Introduction
HEHE (hepatic epithelioid hemangioendothelioma) is an uncommon vascular neoplasm. This neoplasm's natural history varies, ranging from long-term survival without therapy to a quickly progressing path with a deadly outcome. The etiology, characteristics, diagnosis, management, and prognosis of hepatic epithelioid hemangioendothelioma are unknown, and large-scale retrospective investigations are needed to gain a better understanding of this condition.
What Is Hepatic Epithelioid Hemangioendothelioma?
Hepatic epithelioid hemangioendothelioma is an uncommon vascular tumor that was initially identified as a distinct entity by Weiss and Enzinger in 1982. Clinical manifestation varies but is typically characterized by nonspecific symptoms, including right upper quadrant pain and a weight reduction. Some individuals have Budd-Chiari syndrome (obstruction of hepatic venous outflow) or liver failure, whereas others have incidental abnormalities. Diagnosis can be challenging. Many reported cases are misdiagnosed at first.
Compared to other hepatic malignancies, the prognosis is thought to be better even if tumor growth may be progressive and result in hepatic failure, extrahepatic metastases, and death. Hepatic epithelioid hemangioendothelioma is typically classified as a low-to-intermediate-grade cancer. The tumors are histologically constituted of dendritic and epithelioid cells. Signet ring-like formations arise in tumor cells with intracytoplasmic lumina and occasionally red blood cells. The stroma is fibrous and has myxo hyaline regions. Tumors have positive results for at least one endothelial marker and negative results for epithelial markers when examined using immunohistochemistry. Treatment options include hepatic resection, orthotopic liver transplantation, radiation, and chemotherapy.
How Common Is Hepatic Epithelioid Hemangioendothelioma?
Hepatic epithelioid hemangioendothelioma is extremely rare, with only one in every million persons diagnosed worldwide. Because hepatic epithelioid hemangioendothelioma can be difficult to identify, the real number of people suffering from it may be larger.
What Are the Causes of Hepatic Epithelioid Hemangioendothelioma?
Malignant tumors, such as hepatic epithelioid hemangioendothelioma, develop when a healthy cell converts into a cancer cell that grows uncontrollably. Hepatic epithelioid hemangioendothelioma is caused by a specific genetic abnormality, according to researchers. It occurs when two chromosomes link improperly, leading one gene to join another. WWTR1 (also known as TAZ) and CAMTA1 are the genes involved. When WWTR1 and CAMTA1 interact, epithelioid hemangioendothelioma results.
What Are the Symptoms Associated With Hepatic Epithelioid Hemangioendothelioma?
Clinical signs are diverse and nonspecific for hepatic epithelioid hemangioendothelioma. Among symptomatic patients, the most common manifestations were epigastric pain, hepatosplenomegaly, inappetence, distension, weight loss, and fatigue, in addition to other manifestations such as epigastric discomfort, hydrothorax, ascites, fever, and jaundice.
The majority of patients have multiple lesions. At the time of diagnosis, the chance of extrahepatic metastases was nearly half. The lungs, lymph nodes, peritoneum, bones, and spleen were the most prevalent locations of extrahepatic involvement. The pleura and omentum have also been implicated, but infrequently.
How Is Hepatic Epithelioid Hemangioendothelioma Diagnosed?
As hepatic epithelioid hemangioendothelioma rarely causes symptoms, most people find out they have multiple tumors during an imaging test unrelated to EHE. The doctor will take note of the location of the tumor, its size, and whether it has migrated to other places in the body (metastasized).
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CT Scan: A CT (computerized tomography) scan scans the human body with X-rays and projects images onto a computer screen that the doctor can see. A CT scan allows the doctor to see the body's structures such as bones, organs, muscles, and blood arteries.
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MRI: An MRI (magnetic resonance imaging) may be ordered by the doctor to look for epithelioid hemangioendothelioma tumors in the liver and bones. An MRI scans the human body using magnets and radio waves to transmit images onto a computer screen. A whole-body MRI scans the whole person from head to toe. It is very beneficial for finding bone malignancies that have migrated throughout the body.
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Core Needle Biopsy: A core-needle biopsy can determine whether a tumor is epithelioid hemangioendothelioma after it has been detected by CT or MRI. The doctor will use a long hollow needle to extract tissue from the tumor during a core-needle biopsy. A pathologist, a laboratory specialist, analyzes the tissue under a microscope for symptoms of malignancy. A biopsy also allows the doctor to rule out other disorders that could be misdiagnosed with epithelioid hemangioendothelioma, such as breast cancer.
How Is Hepatic Epithelioid Hemangioendothelioma Managed?
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Wait and See - In some circumstances, the tumor grows slowly or even shrinks in the absence of treatment. In this scenario, it may be best for the doctor to monitor the tumor rather than treat it.
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Surgery - Surgery is most commonly used to treat epithelioid hemangioendothelioma that has not progressed to other sections of the body. Surgery can be performed in conjunction with chemotherapy and radiation therapy.
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Radiation Therapy - With or without surgery, doctors may utilize radiation therapy to treat epithelioid hemangioendothelioma. Radiation therapy is typically used to treat epithelioid hemangioendothelioma that develops in the bones. Because radiation therapy has the potential to develop other malignancies in the future, one must consider this option with the doctor.
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Chemotherapy - In addition to other therapies, doctors may employ chemotherapy. It is critical to address the potential side effects with the doctor.
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Targeted Therapy - Because epithelioid hemangioendothelioma is made up of cells that create blood vessels, some doctors are experimenting with treatments that target tumor blood vessels. These medications are known as antiangiogenics.
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Immunotherapy - Immunotherapy employs a variety of treatments to assist the body's immune system in killing tumor cells.
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Embolization - Blocking blood flow to the tumor, also known as embolization, can help shrink and destroy the tumor in some circumstances.
Conclusion
Hepatic epithelioid hemangioendothelioma has a rare and variable clinical course, and individuals with intrahepatic metastases and liver impairment may have a worse prognosis than those without. Regardless of extrahepatic metastases, surgical intervention or transplantation, may be required. For individuals who cannot undergo surgery, doctors might explore combining transhepatic arterial chemotherapy and embolization with antiangiogenic medicines in the therapy of hepatic epithelioid hemangioendothelioma.
