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A 41-year-old male with a past history of Neurofibromatosis Type 1 (NF1) presents with a painful, rapidly enlarging left thigh mass over 1 month. MRI of the left thigh showed a large lobulated plexiform neurofibroma along the course of the sciatic nerve, with a heterogeneous, rim enhancing component, worrisome for sarcomatous transformation (Fig. 1). Staging CT scan of the thorax, abdomen and pelvis did not show any distant metastasis, though there were multiple neurofibromas seen. An en-bloc resection was performed. Intraoperatively, an 11 × 11 × 32 cm mass was found, encompassing the sciatic nerve at the popliteal fossa. Histology demonstrated a malignant peripheral nerve sheath tumour, FNLCC grade 3, pT4 with uninvolved margins. The patient then underwent adjuvant RT (60Gy/ 30#) to the left thigh surgical bed.

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Malignant Peripheral Nerve Sheath Tumour (MPNST)

  • 章节

  • 发表日期：

    2025年

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  • Faimee Erwan Muhamat Nor,
  • Timothy Kwang Yong Tay,
  • Jian Bang Chiang,
  • Chung Ming Chan,
  • Mark E Puhaindran

摘要

A 41-year-old male with a past history of Neurofibromatosis Type 1 (NF1) presents with a painful, rapidly enlarging left thigh mass over 1 month. MRI of the left thigh showed a large lobulated plexiform neurofibroma along the course of the sciatic nerve, with a heterogeneous, rim enhancing component, worrisome for sarcomatous transformation (Fig. 1). Staging CT scan of the thorax, abdomen and pelvis did not show any distant metastasis, though there were multiple neurofibromas seen. An en-bloc resection was performed. Intraoperatively, an 11 × 11 × 32 cm mass was found, encompassing the sciatic nerve at the popliteal fossa. Histology demonstrated a malignant peripheral nerve sheath tumour, FNLCC grade 3, pT4 with uninvolved margins. The patient then underwent adjuvant RT (60Gy/ 30#) to the left thigh surgical bed.