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Washington University Experience | NEOPLASMS - CRANIAL AND PARASPINAL NERVEs | Malignant Peripheral Nerve Sheath Tumor (MPNST) | 4A1 MPNST (Case 4) H&E 1
Case 4 History ---- The patient is a 27-year-old man with history of neurofibromatosis type 1 and a recurrent atypical neurofibroma of the cervical soft tissues and spine. MRI shows interval increase in an intradural extramedullary mass at C1-C2 with mass effect on the spinal cord. There has been an interval increase in a second left paraspinal soft tissue mass from C1-C3 associated with necrosis and causing narrowing of the left distal cervical internal carotid artery and left vertebral artery. Operative procedure: Posterior cervical tumor resection of intradural tumor C1-C2. ---- 4A1-3 Hematoxylin and eosin stained sections of the resection show a tumor composed of alternating hypercellular fascicles and less cellular fibrous to myxoid regions. The hypercellular areas consist of crowded, variably atypical spindled cells with hyperchromatic, tapered nuclei, numerous mitoses (up to 10/10 HPF) and apoptotic bodies. Focal areas of geographic necrosis are present. There are foci of tumor cells with epithelioid differentiation, arranged in small whorled nests. Rare individual rhabdomyoblastic cells are present, as are areas of metaplastic osseous differentiation. The less cellular areas demonstrate minimally atypical, elongated, tapered/pointed, wavy, or buckled nuclei characteristic of lower grade nerve sheath tumors.
