Desmoplastic Nested Sp indle Cell Tumor of Li ver: Report of Four Ca ses of a Proposed New Entity Hill,D Ashley MD; Swanson, Paul E MD; Anderso n, Keith MD; Covinsky, Michael H MD, Phd; Fin n, Laura S MD; Ruchelli, Eduardo D MD; Nascim ento, Antonio G MD; Langer, Jacob C MD; Minke s, Robert K MD; McAlister, William MD; Dehner, Louis P MD ~AJSP 2005 January
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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;
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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
Background
• Pediatric hepatic tumor: a unique age-specific neoplasm
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 st
romal tumor• Ishak et al: ossifying stromal-epithelial tum
ors(OSET)
D/D
• ? Hepatoblastoma: lack fetal or embrynoic hepatoid component, OCH1E5(-)
• ? Synovial sarcoma, thyroid medullary ca, meta spindle cell carcinoid, SETTLE, spindle neuroendocrine carcinoma
Discussion
• ? SETTLE
• ? DSRCT: no malignant round cell, desmin(-), WT-1 protein N-terminal(+)
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
Discussion
• Ectopic ACTH is most commonly observed in neuroendocrine tumors of the lung( carcinoid, small cell carcinoma) and other site
• 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
Discussion
• Unique clinicopathologic entity • Histogenesis and pathogenesis are uncertain• Speculate arising from a primitive mesench
ymal cell with the expression of WT-1 protein reflecting some mesenchymal to epithelial transition abnormlity
• Slowly growing but with unknown malignant potential