Presentation is loading. Please wait.

Presentation is loading. Please wait.

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;

Similar presentations


Presentation on theme: "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;"— Presentation transcript:

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: EM Cytogenetic and molecular analysis: fresh tissue of case-1 and paraffin-embedded tissue of case-2

4 Case-1Case-2Case-3Case-4 Age/sex6/F 2/M14/F S/s Intermittent, self-limited epigastric pain RUQ painRUQ massLUQ 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/mL3.9 ng/mL normal D/D Teratoma, granuloma, cacified hemagnioma hepatoblastom a

5

6 Case-1Case-2Case-3Case-4 Operation method Wedge resection lymph node sampling resection Partial hepatectomy Left lobectomy Adjuvant theraphy --C/T F/uDx 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 x 6.3 x x 5.0 x x 13 x 8.5

7

8 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

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

10

11

12

13

14 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

15 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

16 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

17

18

19

20 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

21

22

23

24

25

26

27

28

29

30

31

32 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)

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

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

35 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

36 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: female

37 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

38 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


Download ppt "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;"

Similar presentations


Ads by Google