Lin B T, Weiss L M, Battifora H
Division of Pathology, City of Hope National Medical Center, Duarte, California 91010-0269, USA.
Am J Surg Pathol. 1997 Oct;21(10):1138-43. doi: 10.1097/00000478-199710000-00003.
Although vascular invasion is common in many malignant tumors, disseminated intravascular anaplastic neoplasms with occult primary tumor are rare occurrences. Intravascular malignant lymphoma, also called angiotropic lymphoma, is a rare variant of large cell lymphoma predominantly involving vessels in multiple organs, and usually without significant nodal involvement. Although initially misinterpreted as an endothelial neoplasm-angioendotheliomatosis-immunohistochemical studies subsequently proved it to represent a peculiar form of malignant lymphoma. In this report, we describe two patients with extensive intravascular dissemination of angiosarcoma initially without clinically obvious primary tumor. These may be interpreted as examples of true angioendotheliomatosis. In each case the immunohistochemical studies ruled out the most common intravascular malignant neoplasms. The diagnosis of intravascular angiosarcoma was confirmed by the immunoreactivity of the tumor cells to several markers of endothelial lineage in both cases. Thus, angiosarcoma may present with intravascular dissemination and occult primary tumor and closely resemble metastatic carcinoma, melanoma, or angiotropic lymphoma. Immunohistochemical studies are crucial in ruling out these possibilities and in confirming the endothelial origin of the neoplastic cells.
尽管血管侵犯在许多恶性肿瘤中很常见，但伴有隐匿性原发肿瘤的播散性血管内间变性肿瘤却很少见。血管内恶性淋巴瘤，也称为嗜血管性淋巴瘤，是大细胞淋巴瘤的一种罕见变体，主要累及多个器官的血管，通常无明显的淋巴结受累。尽管最初被误诊为内皮肿瘤——血管内皮瘤病——但免疫组织化学研究随后证明它代表一种特殊形式的恶性淋巴瘤。在本报告中，我们描述了两名最初无临床明显原发肿瘤的血管肉瘤广泛血管内播散的患者。这些病例可被解释为真正的血管内皮瘤病的实例。在每个病例中，免疫组织化学研究排除了最常见的血管内恶性肿瘤。通过肿瘤细胞对两种情况下内皮谱系的几种标志物的免疫反应性，证实了血管内血管肉瘤的诊断。因此，血管肉瘤可能表现为血管内播散和隐匿性原发肿瘤，并且与转移性癌、黑色素瘤或嗜血管性淋巴瘤极为相似。免疫组织化学研究对于排除这些可能性以及确认肿瘤细胞的内皮起源至关重要。
