Angiomyomatous hamartoma with extensive lymphadenopathy

A 75-year-old man with a history of aortic aneurysm was evaluated with computed tomography (CT) angiography prior to the surgical repair of aortic root dilatation. Imaging incidentally showed prominent mediastinal, retroperitoneal, pelvic, and inguinal lymph nodes, with the largest in the right paratracheal (1.6 cm) and inguinal (2.1 cm) regions. Review of the largest inguinal lymph node revealed parenchymal replacement by a proliferation of haphazardly arranged smooth muscle cells within fibrocollagenous stroma in the hilum, with extension to the cortex (panel A; original magnification ×20, hematoxylin and eosin [H&E] stain). Smooth muscle cells were admixed with blood vessels and adipocytes (panels B-C; original magnification ×100 [B] and ×200 [C]; H&E stain). Benign spindle cells and vascular proliferation were highlighted by smooth muscle actin and CD31 stains, respectively (panels D and E; original magnification ×20), support a diagnosis of angiomyomatous hamartoma (AMH). There was no significant change in the lymphadenopathy by chest CT after 2 months. He had no B symptoms after 6 months.

We present AMH with extensive lymphadenopathy. AMH is rare and predominantly reported in males within a wide age range. Patients typically present with asymptomatic, localized lymphadenopathy within the inguinal nodes. We present a case suggesting extensive pathology of this benign process in a patient lacking B symptoms or other evidence of lymphoma; therefore, AMH can be considered in the extensive lymphadenopathy differential diagnosis.