A case report of primary Kaposiform hemangioendothelioma of the humerus.

IF 3.5 3区 医学
Ye Wang, Zhenqi He, Hua Hao
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Abstract

To examine the clinicopathological and immunohistochemical features of Kaposiform hemangioendothelioma (KHE) and discuss its differential diagnosis and prognosis. A patient with KHE was examined; the patient's clinical and histopathological features were observed, and the expression levels of CD31, CD34, ERG, D2-40, SMA, GLUT-1, and LANA-1 were assessed. The patient was a four-year-old child with primary KHE of the humerus. She was admitted to the hospital because of pain in the right elbow joint and limited movement for more than 2 years. Imaging revealed Langerhans cell histiocytosis. The child was not diagnosed with Kasabach-Merritt phenomenon (KMP). The tumor consists of multiple hemangiomatous nodules with infiltrative growth separated by fibrous connective tissue. The proliferating hemangiomatoid nodules consisted of crisscrossing short spindle-shaped cell bundles and erythrocyte-containing lacunar or crescentic vessels. Immunohistochemical staining showed that the tumor cells diffusely expressed CD31, CD34, ERG, and other vascular endothelium-derived markers; further, the tumor cells expressed neither GLUT-1 nor LANA-1. The patient's general condition improved after surgical resection. There was no tumor recurrence after more than 8 months of follow-up. Primary KHE of the humerus is a rare vasculogenic tumor. It presents with morphological features that require an accurate differential diagnosis.

一例肱骨原发性卡波状血管内皮瘤病例报告。
研究卡波西瘤(Kaposiform hemangioendothelioma,KHE)的临床病理和免疫组化特征,并讨论其鉴别诊断和预后。研究人员对一名KHE患者进行了检查,观察了患者的临床和组织病理学特征,并评估了CD31、CD34、ERG、D2-40、SMA、GLUT-1和LANA-1的表达水平。患者是一名四岁儿童,患有原发性肱骨 KHE。她因右肘关节疼痛和活动受限两年多而入院。影像学检查显示她患有朗格汉斯细胞组织细胞增生症。患儿未被诊断出患有卡萨巴赫-梅里特现象(KMP)。肿瘤由多个浸润性生长的血管瘤结节组成,结节之间由纤维结缔组织分隔。增生的类血管瘤结节由纵横交错的短纺锤形细胞束和含红细胞的裂隙或新月形血管组成。免疫组化染色显示,肿瘤细胞弥漫表达 CD31、CD34、ERG 和其他血管内皮衍生标记物;此外,肿瘤细胞既不表达 GLUT-1 也不表达 LANA-1。手术切除后,患者的一般状况有所改善。经过 8 个多月的随访,肿瘤没有复发。肱骨原发性 KHE 是一种罕见的血管源性肿瘤。它的形态特征需要准确的鉴别诊断。
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来源期刊
International Journal of Immunopathology and Pharmacology
International Journal of Immunopathology and Pharmacology Immunology and Microbiology-Immunology
自引率
0.00%
发文量
88
期刊介绍: International Journal of Immunopathology and Pharmacology is an Open Access peer-reviewed journal publishing original papers describing research in the fields of immunology, pathology and pharmacology. The intention is that the journal should reflect both the experimental and clinical aspects of immunology as well as advances in the understanding of the pathology and pharmacology of the immune system.
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