Presentation
Progressive neck swelling and gradual onset shortness of breath.
Patient Data
CASE OF THE MONTH: This case was selected as the Case of the Month for July 2024.
Mixed density well-defined multiloculated non-enhancing lesion predominantly involving the visceral space of the mediastinum, with extension to other spaces of mediastinum and neck. Lesion contains calcific foci, but no fat. In the mediastinum, it encases the great arteries and the veins without significant stenosis, infiltration or thrombosis. The trachea and bronchi are displaced posteriorly, with mild narrowing. The esophagus is displaced posteriorly and to the right. Adjacent lung segments are compressed. No pleural or pericardial effusion.
Multiloculated cystic parenchymal lesions in the liver and spleen. Several of the splenic lesions show punctate calcifications.
Multiple lytic bone lesions noted, most having geographic margins, involving both anterior and posterior compartments of the spine and pelvis. No vertebral collapse.
Case Discussion
This patient underwent debulking surgery to relieve symptoms. Histology confirmed lymphatic malformation. No thrombocytopenia or coagulopathy to indicate Kaposiform lymphangiomatosis.
Follow up imaging showed more cystic transformation of the solid areas, more calcifications, and reactive lymphadenopathy.
Generalized lymphatic anomaly, previously known as lymphangiomatosis or cystic angiomatosis, is a systemic condition characterized by multifocal lymphatic malformations with systemic distribution. The distribution of lesions is variable; there can be cutaneous, thoracic and abdominal visceral, and osseous involvement.
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