This patient was born with a large vascular lesion involving the left side of the face. He developed airway compromise as a result of the rapid growth of the lesion, but did not require a tracheostomy. He subsequently developed thrombocytopenia and anemia requiring numerous blood transfusions. He was born to 24 year old G1P0-1 via Cesarean section due to breech presentation.
MRA/MRI of the head and neck demonstrated an enhancing vascular tumor without gross intracranial extension that involved the subcutaneous tissues of the left neck and jaw, the oropharynx, the muscles of facial expression, the left maxillary sinus, the left pterygomaxillary fissure, the left maxilla, and the left supraorbital region. A large, warm, non-pulsatile, red-purple, tense, vascular tumor involving the left face and neck with significant distortion of facial anatomy. The patient was treated with systemic steroids, cyclophosphamide, vincristine, and interferon-2 alpha per the hematology/oncology service. Surgical debulking was considered too dangerous given the size and location of the tumor. He required multiple blood product transfusions. A trial of aminocaproic acid, an inhibitor of fibrinolysis, was also initiated. Some decrease in the size of the lesion was noted, and he was discharged at nine weeks of life. Kaposiform hemangioendothelioma (KHE)
is a rare vascular tumor of childhood usually presenting at birth or shortly thereafter. KHE seem to have a predilection for the trunk, extremities, and retroperitoneum. The tumor is locally aggressive, and often involves soft tissues and bone, but distant metastasis has not been reported. The Kasabach-Merritt Phenomenon (KMP), which is characterized by platelet trapping and resultant consumptive coagulopathy manifest as thrombocytopenia and disseminated intravascular coagulation, has been associated primarily with KHE, but may also be seen with tufted angiomas, congenital hemangiopericytomas, and certain lymphatic malformations.
The histology of KHE is complicated, involving features of Kaposi’s sarcoma (KS) and infantile hemangioma (IH). The characteristic appearance is that of irregular, infiltrative nodules composed of tightly coiled and highly convoluted blood vessels lined by spindled endothelial cells budding off of larger vessels. It has been hypothesized that since small convoluted capillaries arise directly from large vessels, high turbulence is created which leads to platelet activation and aggregation. Indeed, evidence of platelet-rich fibrin microthrombi and hemosiderin deposition are often seen. In contrast to IH, the markers GLUT-1 and Lewis Y antigen are not present in KHE.
KHE with KMP is associated with high mortality, in particular when associated with large tumors and those involving the viscera, mediastinum, retroperitoneum, and neck. Small KHE with a normal platelet count and without visceral involvement can be safely followed without treatment. Pharmacologic therapy, when indicated, includes systemic and/or intralesional corticosteroids, interferon-alpha, vincristine, aminocaproic acid, cyclophosphamide, pentoxifylline, and heparin. Surgical resection may be possible with small tumors, and arterial embolization and radiation theraoy have also been attempted.
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large, warm, non-pulsatile, red-purple, tense, vascular tumor involving the left face and neck with significant distortion of facial anatomy