Malignant peripheral nerve sheath tumor of the shoulder: A rare case highlighting the role of multimodal management

Malignant peripheral nerve sheath tumors (MPNSTs) are rare and aggressive sarcomas often associated with neurofibromatosis type 1 (NF1) or prior radiation therapy. We report the case of a 26-year-old male who presented with a rapidly enlarging, painful mass in the left shoulder initially misdiagnosed as an abscess. MRI revealed a large extra-compartmental lesion with heterogeneous enhancement infiltrating the trapezius muscle. Histopathology confirmed MPNST, showing high mitotic activity (16/10 HPF), 10% necrosis, and a Ki-67 index of 40%, with diffuse Sox10 and focal S100 positivity. The patient received neoadjuvant radiotherapy followed by complete surgical excision and defect reconstruction using a pedicled trapezius flap and a split-thickness skin graft. This case highlights the importance of early imaging, multidisciplinary planning, and multimodal treatment in the management of MPNSTs.

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Publication Details

Journal
Radiology Case Reports
Published
2025-06-17
DOI
https://doi.org/10.1016/j.radcr.2025.05.018
Primary Topic
Neurofibromatosis and Schwannoma Cases
Type
article
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article

Malignant peripheral nerve sheath tumor of the shoulder: A rare case highlighting the role of multimodal management

Moatassim Billah Nabil, Kaoutar Znati, Derqaoui Sabrine, Kaoutar Imrani et al.
1 links
Radiology Case Reports
Neurofibromatosis and Schwannoma Cases
article

Malignant peripheral nerve sheath tumor of the shoulder: A rare case highlighting the role of multimodal management

Moatassim Billah Nabil, Kaoutar Znati, Derqaoui Sabrine, Kaoutar Imrani, Guennouni Asma, Ittimad Nassar, S Abdel-Kader, Abourak Chaimae, Oukassem Siham, Iraqui Iman
article en

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are rare and aggressive sarcomas often associated with neurofibromatosis type 1 (NF1) or prior radiation therapy. We report the case of a 26-year-old male who presented with a rapidly enlarging, painful mass in the left shoulder initially misdiagnosed as an abscess. MRI revealed a large extra-compartmental lesion with heterogeneous enhancement infiltrating the trapezius muscle. Histopathology confirmed MPNST, showing high mitotic activity (16/10 HPF), 10% necrosis, and a Ki-67 index of 40%, with diffuse Sox10 and focal S100 positivity. The patient received neoadjuvant radiotherapy followed by complete surgical excision and defect reconstruction using a pedicled trapezius flap and a split-thickness skin graft. This case highlights the importance of early imaging, multidisciplinary planning, and multimodal treatment in the management of MPNSTs.

Radiology Case ReportsVol. 20(9)
Mohammed V University (MA), Ibn Sina Hospital (KW)
Openalex Percentile: Top 78%
Neurofibromatosis and Schwannoma Cases
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