CTICasesMetastatic Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type 1
Imaging findings
Chest radiograph, CT, and thigh MRI demonstrate multiple well-circumscribed, rounded, bilateral pulmonary nodules of varying sizes, along with numerous cutaneous and subcutaneous soft-tissue nodules. MRI of the thigh reveals a large, heterogeneous, aggressively infiltrating soft-tissue mass with peripheral skin lesions and extensive necrotic components.
Key takeaways
Malignant peripheral nerve sheath tumors are highly aggressive sarcomas that can arise from pre-existing plexiform neurofibromas in patients with neurofibromatosis type 1. These tumors characteristically metastasize to the lungs, presenting as multiple well-circumscribed, rounded hematogenous "sarcoma-like" nodules, with the underlying neurofibromatosis type 1 diagnosis often visible on chest imaging via benign cutaneous neurofibromas.
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