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Epithelioid Hemangioendothelioma (EHE)

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Bone, Muscle, & Joint
Diseases & Conditions
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Contributed byKrish Tangella MD, MBASep 10, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Adult Epithelioid Hemangioendothelioma
  • Intravascular Bronchioloalveolar Tumor
  • Low Grade Anaplastic Angiosarcoma

What is Epithelioid Hemangioendothelioma? (Definition/Background Information)

  • An Epithelioid Hemangioendothelioma (EHE) is a tumor involving the blood vessels and surrounding epithelioid cells. The blood vessels are lined by epithelioid cells, which grow abnormally to form the tumor
  • A majority of the tumors act benign; however, these tumors have a malignancy potential (capacity to metastasize)
  • Epithelioid Hemangioendothelioma occurrence is extremely rare, representing 1% of all vascular tumors. The tumor can occur as a single mass or multiple masses
  • EHE frequently affects the soft tissues, just below the skin (subcutaneous tissue). It can also occur deep inside the body organs
  • This tumor has been observed in the liver, lungs, limbs, bones (vascular tumor of bone), and on the skin. Teenagers and young adults are most commonly affected; though, it may be found in any age group
  • An individual may experience a painful mass and tenderness at the site, due to the tumor. Some of them are asymptomatic, causing no significant signs and symptoms

Who gets Epithelioid Hemangioendothelioma? (Age and Sex Distribution)

  • Epithelioid Hemangioendothelioma may occur in individuals of all age group. The peak age range is between teenage and young-adult period
  • Infants and very young children are rarely affected
  • EHE affects both sexes (males and females) equally
  • There is no predilection to any ethnic group or a particular race

What are the Risk Factors for Epithelioid Hemangioendothelioma? (Predisposing Factors)

The following risk factors are suggested for Epithelioid Hemangioendothelioma:

  • Any developmental tissue abnormality involving the soft tissues, could be a risk factor
  • Epithelioid Hemangioendothelioma of the liver is linked to oral contraceptives

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Epithelioid Hemangioendothelioma? (Etiology)

  • The exact cause of Epithelioid Hemangioendothelioma development is unknown; they are thought to occur spontaneously
  • It is suggested that EHE might be related to abnormal blood vessel proliferations originating from veins, for unknown reasons

What are the Signs and Symptoms of Epithelioid Hemangioendothelioma?

The presentations are based on the location of the tumor. Epithelioid Hemangioendothelioma signs and symptoms include:

  • Most tumors are asymptomatic; however, in others, a wide-range of behavior is observed
  • They may grow at a slow rate and appear as painless, irregular/nodular masses below the skin surface (subcutaneous). They may also be present as deep-seated tumors
  • They may be present as well-defined, single or multiple nodules at different locations. Normal skin color changes (to red-brown or pink) are noticed, if these nodules are present subcutaneously
  • They are locally aggressive and may damage the surrounding tissues
  • Most common location of EHE is the liver, lungs, bones, feet, and skin. Other locations include the spine, ribs, and leg

How is Epithelioid Hemangioendothelioma Diagnosed?

A diagnosis of Epithelioid Hemangioendothelioma is made using the following tools:

  • Complete physical examination with a thorough evaluation of medical history
  • X-ray studies of the affected region: Though, by use of this method, it is generally difficult to distinguish between other kinds of hemangiomas, such as telangiectatic osteosarcoma, aneurysmal bone cyst, fibrous dysplasia, etc.
  • CT, MRI scan of the affected region
  • A tissue biopsy of the tumor: A tissue biopsy is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together the clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be the gold standard, in arriving at a conclusive diagnosis
  • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, histochemical stains, molecular testing, and very rarely, electron microscopic studies
  • Differential diagnosis, to eliminate other tumor types are often considered, before arriving at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Epithelioid Hemangioendothelioma?

Complications are dependent on the site and severity of the tumor. These include:

  • Liver degeneration, paraplegia, due to damaged spinal cord, multiple fused bones
  • Recurrence of Epithelioid Hemangioendothelioma after surgery
  • Blood loss during invasive treatment methods may be heavy; blood may accumulate outside the blood vessels, causing hematomas
  • Severe pain, if internal organs are affected
  • Amputation of a limb
  • Damage to vital nerves, blood vessels, and surrounding structures, during surgery
  • Side effects from chemotherapy (such as toxicity), radiation therapy

How is Epithelioid Hemangioendothelioma Treated?

Treatment measures for Epithelioid Hemangioendothelioma include the following:

  • Wide surgical excision of Epithelioid Hemangioendothelioma with removal of the entire lesion is adopted, if necessary. If the lesion is not fully removed, then it might recur
  • Embolization is used to provide temporary relief from the symptoms, and reduce blood loss during a surgical procedure
  • Aggressive lesions may require critical management: Bone tumors may require the use of bone grafts; amputations may be recommended for lesions on the limbs. Liver tumors may require a liver transplant
  • When EHE is at an inaccessible location, or is unsafe for surgical intervention; radiation therapy, chemotherapy, interferon injections, oral medications are employed
  • Post-operative care is important: A minimum activity level is to be ensured, until the surgical wound heals
  • Follow-up care with regular screening and check-ups are important

How can Epithelioid Hemangioendothelioma be Prevented?

  • Current medical research have not established a way of preventing Epithelioid Hemangioendothelioma formation
  • Regular medical screening at periodic intervals with blood tests, radiological scans, and physical examinations are mandatory for those, who have already endured EHE, due to its metastasizing potential and chances of recurrence. Often several years of active vigilance is necessary

What is the Prognosis of Epithelioid Hemangioendothelioma? (Outcomes/Resolutions)

  • Epithelioid Hemangioendothelioma is an extremely rare tumor with metastasizing/malignancy potential. The metastatic tumors can be fatal; the mortality with an aggressive EHE is between 10-20%
  • The long-term prognosis depends on a combination of factors, such as first appearance/detection of the tumor, size and location, its response to treatment, and medical therapy
  • Some tumors are self-limiting, asymptomatic, and may not require any treatment; they may even regress completely
  • The prognosis for Epithelioid Hemangioendothelioma is generally good, when the lesions are small and found below the skin surface. Their recurrence risk is also very low after surgery, if they are completely removed
  • The probability of EHE recurrence is moderate at 10-15%, after surgical removal of the tumor. Hence, regular follow-ups are required
  • In spite of all treatment measures, some Epithelioid Hemangioendothelioma can metastasize causing multi-organ dysfunction syndrome (MODS)

Additional and Relevant Useful Information for Epithelioid Hemangioendothelioma:

It is difficult to manage Epithelioid Hemangioendothelioma, due to the following factors:

  • The extreme rarity of the tumor
  • Its differential behavior
  • The several regions and organs of the body it can potentially affect
  • A lack of standardized treatment guidelines in medical literature on Epithelioid Hemangioendothelioma (EHE)
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Krish Tangella MD, MBA picture
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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