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Malignant Peripheral Nerve Sheath Tumors of the Bilateral Adrenal Glands: Are They Metachronous Primary Tumors - A Case Report -

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초록

Malignant peripheral nerve sheath tumors (MPNSTs) have rarely been reported to occur in the adrenal gland and all of the reported cases were associated with neurofibromatosis, pheochromocytoma or ganglioneuroma. We present here a case of MPNST in the bilateral adrenal glands without any history of neurofibromatosis or combined tumor. Histologic examination showed the tumor cells had a spindle to ovoid shape, they were arranged in sweeping fascicles and there were frequent mitotic figures. The immunohistochemical and ultrastructural features of the tumor are also presented. To the best of our knowledge, this is the first report in the English medical literature about MPNSTs in the bilateral adrenal glands without any history of neurofibromatosis or combined tumor.

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) have rarely been reported to occur in the adrenal gland and all of the reported cases were associated with neurofibromatosis, pheochromocytoma or ganglioneuroma. We present here a case of MPNST in the bilateral adrenal glands without any history of neurofibromatosis or combined tumor. Histologic examination showed the tumor cells had a spindle to ovoid shape, they were arranged in sweeping fascicles and there were frequent mitotic figures. The immunohistochemical and ultrastructural features of the tumor are also presented. To the best of our knowledge, this is the first report in the English medical literature about MPNSTs in the bilateral adrenal glands without any history of neurofibromatosis or combined tumor.

참고문헌 (5)

  1. Chandrasoma P, "Malignant peripheral nerve sheath tumor arising in an adrenal ganglioneuroma in an adult male homosexual", Cancer, 57, pp.2022~2025 (1986)
  2. Miettinen M, "Pheochromocytoma combined with malig-nant schwannoma: unusual neoplasm of the adrenal medulla", Ultrastruct Pathol, 12, pp.513~527 (1988)
  3. Min KW, "Malignant peripheral nerve sheath tumor and pheochromocytoma: a composite tumor of the adrenal", Arch Pathol Lab Med, 112, pp.266~270 (1988)
  4. Sakaguchi N, "A case of von Recklinghausen’s disease with bilateral pheochromocytomamalignant peripheral nerve sheath tumors of the adrenal and gastrointestinal autonomic nerve tumors", Am J Surg Pathol, 20, pp.889~897 (1996)
  5. Bolande RP, "The neurocristopathies: a unifying concept of disease arising in neural crest maldevelopment", Hum Pathol, 5, pp.409~429 (1974)

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