Chan J K, Frizzera G, Fletcher C D, Rosai J
Institute of Pathology, Queen Elizabeth Hospital, Hong Kong.
Am J Surg Pathol. 1992 Apr;16(4):335-50. doi: 10.1097/00000478-199204000-00003.
Primary vascular tumors of lymph nodes other than Kaposi's sarcoma are very rare, as attested to by only a handful of case reports in the literature. Based on an analysis of 39 such cases, we could distinguish five major groups. Hemangiomas of capillary/cavernous, lobular capillary, and cellular types were composed of compact aggregates of blood-filled vessels, variable in size, that replaced the nodal architecture partly or almost completely; some appeared to have originated in the hilum or medulla. These hemangiomas either represented incidental findings in lymph nodes or were seen with solitary lymph node enlargement; the evolution was benign with no recurrence. A distinctive benign lesion occurring exclusively in inguinal lymph nodes, which we propose designating "angiomyomatous hamartoma," showed replacement of the nodal parenchyma by smooth muscle cells and fibrous tissue, in continuity with exuberant proliferation of muscular vessels in the hilum. Epithelioid vascular tumors, characterized by plump endothelial cells with dense eosinophilic cytoplasm and numerous vacuoles, exhibited a range of differentiation, from hemangioma with well-formed vascular channels (with or without tissue eosinophilia) to hemangioendotheliomas composed predominantly of cords and sheets of tumor cells lying in a hyaline-myxoid matrix. Epithelioid hemangioendothelioma was particularly likely to be mistaken for metastatic carcinoma, and local recurrence could occur. A variant, the spindle and epithelioid hemangioendothelioma, was characterized by the presence of an additional component of spindle cells. Another tumor we found, polymorphous hemangioendothelioma, is a previously uncharacterized borderline malignant vascular tumor exhibiting solid, primitive vascular and angiomatous patterns and relatively bland cytologic features. Lymphangiomas of lymph nodes usually showed simultaneous multifocal and extra-nodal involvement and were characterized by cystic endothelium-lined spaces filled predominantly with lymph fluid. It is important to recognize these primary vascular tumors of lymph nodes to avoid mistaking them for a variety of benign vasoproliferative lesions, Kaposi's sarcoma, angiosarcoma, and metastatic cancer.
除卡波西肉瘤外，淋巴结原发性血管肿瘤非常罕见，文献中仅有少数病例报告证实这一点。通过对39例此类病例的分析，我们可区分出五大类。毛细血管/海绵状血管瘤、小叶状毛细血管瘤和细胞型血管瘤由紧密聚集的充血血管组成，大小不一，部分或几乎完全取代淋巴结结构；有些似乎起源于淋巴结门部或髓质。这些血管瘤要么是在淋巴结中偶然发现，要么是在孤立性淋巴结肿大时发现；其发展为良性，无复发。一种仅发生于腹股沟淋巴结的独特良性病变，我们建议将其命名为“血管肌性错构瘤”，表现为淋巴结实质被平滑肌细胞和纤维组织取代，并与淋巴结门部丰富增生的肌性血管相连续。上皮样血管肿瘤的特征是内皮细胞丰满，胞质嗜酸性致密且有许多空泡，表现出一系列分化，从具有良好形成血管腔隙的血管瘤（有或无组织嗜酸性）到主要由位于透明黏液样基质中的肿瘤细胞条索和片层组成的血管内皮瘤。上皮样血管内皮瘤特别容易被误诊为转移性癌，且可能发生局部复发。一种变异型，梭形和上皮样血管内皮瘤，其特征是存在额外的梭形细胞成分。我们发现的另一种肿瘤，多形性血管内皮瘤，是一种以前未被描述的交界性恶性血管肿瘤，表现为实性、原始血管和血管瘤样模式，细胞学特征相对温和。淋巴结淋巴管瘤通常表现为同时多灶性和淋巴结外受累，其特征是主要充满淋巴液的囊性内皮衬里腔隙。认识这些淋巴结原发性血管肿瘤很重要，以避免将它们误诊为各种良性血管增生性病变、卡波西肉瘤、血管肉瘤和转移性癌。
