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

• Intravascular Papillary Endothelial Hyperplasia (IPEH)
• Masson's Pseudoangiosarcoma
• Masson's Tumor

What is Papillary Endothelial Hyperplasia? (Definition/Background Information)

• Papillary Endothelial Hyperplasia is a reactive condition of a blood vessel that may occur at any location, usually superficial, or at a soft tissue location. The cause of the condition is unknown
• Papillary Endothelial Hyperplasia leads to the formation of a single, small mass under the skin (of various colors). The most common location is the thumb, while the arms and legs, head and neck region may also be affected
• The condition may be of 2 types:
  • Primary Papillary Endothelial Hyperplasia: When it occurs in a distended or dilated blood vessel; it is also known as Pure Form of Papillary Endothelial Hyperplasia
  • Secondary Papillary Endothelial Hyperplasia: When it occurs in an existing vascular abnormality; it is also known as Mixed Form of Papillary Endothelial Hyperplasia
• In majority of the cases, there are no significant signs and symptoms or complications associated with Papillary Endothelial Hyperplasia, apart from the visible presence of a lesion on the skin in some cases
• The condition may be treated through a simple excision, and with appropriate treatment, the prognosis of Papillary Endothelial Hyperplasia is generally excellent

Who gets Papillary Endothelial Hyperplasia? (Age and Sex Distribution)

• Papillary Endothelial Hyperplasia is a condition that may be observed in individuals of all ages and both male and female genders
• There is no racial, ethnic, or geographical predilection observed

What are the Risk Factors for Papillary Endothelial Hyperplasia? (Predisposing Factors)

The risk factors for Papillary Endothelial Hyperplasia include preexisting vascular conditions such as:

• Varicose veins
• Hemangiomas
• Pyogenic granulomas
• Lymphangiomas
• Arteriovenous malformations (AVMs)

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 Papillary Endothelial Hyperplasia? (Etiology)

• The exact cause of Papillary Endothelial Hyperplasia is unknown
• Some researchers believe that it is a reactive process to a trauma, or a reactive process occurring in an existing benign vascular tumor
• Research is currently being performed to identify the relevant causative factors

What are the Signs and Symptoms of Papillary Endothelial Hyperplasia?

The signs and symptoms associated with Papillary Endothelial Hyperplasia may include:

• The appearance of a small and solitary mass under the skin, which can be pigmented in colors ranging from blue to red
• It may be present just under the skin (superficial location) or at a soft tissue location, at any body part; frequently, it is observed in the thumb
• Other locations include the arms and legs, head and neck regions

How is Papillary Endothelial Hyperplasia Diagnosed?

Papillary Endothelial Hyperplasia may be diagnosed using the following tools:

• Complete physical exam and evaluation of medical history
• Examination of the skin by a dermatologist using a special magnified lens (dermoscopy)
• Skin or tissue biopsy: A biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Note: Papillary Endothelial Hyperplasia can be mistaken for an angiosarcoma (a malignant tumor of the blood vessels). Hence, during the exam of the tissue biopsy, angiosarcoma should be considered as a differential 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 Papillary Endothelial Hyperplasia?

• In majority of the cases, there are no significant complications associated with Papillary Endothelial Hyperplasia
• However, the condition may cause cosmetic issues in some individuals, depending on its location

How is Papillary Endothelial Hyperplasia Treated?

The treatment of Papillary Endothelial Hyperplasia may include the following:

• A simple surgical excision of the vascular formation is the treatment of choice and is curative
• Follow-up care with regular screening and check-ups are important

How can Papillary Endothelial Hyperplasia be Prevented?

Currently, there is no effective prevention of Papillary Endothelial Hyperplasia available.

What is the Prognosis of Papillary Endothelial Hyperplasia? (Outcomes/Resolutions)

The prognosis of Papillary Endothelial Hyperplasia is excellent, since it is a benign condition that does not cause any significant signs and symptoms or complications in most cases. It may be removed through a simple surgical excision.

Additional and Relevant Useful Information for Papillary Endothelial Hyperplasia:

A differential diagnosis may be considered to eliminate the following conditions:

• Angiosarcoma
• Arteriovenous malformations (AVM)
• Hemangioma
• Lymphangioma
• Pyogenic granuloma
• Varicose vein