Department of Pathology, Robert J. Tomsich Pathology & Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH.
Am J Surg Pathol. 2020 Nov;44(11):1515-1521. doi: 10.1097/PAS.0000000000001509.
Intravascular lobular capillary hemangioma (ILCH), or intravascular pyogenic granuloma, is relatively rare and likely underrecognized. We reviewed all ILCH cases from our institution confirmed pathologically from 2006 to 2019. Immunostains for smooth muscle actin and Wilms tumor 1 were performed on all cases and prior immunohistochemical stains were reviewed. Forty cases were identified (22 females; 18 males) with a median age of 53 years (range: 13 to 85 y). Clinically, all were well-circumscribed, stable to slow-growing raised/cystic skin lesions ranging from 1 to 40 mm. Most were located on the upper extremities (n=18), followed by head and neck (n=16). Vascular lesions were suspected clinically in one third. Of the consultation cases, provided differential diagnoses included Masson tumor, other hemangiomas, and vascular tumors of intermediate malignancy. The common histologic features were a well-circumscribed, lobular proliferation of closely packed capillaries with central ectatic vessels. The surrounding vascular wall was well-visualized in only half of the cases. About 30% of the cases were mitotically active (mean: 8 to 9 mitotic figures/10 HPFs), and 34% showed mild to moderate cytologic atypia. Hobnail features were present in 40% of cases. All cases were diffusely and strongly positive for Wilms tumor 1. Smooth muscle actin stains highlighted pericytes in all cases. Of the 20 cases with clinical follow-up (median: 40 mo), none recurred. ILCH commonly involves the upper extremities and a vascular tumor is suspected clinically in the minority. Mitotic activity and cytologic atypia, when present, can cause confusion with more aggressive vascular tumors. Recognition of this entity is essential as it is a benign lesion with no risk of recurrence following limited local excision.
血管内小叶状毛细血管血管瘤(ILCH),又称血管化脓性肉芽肿,较为罕见,可能认识不足。我们回顾了 2006 年至 2019 年我院经病理证实的所有 ILCH 病例。对所有病例均行平滑肌肌动蛋白和 Wilms 肿瘤 1 的免疫组化染色,并复习了之前的免疫组化染色。共确定 40 例(22 例女性;18 例男性),中位年龄为 53 岁(范围:13 至 85 岁)。临床特征为边界清楚、生长缓慢或稳定的高出皮肤/囊性皮损,大小为 1 至 40mm。大多数皮损位于上肢(n=18),其次是头颈部(n=16)。三分之一的病例临床怀疑为血管病变。会诊病例的鉴别诊断包括 Masson 瘤、其他血管瘤和血管中间型恶性肿瘤。常见的组织学特征是边界清楚的小叶状毛细血管增生,伴有中央扩张的血管。只有一半的病例能清楚地看到周围的血管壁。约 30%的病例有丝分裂活性(平均值:8 至 9 个有丝分裂象/10 高倍视野),34%的病例有轻度至中度细胞异型性。钉突样特征见于 40%的病例。所有病例均弥漫且强阳性表达 Wilms 肿瘤 1。平滑肌肌动蛋白染色在所有病例中均突出显示周细胞。20 例有临床随访(中位随访时间:40 个月)的病例中均无复发。ILCH 常累及上肢,少数病例临床怀疑为血管肿瘤。有丝分裂活性和细胞异型性,如存在,可与侵袭性更强的血管肿瘤混淆。认识到这一实体非常重要,因为它是一种良性病变,局限性切除后无复发风险。