Kim Kyoung-A, Jang Kyu-Yun, Min Chang-Ki
Department of Oral and Maxillofacial Radiology, School of Dentistry, Jeonbuk National University, Backjedaero 567, Jeonju, Republic of Korea.
Department of Pathology, Jeonbuk National University Medical School, Backjedaero 567, Jeonju, Republic of Korea.
Oral Radiol. 2025 Apr 25. doi: 10.1007/s11282-025-00822-5.
Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor in neonates and children. KHE can occur anywhere in the body, but occurrence in the oral cavity is exceptionally rare. The clinical features of KHE are similar to those of oral lesions, such as infantine hemangioma, pyogenic granuloma, and chronic periodontitis. However, unlike other oral lesions, KHE invades neighboring tissue planes and recurs frequently as malignancy. Moreover, some instances of KHE are accompanied by a life-threatening condition named Kasabach-Merritt phenomenon. Correct diagnosis of KHE is very difficult despite its importance. KHE lacks not only specific clinical characteristics, but also distinct imaging findings even with advanced modalities such as computed tomography (CT) and magnetic resonance imaging (MRI). Thus, KHE is often misdiagnosed as another disease. We report a case of KHE in the mandibular gingiva. The enlarged gingival mass showed reddish granulomatous appearance with intact mucosal surface. It was misdiagnosed as another disease and underwent simple resection, but it recurred several times. In radiological examination using periapical radiography, cone beam computed tomography, and magnetic resonance imaging, no specific findings other than ill-defined alveolar bone destruction were observed. The subsequent pathological examination showed CD-31 and CD-34 positive, and D2-40 and HHV-8 negative. This report includes discussion about radiological, clinical, and histopathological features of the lesion and comprehensive literature review.
卡波西型血管内皮瘤（KHE）是一种发生于新生儿和儿童的具有局部侵袭性的血管肿瘤。KHE可发生于身体的任何部位，但发生于口腔的情况极为罕见。KHE的临床特征与口腔病变相似，如婴儿血管瘤、化脓性肉芽肿和慢性牙周炎。然而，与其他口腔病变不同的是，KHE会侵犯邻近组织平面，并常作为恶性肿瘤复发。此外，一些KHE病例还伴有一种名为卡萨巴赫-梅里特现象的危及生命的情况。尽管KHE的诊断很重要，但正确诊断却非常困难。KHE不仅缺乏特异性临床特征，即使采用计算机断层扫描（CT）和磁共振成像（MRI）等先进检查手段，也没有明显的影像学表现。因此，KHE常被误诊为其他疾病。我们报告一例下颌牙龈的KHE病例。肿大的牙龈肿物呈红色肉芽肿外观，黏膜表面完整。它被误诊为其他疾病并接受了简单切除，但多次复发。在使用根尖片、锥形束计算机断层扫描和磁共振成像的放射学检查中，除了边界不清的牙槽骨破坏外，未观察到其他特异性表现。随后的病理检查显示CD-31和CD-34阳性，D2-40和HHV-8阴性。本报告包括对该病变的放射学、临床和组织病理学特征的讨论以及全面的文献综述。
