Kariv R, Sidi Y, Gur H
Department of Medicine C, Chaim Sheba Medical Center, Tel Hashomer, Israel.
Medicine (Baltimore). 2000 Nov;79(6):349-59. doi: 10.1097/00005792-200011000-00001.
Systemic vasculitis might present as a tumorlike lesion that initially could misdirect the correct diagnosis and the appropriate medical treatment. The aim of the present study is to summarize all reported cases of tumorlike presentation in systemic vasculitides, in order to have comprehensive data on the characteristics of this unusual phenomenon. We report 4 cases of systemic vasculitis presenting as a tumorlike lesion. In addition, we performed a MEDLINE search of all English-language papers published from 1966 to 1999, looking for vasculitis presenting as tumorlike lesion. Details were included concerning vasculitis classification, specific characteristics, location of the "tumor," the presence or absence of systemic involvement, and whether surgery was performed before diagnosis. Seventy-nine cases of vasculitis presenting as a tumorlike lesion were found in the literature, in addition to the 4 new cases described. The average age of the reported cases was 50.5 +/- 15.8 years, and 51% were female. In 82% of the cases the "tumor" was associated with constitutional symptoms and elevated erythrocyte sedimentation rate (ESR). The most common vasculitis categories with tumorlike presentation were Wegener granulomatosis (WG; 28 cases) and giant cell arteritis (GCA; 17 cases). In almost half the patients, surgery was performed before diagnosis. All patients with GCA presented with either a breast or an ovarian tumor. The most common location of a tumorlike lesion was the breast (22%), followed by central nervous system lesions (16%). Other frequent locations were the ovary (10%), caused exclusively by GCA and polyarteritis nodosa (PAN), and the male genitourinary system, almost all caused by PAN. Including vasculitis in the differential diagnosis of a tumorlike lesion might lead to an earlier diagnosis and consequently to prompt and appropriate treatment, avoiding needless operations. Constitutional symptoms and elevated ESR should alert clinicians to the possible diagnosis of vasculitis rather than a tumor. The association of GCA with ovarian pseudotumor is distinct and has not been emphasized before. We therefore suggest that GCA should be included in the list of differential diagnosis of an ovarian or breast tumor in an elderly woman, particularly when systemic symptoms and parameters of inflammation are present.
系统性血管炎可能表现为肿瘤样病变，最初可能会误导正确的诊断和恰当的治疗。本研究的目的是总结系统性血管炎中所有报告的肿瘤样表现病例，以便获得关于这一不寻常现象特征的全面数据。我们报告4例表现为肿瘤样病变的系统性血管炎病例。此外，我们对1966年至1999年发表的所有英文论文进行了MEDLINE检索，寻找表现为肿瘤样病变的血管炎。纳入了有关血管炎分类、具体特征、“肿瘤”位置、是否存在全身受累以及诊断前是否进行手术的详细信息。除了所描述的4例新病例外，文献中还发现79例表现为肿瘤样病变的血管炎病例。报告病例的平均年龄为50.5±15.8岁，51%为女性。82%的病例中，“肿瘤”伴有全身症状和红细胞沉降率（ESR）升高。表现为肿瘤样病变的最常见血管炎类型是韦格纳肉芽肿病（WG；28例）和巨细胞动脉炎（GCA；17例）。几乎一半的患者在诊断前接受了手术。所有GCA患者均表现为乳腺或卵巢肿瘤。肿瘤样病变最常见的位置是乳腺（22%），其次是中枢神经系统病变（16%）。其他常见位置是卵巢（10%），仅由GCA和结节性多动脉炎（PAN）引起，以及男性泌尿生殖系统，几乎全部由PAN引起。在肿瘤样病变的鉴别诊断中考虑血管炎可能会导致更早的诊断，从而实现及时和恰当的治疗，避免不必要的手术。全身症状和ESR升高应提醒临床医生可能诊断为血管炎而非肿瘤。GCA与卵巢假瘤的关联是独特的，之前未被强调。因此，我们建议老年女性卵巢或乳腺肿瘤的鉴别诊断清单中应包括GCA，特别是当存在全身症状和炎症指标时。
