Fig. 4
From: Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences
Radiologic findings, pathological features and treatment response in a patient with KHE (Patient #8). a An enhanced CT scan shows an enhanced soft-tissue mass extending from the posterior thoracic musculature to the vertebrae. Hematoxylin-eosin staining shows an abnormal proliferation of spindle cells and well-formed capillary-like vessels. Occasional slit-like or round lumens, which were comprised of closely packed spindle cells, could be seen (b). The spindle cells were positive for CD31 (c), CD34 (d), and D2–40 (e). The patient received sirolimus in combination with a short-term administration of prednisolone, followed by 22.0 months of sirolimus monotherapy. Prednisolone was successfully tapered and discontinued at 4 and 8 weeks, respectively. MRI showed obvious tumor shrinkage at 6 (f), 12 (g), and 24 (h) months after the start of sirolimus treatment