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

• ? 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

• 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

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