von Recklinghausen病に合併したmalignant peripheral nerve sheath tumor(MPNST)の1例

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  • A case of malignant peripheral nerve sheath tumor associated with von Recklinghausen's disease.

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The imprint cytologic features of malignant peripheral nerve sheath tumor (MPNST) are reported. The patient, a 40-year-old woman, with von Recklinghausen's disease noticed a gradually enlarging subcutaneous nodule on the right shoulder. CT revealed the nodule was a tumor, measuring 9 cm in diameter. The imprint smear of the surgically resected tumor consisted of loosely cohesive groups of spindle-shaped cells and isolated cells. Most of the tumor cells had fusiform nuclei with coarse granular chromatin and indistinct, fibrillary cytoplasm, including cells with twisted nuclei and comma-shaped cells. Moreover, some isolated cells showing large and pleomorphic nuclei with granular chromatin and a few multinucleated cells were found. Intranuclear vacuoles were occasionally observed in these anaplastic cells. Histologic sections demonstrated swirling and interlacing fascicle patterns of spindle-shaped cells. Multinucleated and pleomorphic cells with intranuclear vacuoles were also found around the necrotic areas in the tumor. The majority of the tumor cell were positive for S-100 protein immunostaining.

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