath tumours over the lower back
Summary
Malignant peripheral nerve sheath tumours are known as an aggressive and rare malignant soft tissues neoplasm [1,2,3] It affects 1 in 100,000 people and approximately 3%-4.6% of patients with neurofibromatosis [4]. Malignant peripheral nerve sheath tumours have a wide variety of histologic features and different reported names, including malignant neurilemmoma, neurofibrosarcoma, neurogenic sarcoma, and malignant schwannoma [5] It developed from neurofibromas, especially in patients’ age groups between the second and sixth decades. This article reports a 56-year-old Malay female with underlying neurofibromatosis type I, presented with a rare giant cutaneous exophytic lower back malignant peripheral nerve sheath tumour measuring (15×15 cm) surgically excised. Mass sent for histopathology, which reported malignant tumour cells composed of spindleshaped cells embedded in densely cellular fascicles, alternate with hypo-cellular myxoid areas, confirming the diagnosis of malignant peripheral nerve sheath tumour
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
