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

• Angioblastoma of Nakagawa
• Progressive Capillary Haemangioma
• Tufted Hemangioma

What is Tufted Angioma? (Definition/Background Information)

• Tufted Angioma is an uncommon vascular tumor that is mostly seen in young children to young adults. It can grow very slowly and affect large skin areas on the body
• An angioma is a kind of benign tumor of blood vessel origin. Tufted Angiomas may be seen on any part of the body, though there is a propensity for the upper torso (upper chest and shoulders)
• The cause of formation of Tufted Angioma is unknown. It may occur against a background of conditions including pregnancy, Kasabach-Merritt syndrome, and other vascular tumors/malformations
• The healthcare provider may undertake a treatment of Tufted Angioma presenting severe signs and symptoms. In such cases, a surgical removal of the lesion is the preferred method of treatment
• The prognosis of Tufted Angioma is typically good with suitable therapy. Some tumors, in rare cases, are also known to regress by their own

Who gets Tufted Angioma? (Age and Sex Distribution)

• Tufted Angioma is usually found in children, teenagers, and young adults; most tumors may be observed before the child is 5 years old
• In rare cases, a congenital presentation may be noted. Some are even observed in older adults (late onset)
• There is no gender differentiation and both males and females are affected
• There is no racial or ethnic preference observed

What are the Risk Factors for Tufted Angioma? (Predisposing Factors)

The risk factors for Tufted Angiomas are currently unknown. However, some are known to be associated with the following conditions:

• Hormonal influence due to conditions such as puberty and pregnancy
• Organ transplant, especially liver transplant
• Vascular malformations such as nevus flammeus
• Non-regressing lipodystrophy centrifugalis abdominalis
• Kasabach-Merritt syndrome
• Very infrequently, a positive family history of the tumor has been observed

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 Tufted Angioma? (Etiology)

The exact cause of Tufted Angioma formation is unknown.

• They are known to occur spontaneously or acquired due to an underlying disorder or medical condition
• In case of a family history of the tumor, which is very uncommon, an autosomal dominant manner of inheritance is observed
• Some research scientists have postulated that trauma and hormonal factors can play a role in tumor development

What are the Signs and Symptoms of Tufted Angioma?

The signs and symptoms of Tufted Angioma include:

• It grows at a slow rate and can cover large body areas
• Many tumors may not present any signs or symptoms. Occasionally, some tumors show mild symptoms
• A soft tumor with reddish or brownish discoloration of the overlying skin is observed
• The size of these round or oval lesions may range from 1 cm to several centimeters
• Both solitary and multiple lesions may be present; sometimes, extensive skin involvement is seen, but skin ulceration is rare
• The lesions may be in the form of macules and plaques; sometimes, sclerosing (firm andscar-like) plaques may be seen
• Pain and increased sweating may be noted
• In some cases, a nodular tumor mass is observed below the skin; these may resemble pyogenic granulomas, a much more common vascular growth
• These raised nodules may show a linear formation on the skin, in rare cases
• Most common location of the tumor is the trunk (including the shoulders and upper chest). Other locations include the mouth, arms and legs, head and neck region (face and scalp), etc.
• Signs and symptoms of any underlying condition may be noted

The tumors may grow very slowly, and often they may stop growing for a while. In some cases, the tumors may even appear to shrink in size. However, a systemic involvement and spread of the tumor to other body locations is not observed.

How is Tufted Angioma Diagnosed?

Tufted Angioma may be diagnosed using the following tools:

• Physical exam with evaluation of medical history
• Radiographic studies, such as X-rays and MRI scans of the affected region
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Angiography: It is an imaging technique to study blood vessels and help determine the extent of the tumor
• Skin 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

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 Tufted Angioma?

Complications due to Tufted Angioma could include:

• Severe pain and discomfort (especially in young children)
• Some individuals may have cosmetic concerns and stress due to the presence of the lesions
• Damage to vital nerves, blood vessels, and surrounding structures during surgery
• Recurrence of the tumor after surgery

How is Tufted Angioma Treated?

Even though Tufted Angiomas are benign lesions, a treatment may be performed, if there are significant signs and symptoms, or if large areas of the body are covered. In some individuals, partial and rarely complete regression of this angioma have been observed.

The treatment measures for Tufted Angioma include the following:

• If there are no symptoms, then non-operative measures are adopted (conservative treatment). However, periodic observations are maintained and if there is any pain, it is controlled through pain medication
• Complete surgical excision with removal of the entire lesion is the standard treatment mode used. If the lesion is not fully removed, then there are chances of its recurrence
• In some individuals, cryotherapy or electrocoagulation therapy may be beneficial
• Laser surgery with a device of appropriate wavelength can be effective
• Radiation therapy
• Post-operative care is important with minimum activity level to be ensured, until the surgical wound heals. Also, post-operative follow-up care with regular screening and check-ups are advised, especially to monitor for any recurrences

How can Tufted Angioma be Prevented?

Current medical research has not established a method of preventing Tufted Angioma.

What is the Prognosis of Tufted Angioma? (Outcomes/Resolutions)

• The prognosis of Tufted Angiomas with adequate treatment is usually good. Although, the overall prognosis may depend upon the presence of any underlying/associated condition
• Some tumors are occasionally known to regress spontaneously (either partially or fully)

Additional and Relevant Useful Information for Tufted Angioma:

• An intramuscular angioma is a form of soft tissue (deep-seated) tumor, though its occurrence is rare. It is frequently found in the thigh

The following link may help you find useful additional information on intramuscular angioma:

http://www.dovemed.com/diseases-conditions/intramuscular-angioma/