Angiomyomatous hamartoma of lymph nodes: Clinicopathological study of 6 cases with review of literature.

Angiomyomatous hamartoma (AMH) is a rare disease with predisposition for inguinal and femoral lymph nodes. Histologically, it is characterized by replacement of lymph nodal parenchyma with irregularly distributed thick walled blood vessels, haphazardly arranged smooth muscle cells, variable amount of fat and fibrous tissue in a sclerotic lymphatic stroma. Few cases have also been reported in popliteal and sub ‑ mandibular location. The exact pathogenesis is still not known. Although this entity is very rare, its recognition is important in discriminating it from other benign and malignant vascular lesions of lymph nodes.

Angiomyomatous hamartoma in an inguinal lymph node with proliferating pericytes/smooth muscle cells, plexiform vessel tangles, and ectopic calcification.

Angiomyomatous hamartoma (AMH) is a rare benign lesion, which occurs almost exclusively in the inguinal lymph nodes. We herein report a case of a female elder who presented with a long-standing inguinal mass. Microscopically, the mass showed a zonal distribution, characterized by thick-walled muscular vessels, fi brosis, and calcifi cation in the hilum and proliferating spindle cells around thin-walled vessels and plexiform vessel tangles at the periphery. The spindle cells show positive immunoreactivity of smooth muscle actin and CD34 with a heterogeneous expression of desmin and CD44. Although the pathogenesis of AMH remains uncertain, the histological features and immunohistochemical fi ndings of this case imply a disordered or exuberant angiogenic process.

Hamartoma nodal angiomiomatoso / Angiomyomatous hamartoma

O Hamartoma Nodal Angiomiomatoso (HNA) é uma doença benigna rara, descrita pela primeira vez em 1992 por Chan et al. Apresenta-se como um tumor vascular primário dos linfonodos que ocorre, na maioria das vezes, exclusivamente nos linfonodos inguinais e femoral, e está ocasionalmente associado a edema de membro inferior ipsilateral. Relata-se o caso de uma paciente de 74 anos, do sexo feminino, com HNA envolvendo linfonodos inguinais direitos; descrevendo seus aspectos patogênicos, clínicos, diagnósticos e terapêuticos. Enfatiza-se a importância do reconhecimento desta patologia para adequado diagnóstico e tratamento. Juntamente com o relato de caso, será realizada breve revisão da literatura (AU)

Angiomyomatous Hamartoma (AH) is a rare benign disease which presents itself as a primary vascular tumor of the lymph nodes, primarily found in the inguinal and femoral lymph nodes and occasionally associated with edema of the ipsilateral limb. Here we report the case of a female patient with AH involving the right inguinal lymph nodes. In addition to the case report, a brief literature review is presented describing the pathogenic, clinical, diagnostic and therapeutic aspects of the condition. The importance of proper recognition of the disease is emphasized for adequate diagnosis and treatment (AU)