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Presented by Ralph Hruban, M.D. and prepared by Lynette S. Nichols, M.D.
Case 4: This patient with neurofibromatosis presented with a large mass in the upper leg.
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Question 1 of 1
1. Question
Week 137: Case 4
This patient with neurofibromatosis presented with a large mass in the upper leg./images/Lyn’s/h4a.jpg
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/images/Lyn’s/h4d.jpgCorrect
Answer: Malignant peripheral nerve sheath tumor
Histology: This highly cellular spindle cell neoplasm had areas of necrosis and an invasive growth pattern. Numerous mitoses were present as was significant pleomorphism. Immunolabeling for the S-100 protein was positive.
Discussion: This malignant periplial nerve sheath tumor (MPNST) contrasts nicely with the benign nerve sheath tumors presented in cases 1-3. Most MPNST are high grade and resemble fibrosarcomas. Gross origin in a nerve, origin in a patient with neurofibromatosis or the expression of nerve sheath markers such as S-100 protein can help establish the diagnosis of a nerve sheath tumor. High cellularity, pleomorphism, necrosis and especially a high mitotic rates all point to a malignancy.
Incorrect
Answer: Malignant peripheral nerve sheath tumor
Histology: This highly cellular spindle cell neoplasm had areas of necrosis and an invasive growth pattern. Numerous mitoses were present as was significant pleomorphism. Immunolabeling for the S-100 protein was positive.
Discussion: This malignant periplial nerve sheath tumor (MPNST) contrasts nicely with the benign nerve sheath tumors presented in cases 1-3. Most MPNST are high grade and resemble fibrosarcomas. Gross origin in a nerve, origin in a patient with neurofibromatosis or the expression of nerve sheath markers such as S-100 protein can help establish the diagnosis of a nerve sheath tumor. High cellularity, pleomorphism, necrosis and especially a high mitotic rates all point to a malignancy.
