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Intracranial kaposiform hemangioendothelioma presenting as epistaxis: a rare case report with review of literature
Child's Nervous System ( IF 1.4 ) Pub Date : 2020-09-28 , DOI: 10.1007/s00381-020-04905-y
Soutrik Das 1 , Harsh Deora 2 , Shilpa Rao 1 , Sandeep Kandregula 2 , Suma Mysore Narayana 3
Affiliation  

Introduction

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of intermediate malignancy with tendency for local invasion and recurrence. The tumor almost exclusively occurs in children, especially in infants. Intracranial KHE are extremely rare with only two cases reported in the literature.

Report

We report the clinical and pathological features of this rare tumor arising from basitemporal region in a 21-month child. Our case did not present with Kasabach-Merritt phenomenon. Histopathological examination confirmed the diagnosis of KHE.

Conclusion

KHE should be considered in the differential diagnosis of intracranial extra-axial neoplasm in children, and histopathological examination plays an important role in distinguishing KHE from its morphologic mimics. It is essential to diagnose KHE due to its locally aggressive nature.



中文翻译:

颅内卡波西样血管内皮瘤表现为鼻衄:罕见病例报告并文献复习

介绍

卡波西样血管内皮瘤(KHE)是一种罕见的中等恶性血管肿瘤,具有局部侵袭和复发的趋势。肿瘤几乎只发生于儿童,尤其是婴儿。颅内 KHE 极为罕见,文献中仅报道了两例。

报告

我们报告了这种罕见肿瘤的临床和病理特征,该肿瘤起源于一名 21 个月大的儿童的基底颞区。我们的案例没有出现 Kasabach-Merritt 现象。组织病理学检查证实了 KHE 的诊断。

结论

在儿童颅内轴外肿瘤的鉴别诊断中应考虑 KHE,组织病理学检查在区分 KHE 与其形态学类似物方面起着重要作用。由于其局部侵袭性,诊断 KHE 至关重要。

更新日期:2020-09-29
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