PMC6503595_01
Case 1: (a) macroscopic appearance of EAML occupying the left liver lobe,
with distinct nodules of FNH and leiomyoma (asterisk); (b) computed
tomography appearance of EAML (asterisk) and FNH foci of the right lobe;
(c) histological features of EAML [hematoxylin-eosin (H&E) stain, 4x
magnification] with HMB45 immunoreaction (4x magnification); (d) a
distinct leiomyoma nodule within the EAML (H&E stain, 4x
magnification), with alpha-SMA immunoreaction (2x magnification); and (e)
histological features of FNH (H&E stain, 4x magnification) with
'map-like pattern' GS immunoreactivity (2x magnification).. EAML, epithelioid angiomyolipoma; GS, glutamine synthetase; FNH, focal
nodular hyperplasia; SMA, smooth muscle actin.
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These findings suggested HCA (Figure 1(b)). The surgical specimen presented a large red-purple solid area, with yellowish nodular areas and two distinct nodules of grayish compact tissue and a central scar (Figure 1(a)). On definitive slides, some areas of the red-purple lesion were characterized by dystrophic vascular structures, with a tortuous pathway, epithelioid cells with abundant eosinophilic cytoplasm, and nuclei with prominent nucleoli (Figure 1(c)), mixed to mature adipose tissue and areas of smooth muscle tissue, with a distinct leiomyoma-like nodule (of 1.4 cm; Figure 1(d)). The two focal nodular hyperplasia (FNH) were positive for GS with a map-like pattern and negative for HMB45 (Figure 1(e)).
PMC6503595_02
Case 2: (a) PEComa: epithelioid cells and spindle cells in a solid and
nested architecture, with scattered mature adipocytes and a rich
vascularization (H&E stain, 4x); tumor cells are diffusively
positive for HMB45 (b) with scant cells positive for alpha-SMA (c). (d) A
detailed picture at higher magnification (40x), showing the marker cell
atypia.. H&E, hematoxylin-eosin; PEComa, perivascular epithelioid cell tumor;
SMA, smooth muscle actin.
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At histology, the lesion constituted epithelioid cells and spindle cells with marked atypia, very low mitotic index, scattered mature adipocytes, and a rich vascularization (Figure 2(a)-(d)). At IHC, tumor cells were diffusely immunoreactive for HMB45, with occasional spindle cells positive for alpha-SMA and low proliferative index or Ki-67 (Figure 2(b) and (c)).
PMC6503595_03
Case 3: (a) magnetic resonance imaging of the multiple liver lesions; (b)
histological appearance of a focus of mature ectopic splenic tissue
(H&E stain, 2x); and (c) imaging and (d) histological picture of the
associated FNH (H&E stain, 2x).. FNH, focal nodular hyperplasia; H&E, hematoxylin-eosin.
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MRI confirmed the presence of multiple liver lesions (the biggest of 2.6 cm), characterized by enhancement in arterial phase and wash out in portal or delayed phase, without evident signal reduction in sequence T1, compatible with nonsteatotic or noninflammatory adenomas (with recommendation of histological confirmation) (Figure 3(a)). Only one lesion (of 4.4 cm) showed a central scar and enhancement in arterial and portal or delayed phase, with inhomogeneous hyperintensity in hepatobiliary phase, compatible with FNH (Figure 3(c)). At histological evaluation, the red-purple nodules constituted mature ectopic splenic tissue, with a normal red pulp largely composed of cordal macrophages and a poorly formed lymphocytic white pulp (Figure 3(b)). The single grayish lesion showed morphological features and GS immunoreactivity diagnostic of FNH (Figure 3(d)).