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Desmoplastic Nested Spindle Cell Tumor of Liver: Report of Four Cases of a Proposed New Entity

Desmoplastic Nested Spindle Cell Tumor of Liver: Report of Four Cases of a Proposed New Entity.

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Desmoplastic Nested Spindle Cell Tumor of Liver: Report of Four Cases of a Proposed New Entity

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  1. Desmoplastic Nested Spindle Cell Tumor of Liver: Report of Four Cases of a Proposed New Entity Hill,D Ashley MD; Swanson, Paul E MD; Anderson, Keith MD; Covinsky, Michael H MD, Phd; Finn, Laura S MD; Ruchelli, Eduardo D MD; Nascimento, Antonio G MD; Langer, Jacob C MD; Minkes, Robert K MD; McAlister, William MD; Dehner, Louis P MD ~AJSP 2005 January

  2. Background • Pediatric hepatic tumor: a unique age-specific neoplasm • Hepatoblastoma, infantile hemangioendothelioma, undifferentiated embryonal sarcoma, • Nosologically unique enitity

  3. Material and method • Washington University’s Institutional Review Board • IHC: 0-+++ • EM • Cytogenetic and molecular analysis: fresh tissue of case-1 and paraffin-embedded tissue of case-2

  4. Light Microscopy • Well demarcated nests with ovoid, plump to fusiform spindle cells • With or without intermixed rounded epithelioid cells • Invested by prominent desmoplastic stroma • Well-defined membrane, chromatin fine • Necrosis and cystic degeneration focally • Psammomatous calcification, ossification

  5. Light microscopy • Desmoplastic stroma: variable celluar, with morphologic feature of myofibroblasts • Surrounding liver parenchyma: unremarkable, no ductal plate abnormality

  6. Cytogenetics and Molecular Anaylsis • Normal karyotype, 46xx in case-1 • RT-PCR for SYT-SSX and EWS-WT1 were negative in case-1 and case-2

  7. Nested Stromal Epithelial Tumor of the Liver: Six Cases of a Distinctive Pediatric Neoplasm With Frequent Calcification and Association With Cushing Syndrome Heerema-McKenney, Amy MD; Leuschner, Ivo MD; Smith, Nicholas FRCPath; Sennesh, Joel MD; Finegold, Milton J MD ~AJSP 2005 January

  8. Nonhepatocytic, nonbiliary liver tumor with nests of epithelial and spindle cells, myofibroblastic stroma, variable calcification and ossification • Ishak et al: ossifying stromal-epithelial tumor • All cases were received by one of the author

  9. Result • Gross: confined to the liver, not encapsulated • Irregular, multinodular borders • Homogeneous, tan, granular-appearing cut surface • Focal softening or cyst formation • Ranged 4-30 cm greatest diameter

  10. Discussion identical hepatic tumor, resembled several other pathological entities including some that are not native to the liver • Fletman et al: mixed cell type • Ossifying malignant mixed epithelial and stromal tumor • Ishak et al: ossifying stromal-epithelial tumors(OSET)

  11. D/D • ? Hepatoblastoma: lack fetal or embrynoic hepatoid component, OCH1E5(-) • ? Synovial sarcoma, thyroid medullary ca, meta spindle cell carcinoid, SETTLE, spindle neuroendocrine carcinoma

  12. Discussion • ? SETTLE • ? DSRCT: no malignant round cell, desmin(-), WT-1 protein N-terminal(+)

  13. Discussion • Origin in AFIP: young adult, calcified mass since children • Our case: all in children, localized, common histological appearance, at least focal nuclear WT-1 expression was detected in the nests, no significant nuclear pleomorphism, hyperchromasia, no atypical mitosis or vascular invasion

  14. Discussion • Ectopic ACTH is most commonly observed in neuroendocrine tumors of the lung( carcinoid, small cell carcinoma) and other site • Hepatoblastoma, cholestatic hepatic adenoma, hepatic adrenocortical tumor • Our case: 11-14 female

  15. Discussion • The two categories of tumor may ultimately prove to be related showing a spectrum of morphologic feature • Calcification difference: maturation changes with the tumor( dystrophic calcification and ossification), decreased activity at the tumor-liver interface • Prefer descriptive name DNSTL over OSET • D/D: calcified liver mass in childhood

  16. Discussion • Unique clinicopathologic entity • Histogenesis and pathogenesis are uncertain • Speculate arising from a primitive mesenchymal cell with the expression of WT-1 protein reflecting some mesenchymal to epithelial transition abnormlity • Slowly growing but with unknown malignant potential

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