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Presented by Ralph Hruban, M.D. and prepared by Bahram R. Oliai, M.D.
Case 2: This 23-year-old male with von Recklinghausens neurofibromatosis presented with leg pain.
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Week 65: Case 2
This 23-year-old male with von Recklinghausens neurofibromatosis presented with leg pain./images/01-48642a.jpg
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/images/01-48642d.jpgCorrect
Answer: Malignant peripheral nerve sheath tumor
Histology: This spindle cell neoplasm has several components. In some areas it had the typical appearance of a neurofibroma with serpentine nuclei, numerous mast cells and a “shredded carrots” appearance to the collagen. In other areas, the neoplasm is more cellular and showed significant cytological atypia. Finally, focal glandular differentiation was present. The spindle cell component expressed S-100 protein and the glandular component cytokeratin.
Discussion: The neurofibromatous component of this neoplasm helps establish the diagnosis of a malignant peripheral nerve sheath tumor (MPNST) as does the expression of S-100 protein. The finding of glandular differentiation in a MPNST has been reported by Dr. J.M. Woodruff (Cancer 1992,72:3549-3552). The presence of focal epithelial differentiation in a MPNST should not be surprising considering their neural crest origin. Importantly, this phenomenon should be recognized and MPNSTs with glandular differentiation should not be confused with synovial sarcoma.
Incorrect
Answer: Malignant peripheral nerve sheath tumor
Histology: This spindle cell neoplasm has several components. In some areas it had the typical appearance of a neurofibroma with serpentine nuclei, numerous mast cells and a “shredded carrots” appearance to the collagen. In other areas, the neoplasm is more cellular and showed significant cytological atypia. Finally, focal glandular differentiation was present. The spindle cell component expressed S-100 protein and the glandular component cytokeratin.
Discussion: The neurofibromatous component of this neoplasm helps establish the diagnosis of a malignant peripheral nerve sheath tumor (MPNST) as does the expression of S-100 protein. The finding of glandular differentiation in a MPNST has been reported by Dr. J.M. Woodruff (Cancer 1992,72:3549-3552). The presence of focal epithelial differentiation in a MPNST should not be surprising considering their neural crest origin. Importantly, this phenomenon should be recognized and MPNSTs with glandular differentiation should not be confused with synovial sarcoma.
