Vascular and lymphatic neoplasms

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Chapter 42 Vascular and lymphatic neoplasms

Joseph G. Morelli, MD

1. Which is the most common benign vascular neoplasm of childhood?

Hemangioma of infancy (HOI), which is a benign tumor of capillary endothelium. This tumor typically presents at ages 2 to 8 weeks and then goes through a rapid growth phase for the first 6 to 9 months of life. It then begins to regress, with complete regression in 50% of patients by age 5 years. Regression does not necessarily imply return of the skin to normal.

Kilcline C, Frieden IJ: Infantile hemangiomas: how common are they? A systematic review of the medical literature, Pediatr Dermatol 25:168–173, 2009.

2. What are the clinical subtypes of HOI?

• Superficial: Bright red papules

• Deep: Soft blue nodule (Fig. 42-1)

• Mixed: Combination of the above

Figure 42-1. Deep hemangioma.

Most HOI are clinically the mixed type.

3. Name the complications of hemangiomas.

Obstruction of a vital function (such as vision, breathing, eating, defecation, or urination), ulceration followed by bleeding and infection, and high-output cardiac failure. High-output cardiac failure rarely results from a solitary cutaneous HOI.

4. What is the most common complication of hemangiomas?

Ulceration is the most frequent complication and occurs predominantly in the diaper areas (Fig. 42-2).

5. Are there any residua of hemangiomas after regression?

Hypopigmentation, telangiectasia, excess skin, fibrofatty deposits, and, if ulceration occurred, scarring (Fig. 42-3).

6. What is Kasabach-Merritt syndrome?

Platelet trapping and consumption coagulopathy associated with a vascular tumor.

Figure 42-2. A, Ulcerated hemangioma. B, The same hemangioma three months after one pulsed dye laser treatment.

Figure 42-3. Scarring, hypopigmentation, and telangiectasia after regression of a hemangioma.

Rodriguez V, Lee A, Wiltman PM, Anderson PA: Kasabach-Merritt phenomenon: case series and retrospective review of the Mayo Clinic experience, J Pediatr Hematol Oncol 31:522–526, 2009.

7. Name the two vascular tumors most commonly associated with the Kasabach-Merritt syndrome.

Kaposiform hemangioendothelioma and tufted angioma.

8. Port wine stains and lymphangiomas are not neoplasms. What are they?

They are vascular malformations.

9. What is the difference between a vascular malformation and a vascular neoplasm?

A vascular malformation is a developmental error. A vascular neoplasm is a tumor. Vascular malformations stay rather static and grow proportionately with the patient. Vascular tumors grow at a rate greater than the patient does.

10. What is Klippel-Trénaunay syndrome?

This is overgrowth of an extremity secondary to a vascular malformation. The overgrowth invariably involves soft tissue but can also affect the bone (Fig. 42-4). The vascular malformation may be a capillary malformation (port wine stain), a venous malformation, or a combination of the two.

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