上皮样血管内皮瘤(EHE)是一种罕见的低度恶性血管肿瘤。它主要发生在肝脏,肺,骨头,和身体的其他部位。腮腺上皮样血管内皮瘤的报道在国内和国际文献中都很少见。这里,我们提出了一个腮腺上皮样血管内皮瘤的病例报告,包括其完整的临床过程和影像学检查结果,改善这种不寻常疾病的诊断和治疗。
■病人,一位75岁的女性,出现右耳肿胀2个月和疼痛20天。增强的腮腺MRI显示明确的,具有均匀信号强度的圆形质量。肿块在T1加权成像上显示低信号强度,在T2加权成像上的高信号强度,结节内信号强度低,DWI序列上显著的高信号强度,ADC序列的信号强度低,静脉注射Gd-DTPA后动脉期的异质性增强。内部观察到结节非增强低信号强度,静脉期有轻微的间隙。手术前的初步诊断是良性病变,但经过组织病理学和免疫组织化学检查,证实为上皮样血管内皮瘤。
■进行完全肿瘤切除。
■患者恢复良好,进行了长达1年的细致随访,未发现复发或转移的迹象。持续的患者监测正在进行中,以证实和验证治疗的长期疗效。
■由于腮腺上皮样血管内皮瘤极为罕见,它经常导致高误诊率。最常见的误诊是涎腺淋巴瘤,其次是上皮样血管肉瘤.当病变是多灶性的,梭形,内部坏死,并在T2加权成像上显示点状低信号强度,动脉期显着增强,特别是更明显的外周增强,静脉和延迟期持续增强,应考虑上皮样血管内皮瘤。然而,目前上皮样血管内皮瘤的临床诊断仍主要依靠免疫组织化学方法。
Epithelioid hemangioendothelioma (EHE) is a rare low-grade malignant vascular tumor. It mainly occurs in the liver, lungs, bones, and other parts of the body. Reports of epithelioid hemangioendothelioma in the parotid gland are rare in both domestic and international literature. Here, we present a case report of a parotid gland epithelioid hemangioendothelioma, including its complete clinical course and imaging findings, to improve the diagnosis and treatment of this unusual disease.
UNASSIGNED: The patient, a 75-year-old female, presented with a swelling around the right ear for 2 months and pain for 20 days. Enhanced MRI of the parotid gland revealed a well-defined, round mass with homogeneous signal intensity. The mass showed low signal intensity on T1-weighted imaging, high signal intensity on T2-weighted imaging, nodular low signal intensity within, significant high signal intensity on DWI sequence, low signal intensity on ADC sequence, and heterogeneous enhancement in the arterial phase after intravenous injection of Gd-DTPA. Nodular non-enhancing low signal intensity was observed internally, and slight clearance was seen in the venous phase. The initial diagnosis before surgery was a benign lesion, but after histopathological and immunohistochemical examination, it was confirmed as epithelioid hemangioendothelioma.
UNASSIGNED: Complete tumor resection was performed.
UNASSIGNED: The patient experienced a favorable recovery, with meticulous follow-up conducted for up to 1 year revealing no signs of recurrence or metastasis. Continued patient surveillance is ongoing to substantiate and validate the long-term efficacy of the treatment.
UNASSIGNED: Due to the extreme rarity of parotid gland epithelioid hemangioendothelioma, it often leads to a high misdiagnosis rate. The most common misdiagnosis is salivary gland lymphoma, followed by epithelioid hemangiosarcoma. When the lesion is multifocal, fusiform, with internal necrosis, and shows punctate low signal intensity on T2-weighted imaging, significant enhancement in the arterial phase, particularly with more pronounced peripheral enhancement, and persistent enhancement in the venous and delayed phases, epithelioid hemangioendothelioma should be considered. However, the current clinical diagnosis of epithelioid hemangioendothelioma still primarily relies on immunohistochemical methods.