Sakurai Y, Shoji M, Matsubara T, Imazu H, Hasegawa S, Ochiai M, Funabiki T, Mizoguchi Y, Kuroda M, Kasahara M
Department of Surgery and Pathology, Fujita Health University School of Medicine, Toyoake, Aichi, Japan.
Pathol Int. 2000 Aug;50(8):655-9. doi: 10.1046/j.1440-1827.2000.01092.x.
Angiomyomatous hamartoma is a rare disease with a predisposition for the inguinal lymph nodes. A 51-year-old male patient visited a local hospital because of a right inguinal mass, measuring 3 x 4 cm in size, which was resected. The resected specimen showed irregularly distributed thick-walled vessels in the hilum, extending into the medulla and focally into the cortex of the node, eventually becoming more dispersed and associated with smooth muscle cells splaying into sclerotic stroma. These findings are compatible with an angiomyomatous hamartoma. Another tumor-like mass appeared shortly after the resection at the same location, but was not an angiomyomatous hamartoma, rather it was composed of edematous stromal tissue with proliferating smooth muscle cells. The stromal component included thick-walled blood vessels and lymphatics. Although it could not be determined whether these associated changes in the surrounding stroma are a cause or an effect of angiomyomatous hamartoma, they indicate the clinical difficulty in determining an appropriate area of resection and may provide clues to the pathogenesis of angiomyomatous hamartoma.
血管肌性错构瘤是一种罕见疾病,好发于腹股沟淋巴结。一名51岁男性患者因右侧腹股沟区一个大小为3×4厘米的肿物就诊于当地医院,该肿物被切除。切除标本显示,在淋巴结门部有分布不规则的厚壁血管,延伸至髓质并局部延伸至皮质,最终变得更加分散,并伴有平滑肌细胞呈扇形分布于硬化的间质中。这些表现符合血管肌性错构瘤。切除后不久,在同一部位又出现了另一个肿瘤样肿物,但并非血管肌性错构瘤,而是由伴有平滑肌细胞增生的水肿性间质组织构成。间质成分包括厚壁血管和淋巴管。虽然无法确定周围间质的这些相关改变是血管肌性错构瘤的病因还是结果,但它们提示了确定合适切除范围的临床困难,并可能为血管肌性错构瘤的发病机制提供线索。