KAPOSIFORM HEMANGIOENDOTHELIOMA OF INFANCY AND CHILDHOOD - AN AGGRESSIVE NEOPLASM ASSOCIATED WITH KASABACH-MERRITT SYNDROME AND LYMPHANGIOMATOSIS
KAPOSIFORM HEMANGIOENDOTHELIOMA OF INFANCY AND CHILDHOOD - AN AGGRESSIVE NEOPLASM ASSOCIATED WITH KASABACH-MERRITT SYNDROME AND LYMPHANGIOMATOSIS
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DOI：
10.1097/00000478-199304000-00001
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发表时间：
1993-04-01
影响因子：
5.6
通讯作者：
WEISS, SW
中科院分区：
文献类型：
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作者：
ZUKERBERG, LR;NICKOLOFF, BJ;WEISS, SW
我们报告了9个独特的，但相对未知的，婴儿和儿童血管肿瘤的临床和病理特征，表现为软组织肿块，往往与局部侵袭性疾病，淋巴管瘤病和Kasabach-Merritt综合征。患者中，4名男性和5名女性，除2名（中位数，2年;范围，5个月至19年）外，均在生命的前10年。肿瘤累及上肢深部软组织4例，腹膜后2例，胸壁、头皮、颈部各1例。4例患者还患有Kasabach-Merritt综合征，3例患者患有淋巴管瘤病。淋巴管瘤病包括软组织的弥漫性浸润淋巴管瘤（3例）和另外2例骨病变。在这三个案例中，淋巴管瘤病早于血管肿瘤的诊断，其余的同时诊断。肿瘤的特征是浸润，互连片或不规则结节的细长内皮细胞内衬新月形或裂缝样血管，并不常见，圆形毛细血管型血管。在一些肿瘤中，发现了上皮样内皮细胞巢，其具有明显的含有细颗粒状含铁血黄素、透明小滴和胞质空泡的嗜多染性细胞质。在梭形内皮细胞内观察到少量含铁血黄素，在微小的管腔内偶见微血栓。这些肿瘤中的核分裂极轻微，核分裂像不常见，平均为2 - 3/10高倍视野（HPF）（范围0-7/10 HPF）。肿瘤周围有较大的、成形良好的供血血管。这些血管的内皮细胞表达因子VIII-AG，CD 34，并结合荆豆，偶尔包含表达肌肉特异性肌动蛋白的周上皮细胞。相反，梭形肿瘤细胞仅表达CD 34。人乳头状瘤病毒（HPV）-16-样DNA转录本，已确定在卡波西肉瘤的情况下，没有检测到聚合酶链反应在两个案件。随访信息显示，4例患者在广泛切除、多处切除或截肢（1例）后无疾病存活; 3例患者有疾病存活; 2例患者死亡，1例死于淋巴管瘤病伴呼吸道损害，另1例死于Kasabach-Merritt综合征并发出血。治疗应包括广泛的局部切除和相关症状的支持治疗。
We report the clinical and pathological features of nine distinctive, but relatively unknown, vascular tumors of infancy and childhood presenting as soft tissue masses often associated with locally aggressive disease, lymphangiomatosis and Kasabach-Merritt syndrome. The patients, four males and five females, were all in their first decade of life except for two (median, 2 years; range, 5 months to 19 years). These tumors involved deep soft tissues of the upper extremity (four cases), retroperitoneum (two cases), chest wall, scalp, and neck (one case each). Four patients also had Kasabach-Merritt syndrome, and three patients had lymphangiomatosis. Lymphangiomatosis consisted of diffusely infiltrating lymphangioma of soft tissue (three cases) and in two by the additional presence of bone lesions. In one of these three cases, lymphangiomatosis antedated the diagnosis of the vascular tumor, and in the remainder they were concurrently diagnosed. Tumors were characterized by infiltrating, interconnecting sheets or irregular nodules of slender endothelial cells lining crescentic or slit-like vessels and, less commonly, rounded capillary-type vessels. Within some tumors, nests of epithelioid endothelial cells with prominent cosmophilic cytoplasm containing finely granular hemosiderin, hyaline droplets, and cytoplasmic vacuoles were identified. Smaller amounts of hemosiderin were observed within the spindled endothelial cells and microthrombi could be seen occasionally within the tiny lumina. Nuclear atypia was minimal within these tumors and mitotic figures were infrequent, averaging 2 to 3/10 high-power fields (HPF) (range 0-7/10 HPF). Larger, well-formed feeding vessels were present at the periphery of the tumor. The endothelium of these vessels expressed factor VIII-AG, CD34, and bound Ulex europaeus, and contained an occasional perithelial cell expressing muscle-specific actin. In contrast, the spindled tumor cells expressed only CD34. Human papilloma virus (HPV)-16-like DNA transcripts, which have been identified in cases of Kaposi's sarcoma, were not detected by polymerase chain reaction in two cases. Follow-up information revealed that four patients were alive without disease after wide excision, multiple excision(s), or amputation (one case); three were alive with disease; and two died, one from lymphangiomatosis with respiratory compromise and the other from hemorrhage complicating Kasabach-Merritt syndrome. It appears that treatment should consist of wide local excision and supportive therapy for associated symptoms.