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

• SCH (Spindle Cell Hemangioma)
• Spindle Cell Hemangioendothelioma

What is Spindle Cell Hemangioma? (Definition/Background Information)

• Hemangiomas are benign malformation of blood vessels that can occur in a number of tissues such as the brain, kidney, liver, lung, skin, and nasal cavity
• Spindle Cell Hemangiomas (SCHs) are a special type of hemangiomas, which are benign growths composed of small blood vessels that occur on the skin or within the skin tissues, anywhere in the body. These benign tumors can sometimes rupture and bleed if injured
• They are mostly present as single tumor formations, but multiple vascular tumors may be observed in association with a genetic disorder called Maffucci syndrome, in rare cases
• The cause for development of the condition is not yet well understood. Some studies though, show a genetic component
• Spindle Cell Hemangiomas are generally asymptomatic and may not require any treatment. However, they may be treated for cosmetic purposes, or in case of severe and chronic bleeding. The treatment options include removal of the tumor. The prognosis of the condition depends upon the tumor location, but the prognosis is generally excellent 

Who gets Spindle Cell Hemangioma? (Age and Sex Distribution)

• Spindle Cell Hemangiomas are most common in young adults, though they may be found in other age groups too
• It can affect individuals of all races, ethnic backgrounds, and genders across the world

What are the Risk Factors for Spindle Cell Hemangioma? (Predisposing Factors)

The following risk factors contribute to Spindle Cell Hemangiomas development:

• Individuals with a positive family history are more likely to develop the condition
• Almost 5% of Spindle Cell Hemangiomas have been associated with Maffucci syndrome; in which there are multiple endochromas of bone and multiple vascular tumors

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 Spindle Cell Hemangioma? (Etiology)

• The exact cause for development of Spindle Cell Hemangiomas is unknown
• Since family history is an important risk factor, it may be caused by genetic mutations that are yet to be well-characterized
• Spindle Cell Hemangiomas have been known to occur in association with Maffucci syndrome, a genetic disorder affecting the bone and skin

What are the Signs and Symptoms of Spindle Cell Hemangioma?

Spindle Cell Hemangiomas are generally asymptomatic and do not present any signs and symptoms.

• They occur on the skin (superficial location) or subcutaneously. Some are known to occur in deep tissue
• The hemangiomas may be present anywhere on the body, but are common in the distal extremities
• These benign growths are small, usually single, and slow-growing; they also have a tendency to grow along a blood vessel. This is the reason for their recurrence after surgery
• On the skin, they may appear as small, pigmented nodule
• Rarely the hemangiomas may be many in number
• Sometimes, these benign tumors may rupture and bleed, if they are injured

How is Spindle Cell Hemangioma Diagnosed?

A diagnosis for Spindle Cell Hemangioma includes evaluating the clinical history (physical exam) and a thorough family history. Other tools to help diagnose the condition may include:

• A visual assessment by the physician can help diagnose the condition
• CT and MRI scan of the affected region: Deep-seated tumors may be incidentally discovered during imaging for other reasons
• Radiological studies, such as x-rays and CT scans, may show areas of calcification within the tumor
• 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
• If necessary, skin or tissue biopsies may be performed to diagnose hemangioma and to rule-out other conditions. The pathology reports can help in distinguishing it from other types of hemangiomas

Note: Spindle Cell Hemangiomas are commonly mistaken for Kaposi sarcoma (a malignant tumor). Hence, a careful examination and study of the tissue biopsy is often necessary.

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 Spindle Cell Hemangioma?

The complications of Spindle Cell Hemangiomas may include:

• Bleeding following an injury or trauma
• If large tumors involve the internal organs, it may affect their proper function
• Some individuals may feel stressed due to the presence of these tumors, or due to the cosmetic issues they create
• Recurrence of the tumor following treatment
• Damage to vital nerves, blood vessels, and surrounding structures during surgery to remove the tumor

How is Spindle Cell Hemangioma Treated?

Spindle Cell Hemangiomas do not cause any symptoms in most cases, and therefore may not require any treatment.

• In case of frequent bleeding or cosmetic issues related to physical appearance, individuals may choose to remove them
• Surgical excision and removal of the tumor
• Post-operative care is important: One must maintain minimum activity levels, until the surgical wound heals
• Post-operative follow-up care with regular screening and check-ups are important, especially to monitor for any recurrences

Procedures for removing a Spindle Cell Hemangioma may include:

• Burning (electrosurgery/cautery)
• Freezing (cryotherapy)
• Laser surgical excision

How can Spindle Cell Hemangioma be Prevented?

• Currently there are no specific methods or preventative techniques for the formation of Spindle Cell Hemangiomas. The condition may be caused by genetic factors, when associated with Maffucci syndrome
• If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders

What is the Prognosis of Spindle Cell Hemangioma? (Outcomes/Resolutions)

• Spindle Cell Hemangiomas are noncancerous and mostly asymptomatic. However, they have a tendency to bleed, if they rupture, and the prognosis depends upon the hemangioma location
• Tumors that are located in the superficial regions (such as the skin) have excellent prognosis with complete removal of the hemangioma
• The prognosis is also based on the underlying Maffucci syndrome, if present
• Removal of the hemangioma does not usually lead to scarring

Additional and Relevant Useful Information for Spindle Cell Hemangioma:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/healthy-living/skin-disorders/