Guillou L, Fletcher C D
University Institute of Pathology, Lausanne, Switzerland.
Am J Surg Pathol. 2000 Aug;24(8):1047-57. doi: 10.1097/00000478-200008000-00002.
The clinicopathologic features of 12 cases of benign lymphangioendothelioma (acquired progressive lymphangioma) are reported. There were five male and seven female patients. Age at diagnosis ranged from 17 to 90 years (median age, 54 yrs). Development of a single macular/papular hemangiomatous or pigmented lesion was the main presenting symptom. Symptom duration before diagnosis ranged from 2 months to 20 years (median, 5.5 yrs). Tumor size ranged from 0.3 cm to 10 cm (median. 1.5 cm). Location included skin of the head and neck (n = 5), back (n = 1), breast (n = 1), shoulder (n = 1), forearm (n = 1), plantar aspect of the foot (n = 2), and oral mucosa (n = 1). No patient had any other concomitant vascular anomaly (for example, lymphangiomatosis) or was suspected to have acquired immunodeficiency syndrome. Treatment consisted of excisional biopsy in nine patients, incisional biopsy in two, and wide excision in one. Follow-up information on nine patients (range, 4-40 mos; median, 12 mos) showed two local recurrences in one patient. Microscopically, the lesions consisted of anastomosing, often widely dilated vascular structures developing in the superficial dermis. As the lesion grew within deeper dermis, the vascular spaces collapsed and dissected the dermal collagen in an angiosarcoma-like pattern. The lining endothelium was flat and monolayered, with little or no cytologic atypia and no evident mitoses. Some vascular structures contained stromal papillary projections resembling papillary endothelial hyperplasia, and intravascular red blood cells were present occasionally. Immunohistochemistry performed in eight specimens showed variable endothelial cell reactivity for CD31 (7 of 8), CD34 (7 of 7), and factor VIII-related antigen (4 of 6). A smooth muscle cell layer was observed focally around the vascular spaces in six lesions. Benign lymphangioendothelioma (acquired progressive lymphangioma) is an uncommon benign lesion that, in view of major differences in treatment and prognosis, should be distinguished from well-differentiated angiosarcoma and Kaposi's sarcoma, especially the patch stage and lymphangioma-like variants of the latter.
报告了12例良性淋巴管内皮瘤（获得性进行性淋巴管瘤）的临床病理特征。患者中男性5例，女性7例。诊断时年龄为17至90岁（中位年龄54岁）。主要表现症状为单个斑疹/丘疹性血管瘤样或色素沉着性病变。诊断前症状持续时间为2个月至20年（中位时间5.5年）。肿瘤大小为0.3厘米至10厘米（中位大小1.5厘米）。病变部位包括头颈部皮肤（5例）、背部（1例）、乳房（1例）、肩部（1例）、前臂（1例）、足底（2例）和口腔黏膜（1例）。无患者伴有其他血管异常（如淋巴管瘤病），也无患者疑似患有获得性免疫缺陷综合征。9例患者接受了切除活检，2例接受了切开活检，1例接受了广泛切除。9例患者的随访信息（随访时间4至40个月，中位时间12个月）显示1例患者出现2次局部复发。镜下，病变由在浅表真皮内形成的相互吻合且常广泛扩张的血管结构组成。随着病变在更深层真皮内生长，血管腔塌陷并以血管肉瘤样模式分割真皮胶原。内衬内皮细胞扁平且为单层，几乎没有或没有细胞异型性，也无明显核分裂象。一些血管结构含有类似于乳头内皮增生的间质乳头样突起，血管内偶尔可见红细胞。对8个标本进行的免疫组织化学检测显示，内皮细胞对CD31（8例中的7例）、CD34（7例中的7例）和因子VIII相关抗原（6例中的4例）的反应性各不相同。6个病变中在血管腔周围局灶性观察到平滑肌细胞层。良性淋巴管内皮瘤（获得性进行性淋巴管瘤）是一种罕见的良性病变，鉴于其在治疗和预后方面的重大差异，应与高分化血管肉瘤和卡波西肉瘤相鉴别，尤其是后者的斑块期和淋巴管瘤样变体。
