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2 "Primitive neuroectodermal tumor (PNET)"
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Case Reports
Pathologic Study of Primitive Neuroectodermal Tumor: Report of 5 cases.
Sook Nyo Lee, Jong Eun Joo, Dong Soo Suk, Hyung Dong Kim, Soo Hyu Kim, Jae Hong Sim, Je G Chi
Korean J Pathol. 1989;23(3):331-341.
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AbstractAbstract PDF
Primitive neuroectodermal tumor (PNET) occuring in the brain (especially in the cereburm) of young individuals is a rare and highly malignant neoplasm. The authors analyzed 5 cases of primitive neuroectodermal tumor, operated from January 1986 to September 1987. They had characteristic clinical, radiologic and pathologic features. Patient's age ranged from 2 years to 14 years old (mean 8.2 years old) and there were 3 boys and 2 girls. They were rapidly growing tumor, with a brief duration of symptoms taking a rapidly progessive course. Computerized tomographic findings were characteristic, revealing the large, irregular, typically iso-to hyper-dense mass lesions with calcification and cystic or necrotic areas, and showing dense heterogeneous contrast enhancement of the mass in the cerebral hemisphere. Grossly all of the 5 cases showed hemorrhage and necrosis with sharp border and 2 cases showed calcification and cystic change grossly. Microscopically, they were predominantly composed of undifferentiated small dark cells with evidence of focal differentiation along glial and or neuronal lines. Four cases showed glial differentiation and three cases showed neuronal differentiation. Mesenchymal components were predominant in 3 cases.
Primitive Neuroectodermal Tumor of the Kidney: A case report .
Sang Yong Song, Eun Youn Cho, Jung Won Lee, Jai Hyang Go, Mi Kyung Kim, Dae Shick Kim, Young Hyeh Ko
Korean J Pathol. 1998;32(3):231-236.
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AbstractAbstract PDF
Peripheral primitive neuroectodermal tumor (pPNET), a rare, highly aggressive neoplasm of indetermined histogenesis, occurs typically in the soft tissues of the chest wall and the paraspinal region. Comprehensive diagnostic studies including histological, ultrastructural, immunohistochemical and molecular analyses have been stressed to diagnose this entity. We report a case of primary renal PNET which was incidentally found in a 59-year-old man who presented with generalized weakness for 4 months. He was diagnosed as a non-insulin dependent diabetes mellitus 15 years ago and has been made well by oral therapy. An ill-defined mass, measuring 3.5 3 cm, located in the left kidney and perirenal fat, was incidentally found by ultrasonogram during a renal diabetic examination. The mass was resected because of the unresponsiveness against one-year chemotherapy and radiation therapy. Grossly, a homogeneously solid, gray-white mass, measuring 2.8 1.8 cm, was noted in the mid portion of renal cortex. The mass showed severe adhesion to the perirenal fatty tissue. Microscopically, tumor cells were rather uniform, small round with scanty cytoplasm and often showed rosette formation. Ultrastructurally, they showed membrane-bound dense core granules, measuring 125~150 nm, intercellular junctions and microvillous cytoplasmic projections. The tumor cells were uniformly immunoreactive for neuron-specific enolase and were focally immunoreactive for CD99 (013), chromogranin, synaptophysin and cytokeratin. They were not reactive for S-100 protein, vimentin, Leu-7, leukocyte common antigen, desmin and smooth muscle actin. To our knowledge, this is the smallest renal PNET in literature.

J Pathol Transl Med : Journal of Pathology and Translational Medicine
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