Dermatology Online Journal

Rapidly involuting congenital hemangioma: case report and review of the literature
John C Browning MD, Denise W Metry MD
Dermatology Online Journal 14 (4): 11

Department of Dermatology, Baylor College of Medcine andDermatology Service, Texas Children's Hospital, Houston, TX. jcbrowni@texaschildrenshospital.org

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Abstract

Various forms of angiomata may be encountered in the neonate and infant. Both histology and histochemistry, along with history and morphology, may help establish the correct diagnosis. We report a case of rapidly involuting congenital hemangioma and summarize the features characteristic of this vascular neoplasm.

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Clinical Synopsis

A newborn male infant was born via an uncomplicated Caesarean-section performed because a prenatal ultrasound that showed a prominent neck mass and atrial enlargement. Prenatal MRI demonstrated the mass to be vascular in nature. On physical examination in the newborn nursery, a 10 x 7cm firm, violaceous tumor was present on the left shoulder (Fig. 1). Repeat MRI showed the mass to be superficial, limited to the dermis and subcutis, and confirmed the prominent vascularity.

Figure 1	Figure 2

Echocardiogram demonstrated mild right atrial and right ventricular dilation, though the infant was hemodynamically stable. Routine laboratories were significant for a decreased platelet count of 46,000 platelets/microliter. The diagnoses of kaposiform hemangioendothelioma (KHE) and rapidly involuting congenital hemangioma (RICH) were considered. Treatment with prednisolone 3 mg/kg/day was initiated, and a significant reduction in size, softening and lightening of color occurred over the following week (Fig. 2). The prednisolone was slowly tapered over the next 2 months, during which time the appearance of the tumor continued to improve. At that time, biopsy was obtained for definitive diagnosis, and was consistent with a RICH. This diagnosis was supported by a lack of spindle cells as would be characteristic of a KHE, and lack of Glut-1 staining typical of infantile hemangioma (IH).

Discussion

Background

The rapidly involuting congenital hemangioma and its cousin, the non-involuting congenital hemangioma (NICH), were thought to be congenital forms of IH until North and colleagues demonstrated in 2000 that GLUT-1 is expressed in IH but not in these congenital subtypes [1]. North further went on to demonstrate that congenital hemangiomas and IH have distinguishing histologic features [2]. Congenital hemangiomas were subsequently divided into those present at birth that do not involute (NICH) [3] and those that undergo rapid involution within the first year of life (RICH) [4]. It was later observed that some cases of RICH undergo rapid but incomplete involution, with a resulting clinical appearance and histology similar to NICH. It has thus been proposed that RICH and NICH may lie within the same spectrum of vascular tumors [4].

Course

The rapidly involuting congenital hemangioma seems to be uncommon but not rare. It is likely often under-recognized and/or misdiagnosed. By definition, RICH is present at birth, and most commonly occurs on the limb, head, or neck [5]. In some cases it has been associated with heart failure from cardiac overload [6]. The rapidly involuting congenital hemangioma has also been associated with decreased platelets, thought to be due to localized intravascular coagulation [6]. This is in contrast to the profound thrombocytopenia seen with the Kasabach-Merritt phenomenon that occurs with KHE and tufted angioma.

In most cases, RICH involutes completely by age 1-year, although generally with some degree of atrophic or redundant skin. Involution is not always complete, in which case the residual tumor may have the clinical and histologic appearance of a NICH [5]. The underlying cause for RICH has yet to be established.

Diagnosis

Most cases of RICH can be diagnosed by history and physical examination. However, it is important to keep in mind the possibility of a sarcoma or other malignant tumor in the differential diagnosis. Therefore, if the tumor enlarges or develops a firm texture, a biopsy is warranted. Despite the proposed inclusion within the same spectrum, NICH has a very different clinical appearance, tending to be plaque-like with a pink or purple color and prominent overlying coarse telangiectasias [5].

The MRI of RICH shows a vascular tumor, similar in appearance to IH, which may demonstrate flow voids. In addition, fast flow can often be seen with ultrasonography [5]. Histopathologic features include small capillary lobules separated by abundant collagen. Draining channels are prominent in number and size [6].

Treatment

Observation remains the standard of care for uncomplicated cases of RICH. Ulceration and bleeding, as may occur with all vascular tumors, can be early complications. In these cases, early surgical intervention may be the best option. Other treatment options, when ulceration occurs, include petrolatum under occlusion, pulse-dye laser therapy, and topical becaplermin. The latter two options have been reported to be of benefit in treating ulcerated IH [7, 8] and presumably would be of benefit for ulcerated RICH as well. In cases of hemodynamic instability, cardiology consultation should be obtained. Surgery can often correct any post-involution redundancy. In one case, dermal injection with synthetic filler helped improve atrophic skin following involution of a RICH [9].

Conclusion

The rapidly involuting congenital hemangioma often has an impressive appearance at birth, and may be mistaken for a KHE or malignancy. Rapid improvement, however, helps to distinguish it from these entities. Further study will help determine the reasons behind the development and unique behavior of the RICH.

References

1. North PE, Waner M, Mizeracki A, Mihm MC Jr. Glut1: a newly discovered immunohistochemical marker for juvenile hemangiomas. Hum Pathol 2000;31:11-22. PubMed.

2. North PE, Waner M, James C, Mizeracki A, Fieden I, Mihm M. Congenital nonprogressive hemangioma. Arch Dermatol 2001;137:1607-1620. PubMed.

3. Enjolras O, Mulliken JB, Boon LM, Wassef M, Kozakewich HP, Burrows PE. Non-involuting congenital hemangioma: a rare cutaneous vascular anomaly. Plast Reconstr Surg 2001;107:1647-54. PubMed.

4. Berenguer B, Mulliken JB, Enjolras O, Boon LM, Wassef M, Josset P, Kozakewich HP. Rapidly involuting congenital hemangioma: clinical and histopathologic features. Pediatr Develop Pathol 2003;6:495-510. PubMed.

5. Krol A, MacArthur CJ. Congenital hemangiomas: rapidly involuting and non-involuting congenital hemangiomas. BArch Facial Plast Surg 2005;7:307-311. PubMed.

6. Mulliken JB, Bischoff J, Kozakewich HPW. 2007. Multifocal rapidly involuting congenital hemangiomqa: a link to chorangioma. Am J Med Genet Part A. PubMed.

7. Metz BJ, Rubenstein MC, Levy ML, Metry DW. Response of ulcerated perineal hemangiomas of infancy to becaplermin gel, a recombinant human platelet-derived growth factor. Arch Dermatol. 2004 Jul;140(7):867-70. PubMed.

8. Thomas RF, Hornung RL, Manning SC, Perkins JA. Hemangiomas of infancy: treatment of ulceration in the head and neck. Arch Facial Plast Surg. 2005 Sep-Oct;7(5):312-5. PubMed.

9. Lopez-Gutierrez JC, Diaz M, Ros Z. Giant rapidly involuting congenital hemangioma of the face: 15-year follow-up. Arch Facial Plast Surg 2005;7:316-318. PubMed.

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