Differential Diagnosis of Generalized Cystic Lymphangiomatosis: A Literature Review.

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.

Generalized cystic lymphangiomatosis incidentally recognized in an asymptomatic adult: Peroperative, CT, MRI, and histopathological findings of a very rare case.

Generalized cystic lymphangiomatosis is an uncommon congenital lymphatic malformation that may affect soft and hard tissues as well as organs. It is rarely seen in adult patients. Osseous lesions appear as well-defined cystic lesions with a sclerotic rim without periosteal reaction or a soft tissue component. A nonenhancing lesion in fluid density appears to be one of the most common characteristics of abdominal diseases, and clinical features are directly related to the extent of the disease. In this report, we present peroperative, computed tomography (CT), magnetic resonance imaging (MRI), and histopathological findings of a very rare case of generalized cystic lymphangiomatosis recognized in adulthood.