Abstract
BackgroundRetiform Hemangioendothelioma (RH) is an extremely rare vascular tumor of intermediate biological behavior, which is prone to local recurrence but rarely shows metastasis to distant sites. It may harbor areas resembling Dabska tumor in some cases and angiosarcoma, which in its well differentiated form may exhibit similar pathological appearance in some areas, making it problematic to rule out a possibility of a malignant diagnosis on a core biopsy. Therefore, complete surgical resection with negative margins is essential for accurate diagnosis and local control.ResultsIn our series, two of the three Pakistani cases were in females, with an age range between 18 and 50 years. Our first patient presented with symptoms of cardiac compromise and pulmonary hypertension. Her computed tomography scan showed multiple tumor masses within the mediastinum. The second patient presented with an ulcerated lesion on his scalp, at right temple. The third patient presented with a hard growth on her left 4th toe which was amputated. Histologically, all cases exhibited retiform arborizing vascular spaces lined by bland endothelial cells with hobnail nuclei, characteristic of retiform hemangioendothelioma. Immunohistochemical markers CD31, CD34 and ERG confirmed the vascular nature of the tumor. The first and the second patient are alive and healthy at 4 and 7 months follow up respectively, while the third patient is lost to follow up.ConclusionOwing to the rate of local recurrence, RH should always be considered in the differential diagnosis of vascular tumors showing arborizing blood vessels, as it may have an atypical presentation and it should be carefully differentiated from Dabska tumor and an angiosarcoma.
Highlights
Retiform Hemangioendothelioma (RH) is an extremely rare vascular tumor of intermediate biologi‐ cal behavior, which is prone to local recurrence but rarely shows metastasis to distant sites
None of these cases showed features like nuclear atypia, spindle cell proliferation forming slit like spaces or more than rare mitotic figures that would suggest a diagnosis of angiosarcoma or Kaposi Sarcoma (KS)
Case 1 A 50-year-old, non-smoker Pakistani female presented with shortness of breath and cough
Summary
Retiform Hemangioendothelioma (RH) is an extremely rare vascular tumor of intermediate biologi‐ cal behavior, which is prone to local recurrence but rarely shows metastasis to distant sites. It may harbor areas resembling Dabska tumor in some cases and angiosarcoma, which in its well differentiated form may exhibit similar pathological appearance in some areas, making it problematic to rule out a possibility of a malignant diagnosis on a core biopsy. We describe the clinicopathological and histological features of 3 cases of RH with thorough review of literature which will help us in establishing correct diagnosis and understanding the behavior of this rare tumor
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