Retiform hemangioendothelioma: A case report and literature review.

Introduction And Importance: Retiform hemangioendothelioma(RH) is a rare vascular tumor affecting patients over a wide age range without a gender predilection; only about 50 cases have been described so far.

Case Presentation: We report a case of submandibular retiform hemangioendothelioma in a 58-year-old woman who had been diagnosed with RH 20 years ago and had experienced recurrence four times during the past 20 years. This will increase the limited number of such cases in the hope of gaining a better understanding of this rare type of tumor. The histological features of RH are characterized by arborizing blood vessels arranged in a testicular network pattern with endothelial cells arranged in a hobnail pattern. Immunohistochemistry revealed CD31, CD34, ERG, and other vascular markers.

Clinical Discussion: Based on the morphological and immunohistochemical results, we diagnosed the patient with a rectiform hemangioendothelioma with local malignancy that transformed into angiosarcoma, with enlargement of the lymph nodes in the neck area, possibly indicating tumor lymph node metastasis. The patient underwent extended resection and completed 14 radiotherapy sessions. Follow-up at 3 months after surgery showed no recurrence.

Conclusion: RH is a low-grade malignant intermediate vascular tumor that is prone to recurrence and does not generally metastasize to distant sites. Patients with recurrent relapses may undergo malignant transformation or lymph node metastases. Treatment primarily relies on wide excision and adjuvant radiotherapy may be necessary when required.