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
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
• Hepatoblastoma, infantile hemangioendothelioma, undifferentiated embryonal sarcoma,
• Nosologically unique enitity
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
Case-1 Case-2 Case-3 Case-4
Age/sex 6/F 6/F 2/M 14/F
S/s Intermittent, self-limited epigastric pain
RUQ pain RUQ mass LUQ mass
CT Well-defined, densely calcified tumor in the right lobe
Well-defined lobulated mass in right lobe, not calcified or enhancement
Mass in right lobe with focal calcification
Mass in left lobe
other No contrast enhancement
Peripheral enhancement, necrosis
Lung benign nodule
Past hx: calcified mass in left lobe
A-FP 2.9 ng/mL 3.9 ng/mL normal
D/D Teratoma, granuloma, cacified hemagnioma
hepatoblastoma hepatoblastomahepatoblastoma
Case-1 Case-2 Case-3 Case-4
Operation method
Wedge resection lymph node sampling
resection Partial hepatectomy
Left lobectomy
Adjuvant theraphy
- - C/T C/T
F/u Dx free 4 years
Dx free 8 months
Dx fee 7 years
Dx free 7.5 years
Gross Soft, myxoid, gray tumor with fine granular calcification
Homogenenous firm and white
Dense white-tan with multiple small spicules of calcification
Multilobulated tan-white, focal myxoid area
Tumor size (cm)
2.8 x 1.5 x 1.5
7.5 x 6.3 x 4.0
5.5 x 5.0 x 4.0
15 x 13 x 8.5
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
Light microscopy
• Desmoplastic stroma: variable celluar, with morphologic feature of myofibroblasts
• Surrounding liver parenchyma: unremarkable, no ductal plate abnormality
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
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
• 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
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
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 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
• 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
• Hepatoblastoma, cholestatic hepatic adenoma, hepatic adrenocortical tumor
• Our case: 11-14 female
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