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Competing Interests: Conflicts of interest None disclosed.

Objective: To describe the clinical and imaging characteristics of a new lymphatic disorder with a unique histological pattern and poor prognosis., Study Design: An observational, retrospective study identified and characterized 20 patients with distinct lymphatic histopathology referred to the Vascular Anomalies Center at Boston Children's Hospital between 1995 and 2011., Results: The median age at onset was 6.5 years (range, birth to 44 years). Clinical and radiologic findings suggested a generalized process. The most common presentations were respiratory symptoms (50%), hemostatic abnormalities (50%), and an enlarging, palpable mass (35%). All patients had mediastinal involvement; 19 patients developed pericardial (70%) and/or pleural effusions (85%). Extrathoracic disease manifested in bone and spleen and less frequently in abdominal viscera, peritoneum, integument, and extremities. Despite aggressive procedural and medical therapies, the 5-year survival was 51% and the overall survival was 34%. Mean interval between diagnosis and death was 2.75 years (range, 1-6.5 years)., Conclusions: We describe a clinicopathologically distinct lymphatic anomaly. We propose the term kaposiform lymphangiomatosis (KLA) because of characteristic clusters or sheets of spindled lymphatic endothelial cells accompanying malformed lymphatic channels. The intrathoracic component is most commonly implicated in morbidity and mortality; however, extrathoracic disease is frequent, indicating that KLA is not restricted to pulmonary lymphatics. The mortality rate of KLA is high despite aggressive multimodal therapy., (Copyright © 2014 Mosby, Inc. All rights reserved.)

Vascular anomalies are congenital lesions that most often first present in pediatric patients. Treatment of these lesions is a multidisciplinary team approach, involving several specialties, including diagnostic and interventional radiology, dermatology, general and plastic surgery, otolaryngology, and hematology. Knowing the characteristic findings of vascular anomalies on ultrasound, computed tomography, and magnetic resonance imaging leads to early, accurate diagnosis and therapeutic intervention of these lesions. This article discusses the gross and radiographic appearances and the latest treatment options for vascular anomalies., (Copyright © 2011 Mosby, Inc. All rights reserved.)

A case of epithelioid hemangioendothelioma presenting as violaceous nodules on the skin of the thigh is described. On histologic examination the tumor exhibited a proliferation of vascular channels lined by plump cuboidal and atypical endothelial cells with an epithelioid appearance. Lesions of epithelioid hemangioendothelioma were subsequently discovered in the adjacent femur. These tumors were successfully treated with chemotherapy and irradiation followed by a limb salvage procedure. To our knowledge, cutaneous presentation of this rare tumor has not been reported previously.