Is epithelioid hemangioendothelioma malignant?

Is epithelioid hemangioendothelioma malignant?

Background. Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular neoplasm with increasing incidence. However, its clinical characteristics remain unclear due to its low incidence.

What causes epithelioid hemangioendothelioma?

What is the genetic cause of EHE? As is the case with many other cancers, EHE is caused by a genetic malfunction, a specific chromosomal rearrangement or translocation (swapping of genetic material) called the ‘WC Fusion. ‘ This discovery was made by dedicated scientists studying the molecular biology of EHE.

What is infantile hemangioendothelioma?

Infantile hemangioendothelioma is the most common hepatic vascular tumor in infants less than 6 months of age, with a prevalence of 1%. Serum alpha-fetoprotein levels have been used as an important tumor marker for hepatoblastoma, hepatocellular carcinoma, and germ cell tumors.

What are symptoms of EHE?

What are the symptoms of epithelioid hemangioendothelioma?

  • Tumors in the liver may cause abdominal pain, weight loss, blood work alterations or an abdominal mass.
  • Lesions in the lungs may cause chronic dry cough, shortness of breath or other problems.

How long can you live with EHE?

Although localized epithelioid hemangioendothelioma can be surgically resected, there is no effective therapy for systemic disease, and mortality from the cancer ranges between 13% and 18% when confined to soft tissue, but life expectancy in metastatic cases is unpredictable and ranges from a couple of months to 15 to …

What immunohistochemically findings are characteristic of epithelioid hemangioendothelioma?

Immunohistochemically, the tumor cells are positive for ERG, CD31, CAMTA1, focally positive for keratin and are negative for CD34 and TFE3. The morphology and immunoprofile strongly support the diagnosis of WWTR1-CAMTA1 rearranged epithelioid hemangioendothelioma.

What is the history of epithelioid hemangioendothelioma?

The term epithelioid hemangioendothelioma was introduced in 1982 by Weiss and Enzinger to describe a vascular tumor of bone and soft tissue showing features between hemangioma and angiosarcoma.

What is the ICD 10 code for Epithelioid hemangioma?

ICD coding ICD-O: 9133/3 – epithelioid hemangioendothelioma, malignant ICD-10: D18.0 – hemangioma ICD-11: 2B5Y & XH9GF8 – epithelioid hemangioendothelioma, NOS

What are the histologic features of pulmonary-epithelioid hemangioendothelioma (PE)?

Table 4. Pulmonary-epithelioid hemangioendothelioma histological and cytologic features. Solitary or multiple pulmonary nodules Macroscopical appearance Diameter >5 cm Consistency Rubbery or cartilage-like Cut surface From grey-white to yellow-brown Semi- or normotranslucency Microscopical appearance Periphery of the nodules Hypercellular