Kaposiform Hemangioendothelioma in a 3 Months Old Infant

Abstract

Kaposiform hemangioendothelioma is a rare, highly vascular and aggressive soft tissue tumor frequently associated with Kasabach-Merritt phenomenon, usually seen in early infancy. Early diagnosis by means of MRI and tissue biopsy portends a better outcome. Treatment includes surgical excision when feasible and medical management with steroids, propranolol, vincristine and supportive treatment for coagulopathy. We report a 3 months old female infant who was diagnosed, treated successfully and is now in complete remission.Keywords Kaposiform hemangioendothelioma Á Kasabach-Merritt phenomenon Á Vascular soft tissue tumor Á Vincristine Á Steroids Á Remission Three months old, developmentally normal female infant was evaluated for an incidentally noticed ill-defined, nontender, firm and immobile mass in the right paravertebral dorsolumbar region, with features of consumptive coagulopathy. Investigations revealed anaemia, thrombocytopenia and deranged PT/PTT. Ultrasound abdomen (Fig. 2) revealed an ill defined mixed echoeic mass lesion in the retroperitoneum, predominantly in the midline with extension to both paravertebral regions, with few calcific foci, encasing the vessels and infiltrating the pancreas. MRI abdomen (Fig. 1) confirmed the above findings, additionally revealing that the lesion was encasing the abdominal aorta, inferior vena cava and extending to the bilateral renal hila. The lesion was also extending superiorly from the level of the coeliac axis and inferiorly into the mesentery encasing the branches of the superior mesenteric vessels.The child was started on intravenous corticosteroids (methylprednisolone 2 mg/kg/day) and propranolol (0.2 mg/kg/day) to achieve haemostasis. Open biopsy after 7 days of steroids and after consumptive coagulopathy was controlled, confirmed the diagnosis of Kaposiform hemangioendothelioma (KHE). Oral steroids (prednisolone 1 mg/kg) and propranolol were continued for 12 weeks

Beta-adrenergic receptors are expressed across diverse cancers

“…The β-AR receptors are expressed in normal vessels [ 2 – 5 ] and have more recently been detected in various vascular tumors including hemangiomas, hemangioendotheliomas, and angiosarcomas [ 13 , 16 , 24 , 25 ]. Indeed, beta blockade has shown remarkable efficacy in numerous clinical settings against infantile hemangiomas [ 10 , 19 ], hemangioendothelioma [ 26 – 28 ], and angiosarcoma [ 12 – 15 ]. Due to the success of propranolol against angiosarcoma, this drug has recently received Orphan Drug Designation by the European Medicines Agency for use against soft tissue sarcomas.…”

Lymphatic Markers in Kaposiform Hemangioendothelioma: Implications for Tumor Invasiveness and β-blockers efficacy

“…Propranolol combined with vincristine and glucocorticoid therapy can achieve better e cacy [23][24][25] . At present, the e cacy of propranolol in the treatment of KHE is still controversial, and the factors affecting its effectiveness are still unclear.…”