Title:Differential Diagnosis of Generalized Cystic Lymphangiomatosis: A Literature
Review
Volume: 20
Author(s): Ayşe Keven*Ahmet Faruk Gürbüz
Affiliation:
- Department of Radiology, Akdeniz University School of Medicine, Antalya, Turkey
Keywords:
Lymphatic anomalies, Generalized Cystic Lymphangiomatosis, Kaposiform lymphangiomatosis, Gorham-stout disease, Whole-body magnetic resonance imaging, Lymphatic malformations.
Abstract:
Background:
Generalized cystic lymphangiomatosis (GCL) is a rare disease characterized by the widespread proliferation of lymphatic vessels, often seen in the
pediatric patient group. Imaging techniques are instrumental in revealing the extent and morphological features of the disease.
Objective:
The objective of this study is to interpret the radiological findings of GCL and address the differential diagnosis between GCL and other lymphatic
malformations in light of the relevant literature data.
Methods:
The sample of this retrospective study consisted of six pediatric patients, four males and two females, diagnosed with GCL based on clinical,
radiological, and histopathological findings between 2015 and 2022. The age of the patients at the time of diagnosis and their symptoms at
admission were obtained from the hospital database. Radiological imaging findings were evaluated in detail based on the involved systems (thorax,
abdomen, and musculoskeletal).
Results:
The median age of the sample, 4/6 were male, was 9 years at admission (min. 3, max. 12). The most common symptom at admission was dyspnea,
often accompanied by pleural effusion. Bone involvement was the most common extrathoracic finding. Abdominal involvement was primarily
asymptomatic, and the spleen was the most frequently involved organ in the abdomen.
Conclusion:
The diagnosis of GCL is challenging because of its rarity and overlapping diseases. Whole-body magnetic resonance imaging is a valuable tool as
it reveals the typical radiological features of GCL and how far it has spread throughout the body.