PMC9248042_01
Ischemic ulcer on the right calf.
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For the next 14 years, the ulcer did not heal and it even expanded (Figure 1).
Computed tomography angiogram shows patent aorto-bifemoral bypass with
occlusion of the right superficial femoral artery.
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In July 2016, angiography was performed for worsening pain in the right calf, proving occlusion of the right superficial femoral artery (Figure 2).
Histopathology of the tumor. (a) Structures of spindle and epithelioid-like
cells (original magnification x 100). (b) Spindle-shaped suggestive rhythmic
structures in the myxoid stroma (original magnification x 400). (c) Sections
imitating rhabdoid structures (original magnification x 400). (d) Sections
of pseudogranulomatous appearance (original magnification x 40). (e)
Expression of cytokeratins(CK AE 1-3 in tumor cells (original magnification
x 40). (f) Immunoreactivity with Early Growth Response Protein (ERG-1) in
tumor cells (original magnification x 200). (g) Negative staining on cluster
of differentiation (CD 31) in tumor cells. Positive control in small vessels
(original magnification x 100). (h) Negative staining on desmin in tumor
cells (original magnification x 200). (i) Retained expression of integrase
interactor 1 (INI1) in tumor cells (original magnification x 400).
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Malignant tumor structures of spindle and epithelioid-like cells were found at the base of the ulceration (Figure 3(a)), with focal necrosis and secondary vascular thrombosis. In some places, spindle-shaped suggestive rhythmic structures in the desmoplastic stroma predominated (Figure 3(b)). In some areas of the epithelioid appearance, there were sections imitating rhabdoid structures (Figure 3(c)). Geographical necrosis and some sections of pseudogranulomatous appearance accompanied by mixed inflammatory cellulitis were detected in the tumor structures (Figure 3(d)). With integrase interactor 1 (INI1), also known as sarcoma with retained INI1 (SMARCB1), there was no evidence of loss of expression (Figure 3(e)-(i)).
Imaging studies. (a) CT frontal image with 175 mm large black spot
corresponding with tumor region. (b) CT frontal image of the right calf. The
white arrow shows destruction of the fibula. (c) CT axial image of the
middle third of the right calf with tumor infiltrating the skin,
subcutaneous tissue, and destruction of the fibula. (d) CT axial image of
the middle third of the right calf with tumor infiltrating the skin with
18F-fluorodeoxyglucose (FDG) PET/CT uptake by the tumor. (e) MRI on
T2-weighted (T2W) axial image with enlarged lymph nodes in the right groin
(yellow arrow).
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Subsequent computed tomography revealed a large pathological formation of 65x55 mm in the middle third of the right leg, infiltrating the skin, subcutaneous tissue, destroying the fibula and numerous satellite deposits in its close proximity:all with significantly increased accumulation of 18F-fluorodeoxyglucose (FDG) (Figure 4(a)-(d)). After right leg amputation, pelvic MRI was performed, which showed enlarged lymph nodes in the right groin and along the iliac vessels (Figure 4(e)).