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Lymphangioleiomyomatosis (LAM) with Subtle Cysts and Retroperitoneal Lymphadenopathy

Imaging findings

CT of the abdomen/pelvis showed retroperitoneal lymphadenopathy adjacent to the aorta, initially concerning for lymphoma. Biopsy of these nodes revealed lymphangioleiomyomatosis (LAM). Subsequent chest CT, though showing sparse and small thin-walled cysts in the lungs, was considered consistent with LAM in the context of the pathological diagnosis.

Key takeaways

Lymphangioleiomyomatosis (LAM) can be diagnosed by biopsy of extrapulmonary manifestations, such as retroperitoneal lymphadenopathy, even when lung involvement (cysts) is subtle, sparse, and small on CT. The clinical picture and pathological confirmation from non-pulmonary sites can support a confident diagnosis of LAM despite minimal lung findings.

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