Challenging diagnosis of Kaposiform lymphangiomatosis: Unveiling a rare primary lymphatic disorder

Abstract

Kaposiform lymphangiomatosis (KLA) is a rare and complex lymphatic anomaly associated with substantial morbidity and mortality. It features diffuse and multifocal malformed lymphatic channels, often leading to diagnostic difficulties due to its uncommon occurrence and non-specific clinical presentation. This case report emphasizes the crucial role of expert radiologists in accurately diagnosing a challenging KLA case initially mistaken for a neoplasm.

Keywords

• Lymphatic anomalies
• Computer tomography
• Magnetic resonance
• MTOR-inhibitors
• Kaposiform lymphangiomatosis