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Synchronous Adenocarcinoma, Carcinoid Tumor, and Pulmonary Langerhans Cell Histiocytosis in the Same Lobe

Imaging findings

A patient with cough had bilateral upper-lobe-predominant nodules with bizarre-shaped cysts typical of pulmonary Langerhans cell histiocytosis (sparing the right middle lobe, lingula, and lower lobes), but a more discrete, morphologically distinct nodule stood out on radiograph, along with a separate mixed solid and ground-glass nodule. Bronchoscopic sampling of the discrete nodule yielded neuroendocrine cells, and subsequent left lower lobectomy revealed that the mixed solid/ground-glass nodule was an adenocarcinoma, the discrete nodule was a carcinoid tumor, and the remaining background nodularity and cysts represented Langerhans cell histiocytosis.

Key takeaways

A discrete nodule that looks morphologically different from a background of cystic/nodular Langerhans cell histiocytosis warrants dedicated evaluation rather than being attributed to the underlying disease, since this case demonstrates that two separate primary neoplasms (adenocarcinoma and carcinoid) can coexist with pulmonary Langerhans cell histiocytosis in the same lobe; recognizing the classic upper-lobe-predominant, lower-lobe-sparing cystic and nodular pattern of Langerhans cell histiocytosis on the initial radiograph is key to correctly triaging the truly discrepant nodule for biopsy.

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