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Table 3 Morphology of SEF

From: Primary sclerosing epithelioid fibrosarcoma of kidney with variant histomorphologic features: report of 2 cases and review of the literature

Typical features

Macroscopy

Large, homogeneously white or white–tan, lobulated, and hard tumors

Cell size and shape

Small to medium-sized plump to epithelioid cells

Cytoplasm

Scant clear or eosinophilic cytoplasm

Nuclei

Oval to slightly elongate angulated nuclei with finely speckled chromatin

Cellularity

Variable within the neoplasm

Atypia

Mild

Matrix

Densely sclerotic, areas of metaplastic bone in some

Pattern of cellular arrangement

Small clusters, nests and anastomosing cords

Hybrid morphology

Areas of LGFMS or nodules of collagen reminiscent of HSCTGR in some

Immunohistochemistry

MUC4 ++, EMA +/−, CD99 +/−, bcl2 +/−, pan-keratin -, S100/HMB45/MelanA -

FISH

EWSR1 (or rarely FUS) rearrangements

Previously unrecognized features in renal SEF

Current case 1

A lobular or micronodular architecture due to neoplastic cells surrounding entrapped renal tubules in a concentric fashion

Zonation in the neoplastic lobules around tubules with inner hypocellular and outer hypercellular appearance

Current case 2

Exuberant epithelial hyperplasia and small gland budding in the entrapped native renal tubules, mimicking MEST

High grade cytologic atypia

  1. LGFMS Low grade of fibromyxoid sarcoma, HSCTGR Hyalinizing spindle cell tumor with giant rosettes, MEST Mixed epithelial and stromal tumor of kidney