Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences.

ABSTRACT:

Background

Kaposiform lymphangiomatosis (KLA) and kaposiform hemangioendothelioma (KHE) are rare and aggressive vascular disorders. The aim of this study was to examine the clinical features and prognosis of KLA and KHE involving the thorax.

Methods

The clinical features, imaging and pathological findings, treatments and outcome were retrospectively reviewed for 6 patients with KLA and 7 patients with KHE involving the thorax.

Results

The mean ages at the time of the presentation of signs/symptoms were 26.7 months and 4.1 months for KLA and KHE, respectively. Respiratory symptoms, pericardial and pleural effusion, thrombocytopenia and coagulopathy were common in both KLA and KHE. Diffuse lesions involving the lung and extrathoracic sites were observed in KLA but not i

SUBMITTER: Ji Y 

PROVIDER: S-EPMC6612206 | biostudies-literature | 2019 Jul

REPOSITORIES: biostudies-literature