PMC7394668_01
Sequential radiographs showing soft tissue and osseous changes in tibia (white arrows) and fibula (black arrows) over span of two years. Initial X-ray of 2016 a shows ill-defined sclerosis in proximal tibia and cortical erosion along the medial cortex of fibula which further progresses to circumferential cortical erosion in follow up radiograph of 2017 (b). The X-ray following three surgical debridements c demonstrates mixed lytic sclerotic changes involving proximal tibia and fibula. Gradually progressive soft tissue swelling is seen with predominant fat proliferation involving calf of right leg
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Fig1
After a repeat imaging, 2nd debridement was done in June, 2016 (Fig. 1a) and antibiotics were continued. Results were again disappointing and he was started on empirical ATT from April, 2017 (Fig. 1b). A radiograph of right leg showed lytic sclerotic destructive lesion with midzone of transition seen involving right proximal tibia and fibula with soft tissue component (Fig. 1c).
Sequential MRI of patient with Kaposiform haemangioendothelioma. Initial axial MR images a of 2016 show cortical destruction, marrow oedema involving tibia (T) and fibula (F) with surrounding soft tissue oedema on T1 (left) as well as STIR (right) sequences. Subsequently performed MRI in 2017 reveals increase in calf diameter and marrow oedema in both bones with visualization of plaque like hyperintensity along the muscle plane on STIR images (arrows in b). The recent MR images demonstrate trans-compartment involvement of skin (arrow in c) as thickening and hyperintense signal intensity; subcutaneous plane as fat proliferation which shows reticular pattern/lymphedema; muscles as atrophy and fat proliferation within the intermuscular plane and bones with marrow signal alteration. The similar changes are also evident on T1 weighted sagittal images of 2016 (arrow in d) and 2018 (asterisk in e)
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Fig2
MRI done in 2016 and 2017 (Fig. 2) revealed destruction of the right proximal tibia and fibula with marrow oedema, oedematous changes in surrounding soft tissue, atrophy of the calf muscles and a sinus tract due to prior surgical interventions.
Histopathology of Kaposiform haemangioendothelioma. a Low power photomicrograph of the tumor showing nodular architecture with spindle cells arranged in cannon ball fashion with slit like ill formed vascular channels. (H&E 100x). b High power photomicrograph showing cells exhibiting mild pleomorphism with elongated nuclei, finely dispersed chromatin and scanty to moderate cytoplasm. Few of the vascular spaces showed RBC. There were occasional mitotic figures. (H&E 200x). c Immunostaining for CD31 showing diffuse cytoplasmic positivity in tumor cells. d Immunostaining for FLI-1 showing nuclear positivity in tumor cells
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Fig3
Immunohistochemistry was positive for CD34, FLI-1 and CD31 (Fig. 3).