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Liver: Nested stromal epithelial tumor

Written2010-04Y Albert Yeh
North Shore University Hospital, Long Island Jewish Medical Center, Hofstra University School of Medicine, New York, USA

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Identity

ICD-Topo C220 LIVER
Atlas_Id 6243
Phylum Digestive organs: Liver::Mixed epithelial and stromal tumors, malignant
Other namesOssifying malignant mixed epithelial and stromal tumor
Ossifying stromal-epithelial tumor
Desmoplastic nested spindle cell tumor
Calcifying nested stromal-epithelial tumors of the liver
Note Nested stromal epithelial tumor (NSET) of the liver is an extremely rare non-hepatocytic tumor of the liver and is characterized by nests of spindle and epithelioid cells with occasional calcification and ossification.
Synonyms include ossifying malignant mixed epithelial and stromal tumor, ossifying stromal-epithelial tumor, desmoplastic nested spindle cell tumor, and calcifying nested stromal-epithelial tumors of the liver.
Gross and histopathological characteristics of NSET. Grossly, the tumor has a yellow-tan and bulging lobulated appearance. A satellite tumor nodule is present in the periphery (A). Microscopically, nested tumor cells have oval nuclei, stippled chromatin, and inconspicuous nucleoli. The interface between epithelioid and spindle cells is shown (arrows). Courtesy of Sergey V Brodsky.

Clinics and Pathology

Epidemiology Twenty four cases of NSET of the liver have been reported. NSET occurs in patients with age range from 2 to 33 years old. The tumor affects mainly children, and females are more frequently affected than males.
Clinics Patients are presented with palpable abdominal tumors. Cushing syndrome has been reported in a few patients.
Pathology Macroscopically, NSETs predominantly occur in the right lobe of the liver and are unencapsulated, well circumscribed tumors range in size from 2.8 to 30 cm in greatest dimension. Satellite tumor nodules also have been reported. Tumors have a yellow-tan and lobulated appearance.

Microscopically, NSETs are characterized by organoid arrangement of cellular nests composed of spindle and epithelioid cells embedded in a desmoplastic or fibrocytic/myofibroblastic stroma, within which proliferation of bile ductules is noted. Areas of myxoid and cystic degeneration or necrosis are sometimes encountered within or adjacent to the cellular nests. Focal psammomatous calcification or osteoid formation is present in some tumors. Lymphovascular invasion is occasionally seen. The spindle cells within the cellular nests are arranged in short fascicles with a somewhat whorled pattern. The nested cells are characterized by plump nuclei, stippled chromatin, and inconspicuous nucleoli. Scattered mitotic figures with abnormal forms are identified.

Immunohistochemically, the tumor cells are stained positive for cytokeratin AE1/AE3, keratin CK19 (focal), EMA, CD117 (c-kit), CD56, CD99, ACTH, chromogranin, synaptophysin, neuron-specific enolase, and S100 (focally weak in epithelioid cells). Vimentin stain is positive in the nested spindled cell and stroma. Muscle specific actin and smooth muscle actin immunostains highlight stromal myofibroblastic cells. Alpha-fetoprotein and p53 are negative.

 
Immunohistochemical stains of NSET. Tumor cells are stained positive for immunostains including ACTH (A) (Courtesy of Milton J Finegold), b-catenin (B), cyclin D1 (C), Ki-67 (30% of nuclear staining) (D), p21ras (E), topoisomerase II (F).
Cytogenetics Chromosomal analysis of one tumor reveals an abnormal karyotype of 60-63,XXX,-1,-4,-5,-others,+2mar.
Prognosis Most patients are doing well with no tumor recurrence in 6 months to 14 years. Tumor recurrence has been observed in two of twenty four patients. One case with aggressive clinical behavior and extrahepatic lymph node metastasis has been reported.

Bibliography

Recurrent nested stromal epithelial tumor of the liver with extrahepatic metastasis: case report and review of literature.
Brodsky SV, Sandoval C, Sharma N, Yusuf Y, Facciuto ME, Humphrey M, Yeh YA, Braun A, Melamed M, Finegold MJ.
Pediatr Dev Pathol. 2008 Nov-Dec;11(6):469-73. Epub 2008 Feb 25.
PMID 18338937
 
Nested stromal-epithelial tumor (NSET) of the liver: a case report of an extremely rare tumor.
Grazi GL, Vetrone G, d'Errico A, Caprara G, Ercolani G, Cescon M, Ravaioli M, Del Gaudio M, Vivarelli M, Zanello M, Pinna AD.
Pathol Res Pract. 2010 Apr 15;206(4):282-6. Epub 2009 May 31.
PMID 19487085
 
Nested stromal epithelial tumor of the liver: six cases of a distinctive pediatric neoplasm with frequent calcifications and association with cushing syndrome.
Heerema-McKenney A, Leuschner I, Smith N, Sennesh J, Finegold MJ.
Am J Surg Pathol. 2005 Jan;29(1):10-20.
PMID 15613852
 
Ossifying malignant mixed epithelial and stromal tumor of the liver: a case report of a previously undescribed tumor.
Heywood G, Burgart LJ, Nagorney DM.
Cancer. 2002 Feb 15;94(4):1018-22.
PMID 11920471
 
Desmoplastic nested spindle cell tumor of liver: report of four cases of a proposed new entity.
Hill DA, Swanson PE, Anderson K, Covinsky MH, Finn LS, Ruchelli ED, Nascimento AG, Langer JC, Minkes RK, McAlister W, Dehner LP.
Am J Surg Pathol. 2005 Jan;29(1):1-9.
PMID 15613851
 
Miscellaneous malignant tumors.
Ishak KG, Goodman ZD, Stocker JT.
In: Rosai J, Sobin LH, eds. Tumors of the liver and intrahepatic bile ducts. Armed forces Institute of Pathology, Washington DC, 2001:271-2.
 
Calcifying nested stromal-epithelial tumors of the liver: a clinicopathologic, immunohistochemical, and molecular genetic study of 9 cases with a long-term follow-up.
Makhlouf HR, Abdul-Al HM, Wang G, Goodman ZD.
Am J Surg Pathol. 2009 Jul;33(7):976-83.
PMID 19363442
 
Nested (ossifying) stromal epithelial tumor of the liver: case report.
Meir K, Maly A, Doviner V, Gross E, Weintraub M, Rabin L, Pappo O.
Pediatr Dev Pathol. 2009 May-Jun;12(3):233-6.
PMID 18699697
 
Desmoplastic nested spindle cell tumor of the liver in an adult.
Oviedo Ramirez MI, Bas Bernal A, Ortiz Ruiz E, Bermejo J, De Alava E, Hernandez T.
Ann Diagn Pathol. 2010 Feb;14(1):44-9. Epub 2009 Oct 31.
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Citation

This paper should be referenced as such :
Yeh, YA
Liver: Nested stromal epithelial tumor
Atlas Genet Cytogenet Oncol Haematol. 2011;15(1):86-88.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Tumors/NestStromEpithLiverID6243.html


External links

arrayMap Topo ( C22) arrayMap ((UZH-SIB Zurich)   [auto + random 100 samples .. if exist ]   [tabulated segments]
 
 
Disease databaseLiver: Nested stromal epithelial tumor
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