Montgomery Elizabeth, Epstein Jonathan I
Department of Pathology and Oncology, The Johns Hopkins Hospital, Weinberg 2242, 401 North Broadway, Baltimore, MD 21231, USA.
Am J Surg Pathol. 2009 Sep;33(9):1364-9. doi: 10.1097/PAS.0b013e3181ad30a7.
We describe 6 cases of a poorly recognized vascular neoplasm that can simulate angiosarcoma.
Cases of a rare vascular tumor with a proclivity for the genitourinary tract encountered in our consultation material were prospectively collected between the year 1999 and 2008. Follow-up information was obtained when possible.
There were 6 tumors from 4 men (66%) and 2 women, ranging in age from 49 to 75 years (median, 59.5) involving the kidney and renal hilum (4, 66%) and testis (2). Tumors ranged from 1.3 to 1.7 cm (median, 1.6 cm) and were grossly well-marginated with a hemorrhagic mahogany spongy appearance. Microscopically, at low power they had a loosely lobulated architecture and were associated with a medium-caliber vessel (5/6, 83%). Most kidney (3/4, 75%) tumors showed minor extensions into adjacent adipose tissue. At higher magnification, the tumors consisted of anastomosing sinusoidal capillary-sized vessels with scattered hobnail endothelial cells within a framework of nonendothelial supporting cells. There was a minimal inflammatory backdrop consisting of lymphocytes but not plasma cells or acute inflammation. Mitoses were absent (5/6, 83%) or rare (1 case; in supporting cells). There was mild cytologic atypia in one of the cases but no multilayering of endothelial cells in any case. Vascular thrombi were typical (5/6, 83%) and the lesions had zones of central sclerosis with focal necrosis (5/6, 83%). Two (33%) tumors featured prominent extra-medullary hematopoiesis and 2 tumors (33%) had striking hyaline globules reminiscent of those seen in Kaposi's sarcoma. Immunohistochemistry was available on some cases and the lesions stained with CD34, CD31, and FVIII but not human herpes virus type 8, keratin AE1/3, epithelial membrane antigen, HMB45, placental alkaline phosphatase, or human chorionic gonadotropin. In all but one submitted consultation, the possibility of angiosarcoma had been raised based on the anastomosing vascular pattern. On follow-up, there were no recurrences or metastases in 5 cases (range: 8 to 36 mo; median 12 mo, mean 15 mo), and 1 patient was lost to follow-up.
Anastomosing hemangioma of the genitourinary tract is a rare neoplasm displaying some overlapping features of both sinusoidal hemangioma and hobnail hemangioma of soft tissue and skin. However, in our opinion, it is a unique neoplasm with a proclivity for the kidney. Its anastomosing appearance can lead to concern for angiosarcoma but, despite small numbers and limited follow-up in our series, evidence to date supports that the lesion is benign.
我们描述了6例一种鲜为人知的可模拟血管肉瘤的血管肿瘤。
前瞻性收集了1999年至2008年间我们会诊资料中遇到的一种好发于泌尿生殖道的罕见血管肿瘤病例。尽可能获取随访信息。
6例肿瘤患者中，男性4例（66%），女性2例，年龄49至75岁（中位数59.5岁），累及肾脏及肾门（4例，66%）和睾丸（2例）。肿瘤大小为1.3至1.7厘米（中位数1.6厘米），大体上边界清晰，呈出血性红褐色海绵状外观。显微镜下，低倍镜下可见其结构呈松散分叶状，并与中等口径血管相关（5/6，83%）。大多数肾脏肿瘤（3/4，75%）向邻近脂肪组织有轻微浸润。高倍镜下，肿瘤由相互吻合的窦状毛细血管大小的血管组成，在非内皮支持细胞框架内散在有鞋钉样内皮细胞。炎症背景轻微，由淋巴细胞组成，无浆细胞或急性炎症。有丝分裂不存在（5/6，83%）或罕见（1例；见于支持细胞）。1例有轻度细胞异型性，但所有病例均无内皮细胞多层排列。血管血栓形成很典型（5/6，83%），病变有中央硬化区伴局灶性坏死（5/6，83%）。2例（33%）肿瘤有明显的髓外造血，2例（33%）肿瘤有显著的透明小球，让人联想到卡波西肉瘤所见。部分病例可进行免疫组化检查，病变对CD34、CD31和FVIII呈阳性染色，但对人疱疹病毒8型、角蛋白AE1/3、上皮膜抗原、HMB45、胎盘碱性磷酸酶或人绒毛膜促性腺激素呈阴性染色。除1例提交会诊外，其余病例均因血管相互吻合的模式而怀疑为血管肉瘤。随访中，5例无复发或转移（范围：8至36个月；中位数12个月，平均15个月），1例失访。
泌尿生殖道吻合性血管瘤是一种罕见肿瘤，具有软组织和皮肤窦状血管瘤及鞋钉样血管瘤的一些重叠特征。然而，我们认为它是一种好发于肾脏的独特肿瘤。其相互吻合的外观可能导致对血管肉瘤的担忧，但尽管我们系列病例数量少且随访有限，迄今为止的证据支持该病变为良性。
