According to the updated classification by the International Society for the Study of Vascular Anomalies, hemangioendothelioma has been subclassified into Kaposiform, spindle cell, epithelioid, composite, retiform, and polymorphous hemangioendotheliomas, lymphangioendotheliomatosis, and Dabska tumor.[5
] The histopathologic features of our reported lesion were consistent with epithelioid hemangioendothelioma without atypia.
Epithelioid hemangioendothelioma is a rare vascular tumor believed to be in the middle of the spectrum of epithelioid vascular tumors between benign epithelioid hemangioma and highly aggressive epithelioid angiosarcoma.[6
] It is seen mainly in middle-aged adults and usually occurs as a solitary lesion with variable clinical behavior.[1
] It can affect many organs such as the liver, bone, skin, lung, and soft tissues; however, eyelid involvement is exceedingly rare. To our knowledge, this is the fourth report of an epithelioid hemangioendothelioma arising on the eyelid reported in the English literature.[7
Because the eyelid epithelioid hemangioendothelioma is a low-grade malignant lesion that shows a high rate of local recurrence but rarely metastasizes,[1
] complete excision (not biopsy) should be performed to avoid recurrence. As our patient underwent complete excision of the eyelid hemangioendothelioma, she required no therapy other than local excision. Moreover, adjuvant radiotherapy or chemotherapy has not proven to be beneficial.[6
] In fact, because epithelioid hemangioendothelioma is rare and almost always discovered after surgical excision for a supposed benign cause, few patients have undergone complete preoperative investigation to detect the presence of possible metastasis. In addition, it has been reported that possible metastasis might not become evident for many years because of the slow growth of this tumor.[6
] Therefore, follow-up with computed tomography scan is recommended to study regional lymph nodes and lungs, which are the most involved sites. So, although our patient has shown no evidence of recurrence so far, careful follow-up will be necessary to detect possible late recurrence of her epithelioid hemangioendothelioma.
In summary, hemangioendothelioma can masquerade as a chalazion and any abnormal content observed during chalazion evacuation should encourage complete excision.