HEMANGIOMA

When blood vessels rapidly proliferate, they form a typically benign mass or lump called a hemangioma. Although most of these tumors occur in the skin and subcutaneous tissues.  However, they can occur anywhere in the body. They are rarely cancerous, but may conflict with vision, breathing, or eating and may present cosmetic problems of varying degree. Hemangiomas may be present at birth (the less common, congenital type), shortly thereafter (infantile type), or later in life. Growths on the surface of the skin are typically red; growths under the skin are bluish-purple. Hemangiomas are the fourth most common tumor of the hand. The palm may have the highest incidence because of its rich, vascular networks. Intramuscular hemangiomas, although uncommon, can develop in the upper extremity and occur at any age. Overall, hemangiomas affect the upper extremity in 15% of patients.^1-4

Pathophysiology

• General causes are known for specific hemangioma types, but exact mechanisms are not. For example, in the infantile form, fetal hemangiomas may arise owing to faulty vascular system development; however, the underlying cause of this “fault” is not known. During the “involution” phase, hemangiomas undergo a slow process of softening, shrinkage, and color change. Involution occurs by 5 years of age in 50% of cases, and by 7 years in 70% of cases.
• In the congenital form, non-involuting hemangiomas are fully formed at birth. They may show pallor and lack glucose transporter 1 (GLUT-1) immunoreactivity. Involuting hemangiomas are ofen gray and also lack GLUT-1 immunoreactivity.
• Hemangiomas may develop after injury or during pregnancy; their etiologies remain unestablished.
• Finally, some hemangiomas are associated with genetic mutations.

Related Anatomy

The most common types of hemangioma are:

• Capillary: comprises an abnormal number of small capillaries, connected by tissue and tightly packed, clustering near the surface of the skin; the most common hemangioma in the hand
• Cavernous: comprises large blood vessels that have dilated and have “caverns” of blood with them
• Compound: a combination of capillary and cavernous types
• Bone: usually occur in the skull and spine in those aged 50–70 years

Incidence and Related Conditions

• Hemangiomas are the most common benign tumor in infants, affecting up to 10%.¹ Typically they become visible before 4 weeks of age and complete most growth by 5 months of age.³ Close attention must be paid in case vital functions are affected. If more than five infantile hemangiomas are present, then ultrasonographic screening for hepatic infantile hemangiomas is recommended.¹ Some types of hepatic infantile hemangiomas can lead to severe complications and death.¹ However, most infantile hemangiomas will shrink completely on their own, without treatment.⁴

Differential Diagnosis

• Vascular malformations (eg, angiosarcoma)
  • Arteriovenous malformation
  • Capillary (port-wine stains)
  • Lymphatic lesions
  • Venous lesions such as venous aneurysms

The typical presentation will vary according to the age of the patient and the type of hemiangioma.  In the first case shown here, the patient was a right handed young teenager with a red skin birth mark on the dorsum of the left long finger.  The red mark had been present since birth but recently a painful enlarging lump had developed under the birth mark.

• Ulceration
• Infection
• Bleeding
• Hemorrhage
• Recurrence
• growth disturbance

• Outcomes vary widely depending on patient age, hemangioma location, and complicating factors
• Successful conservative approaches are usually palliative
• Surgical approaches generally result in complete excision of the lesion and stabilization of symptoms

• Hemangiomas are generally soft with palpation
  • Superficial: lobulated and bright red (eg, “capillary”)
  • Deep: blue or skin color (eg, “cavernous”)
• Capillary: typically bright red, any size, flat or raised; some are a spongy mass (“diffuse hemangioma”)
• Cavernous: sometimes appear as a bluish swelling under the skin and may be any size; the swelling associated with this type may decrease if the hand is elevated
• Intramuscular: often painful; pain and swelling increases with activity
• Bone: usually do not exhibit symptoms, but may cause pain, bone enlargement or growth disturbance.
• Hemangiomas along with boney and soft tissue hypertrophy and varicose veins are part of Klippel-Trenaunay-Webers syndrome.
• Hemangiomas along with multiple enchondromas are part of Maffucci Syndrome.

Cited Articles

1. Gnarra M, Behr G, Kitajewski A, et al. History of the infantile hepatic hemangioma: From imaging to generating a differential diagnosis. World J Clin Pediatr 2016;5(3):273-80. PMID: 27610342
2. Jacobs BJ, Anzarut A, Guerra S, et al. Vascular anomalies of the upper extremity. J Hand Surg Am 2010;35:1703-9. PMID: 20888510
3. Darrow DH, Greene AK, Mancini AJ, Nopper AJ. Diagnosis and management of infantile hemangioma: Executive summary. Pediatrics 2015;136(4):786-91. PMID: 26416928
4. American Academy of Orthopedic Surgeons. Hemangiomas. 2012. Accessed January 24, 2017 at:  http://orthoinfo.aaos.org/topic.cfm?topic=A00630

Reviews

1. Willard KJ, Cappel MA, Kozin SH, Abzug JM. Congenital and infantile skin lesions affecting the hand and upper extremity, part 1: vascular neoplasms and malformations. J Hand Surg Am 2013;38:2271-83. PMID: 23707594
2. Darrow DH, Greene AK, Mancini AJ, Nopper AJ. Diagnosis and management of infantile hemangioma: Executive summary. Pediatrics 2015;136(4):786-91. PMID: 26416928

Classic

1. Palmieri TJ. Subcutaneous hemangiomas of the hand. J Hand Surg 1983;8:201-4. PMID: 6300216

Other

1. American Academy of Orthopedic Surgeons. Hemangiomas. 2012. Accessed January 24, 2017 at:  http://orthoinfo.aaos.org/topic.cfm?topic=A00630
2. Massachusetts General Hospital/Harvard Medical School Neurovascular Center. Cavernous malformations. 2007. Accessed January 24, 2017 at https://neurosurgery.mgh.harvard.edu/neurovascular/v-w-94-2.htm