Retiform hemangioendothelioma

Retiform hemangioendothelioma is a rare type of vascular tumor that typically presents as a slow-growing cutaneous nodule or plaque. It was first described in 1994 by Calonje et al. and is characterized by its distinctive "net-like" or "retiform" vascular pattern. Despite its benign clinical course, it is histologically malignant and can be locally aggressive.

Clinical Presentation

Patients with retiform hemangioendothelioma typically present with a slow-growing cutaneous nodule or plaque. The lesions are often solitary and are most commonly found on the limbs, although they can occur anywhere on the body. The average age of onset is 33 years, but the condition has been reported in patients ranging from 9 to 76 years of age. There is no significant gender predilection.

Pathology

Retiform hemangioendothelioma is characterized by its distinctive "net-like" or "retiform" vascular pattern. The tumor is composed of elongated, thin-walled blood vessels that closely resemble the normal rete arteriole of the skin. The vessels are lined by a single layer of endothelial cells and are often accompanied by a dense inflammatory infiltrate.

Diagnosis

The diagnosis of retiform hemangioendothelioma is primarily based on histological examination. Immunohistochemical staining can be helpful in confirming the diagnosis. The tumor cells are typically positive for vascular markers such as CD31, CD34, and Factor VIII.

Treatment and Prognosis

The treatment of choice for retiform hemangioendothelioma is complete surgical excision. Despite its histologically malignant appearance, the tumor has a benign clinical course and metastasis is extremely rare. However, local recurrence is common and long-term follow-up is recommended.

See Also

• Vascular tumor
• Hemangioendothelioma
• CD31
• CD34
• Factor VIII

References

Retiform hemangioendothelioma Resources
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