PMC7708871_01
(a) Clinical picture of multiple recurrent nodular swellings at scar site in the left upper limb during eighth recurrence in 2017. (b) Operative photograph showing the radial nerve pointed with artery forceps separate from the tumour being excised. Tumour is held with Alice forceps. (c) Excised swelling during eighth recurrence.
gr1.jpg
PMC7708871_01_gr1.jpg
fig0005
The patient returned in November 2017 with bigger swellings in both the primary surgical sites (Fig. 1a). During excision, the radial nerve was seen not involved by the lesion (Fig. 1b). The multiple nodular tumour was excised free of the nerve (Fig. 1c).
Clinical picture of non healing wound with proliferating swellings at scar site after 13th excision. This appearance forced the patient to accept amputation.
gr2.jpg
PMC7708871_01_gr2.jpg
fig0010
The wound would not heal and in two months, the tumour recurred at the site of the unhealed area and extended proximally towards the shoulder (Fig. 2).
a & b-Amputated limb with ulcerated growth and Cross-section showing tumour eroding bone cortex with marrow infiltration grossly.
gr3.jpg
PMC7708871_01_gr3.jpg
fig0015
A thorough gross examination of the amputated limb (Fig. 3a) showed a fleshy whitish growth of size 17 x 7 x 6.5 cm, seen in the subcutaneous plane with deep extension and eroding bone cortex (Fig. 3b).
X-ray of amputated specimen showing tumour calcification and involvement of humerus.
gr4.jpg
PMC7708871_01_gr4.jpg
fig0020
X-ray showed irregularity of the humerus indicating tumour infiltration and loss of cortical density with extraneous calcifications near the elbow portions of the soft tissue tumour (Fig. 4).
Microscopy showing monomorphic spindle cells in fascicles infiltrating muscle bundles at periphery (H&E x400).
gr5.jpg
PMC7708871_01_gr5.jpg
fig0025
Periphery showed muscle bundles, cortical bone and marrow spaces being infiltrated by the neoplasm (Figs. 5, 6).
Tumour infiltrating bone cortex and involving marrow spaces (H&E x400).
gr6.jpg
PMC7708871_01_gr6.jpg
fig0030
Periphery showed muscle bundles, cortical bone and marrow spaces being infiltrated by the neoplasm (Figs. 5, 6).
Metaplastic bone formation within the tumour (H&E x400).
gr7.jpg
PMC7708871_01_gr7.jpg
fig0035
Extensive areas of metaplastic bone formation were noted (Fig. 7).
Cytokeratin (CK) immunostaining: diffuse strong positivity with intense perinuclear zone reactivity (CK IHC x400).
gr8.jpg
PMC7708871_01_gr8.jpg
fig0040
IHC sarcoma panel showed patchy positivity for S100, diffuse strong positivity for CK (Fig. 8) and BCL2.IHCERG, SOX10, EMA, CD34, CD99, DESMIN, SMA, TLE1 and STAT-6 were negative.