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Three of the 30 cases (10%) were clinically useful but not accurate: (1) a 4.6-cm tibial lesion diagnosed as spindle cell sarcoma by FNACBP with a subsequent diagnosis of MPNST on resection, (2) a 1.9-cm benign spindle cell acetabular lesion with a subsequent diagnosis of benign bone cyst on excision, and (3) a 3.7-cm frontal sinus, benign osteoblastic lesion subsequently diagnosed as a cranio-facial osteoma with osteoid osteoma-like nidus on excision.
MPNST occurs most commonly in deep soft tissue along the nerves that runs from the buttocks to legs, neck to arm, or with in pelvis.
This included 3 partial responses (PR), two from the sporadic MPNST patients and one from an NF1 patient, and 2 individuals with NF1 exhibiting stable disease (SD) by RECIST criteria, making the clinical benefit rate (CBR = PR + SD) 100%.
A 2002 MPNST consensus statement reviewed current knowledge, provided guidance for the diagnosis and management of MPNST, and identified research priorities .
Worse 5-year overall survival has been reported for NF1 MPNST (32%, 11%) compared to sporadic MPNST (55%, 44.7%), though, inrecentyears, studies have shown this difference is decreasing [3, 14, 15].
The final histopathology report was suggestive of MPNST. (Fig 3) Immunohistochemistry for S-100 antigen was done revealing 60% positivity.
Primary cancer was adenocarcinoma colon, adenocarcinoma pancreas and MPNST respectively which is rare.
Many previously diagnosed spindle cell tumors such as mesothelioma, fibrosarcoma, hemangiopericytoma, and even malignant peripheral nerve sheath tumor (MPNST) and undifferentiated sarcoma could well represent underdiagnosed SS in this location.
INTRODUCTION: Malignant peripheral nerve sheath tumor (MPNST) is an aggressive and uncommon neoplasm that develops within a peripheral nerve; most cases of which are associated with neurofibromatosis type 1 (NF1).
The differential diagnosis of plexiform schwannoma includes plexiform neurofibroma and malignant peripheral nerve sheath tumor (MPNST).
A novel geranylgeranyl transferase inhibitor in combination with lovastatin inhibits proliferation and induces autophagy in STS-26T MPNST cells.
Malignant peripheral nerve sheath tumors (MPNST) often produce loss of peripheral function unlike schwannomas (12).
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