Elsevier

The Journal of Pediatrics

Volume 164, Issue 2, February 2014, Pages 383-388
The Journal of Pediatrics

Original Article
Kaposiform Lymphangiomatosis: A Distinct Aggressive Lymphatic Anomaly

https://doi.org/10.1016/j.jpeds.2013.10.013Get rights and content

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.

Section snippets

Methods

Patients referred to our Vascular Anomalies Center from 1995-2011 with various types of lymphatic anomalies were reviewed at an interdisciplinary conference. Twenty patients had a GLA with novel histopathology. The histologic hallmark was clusters or sheets of “kaposiform” hemosiderotic, spindled lymphatic endothelial cells oriented in parallel fashion amid abnormal and dilated lymphatic channels. These spindled cells were immunoreactive for lymphatic markers (D2-40, Lyve-1, and Prox-1).

Results

All patients had a distinctive histopathologic pattern,10 characteristic imaging, and poor prognosis (Figure 1). They were often referred with a diagnosis of “lymphangiomatosis.” Thoracic disease typically manifested as pleural and/or pericardial effusions. High-resolution computed tomography demonstrated interlobular septal thickening due to dilated lymphatic channels usually with a prominent soft tissue component in the mediastinal, paraspinal, or retroperitoneal region. On magnetic resonance

Discussion

We describe clinical features of a distinct GLA that we term KLA given the foci of “kaposiform” spindled lymphatic endothelial cells and the progressive nature of the anomaly. Intrathoracic disease with worsening respiratory symptoms and hemorrhagic effusions are hallmarks of KLA. Current therapies are largely temporizing.

Spindled cells within anomalous pulmonary lymphatic channels have been noted previously,4, 6, 9 but such lesions have not been designated as a specific entity. It is known

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Supported by American Society of Hematology Trainee Research Grant, the National Institutes of Health's Heart, Lung, and Blood Institute (NIH NHLBI) (T32 HL007574 [to S.C.] and K08 HL089509 [to C.T.]), and Lymphatic Malformation Institute (to C.T.). The authors declare no conflicts of interest.

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