Truncal location of hemangioendotheliomas may indicate potentially more serious involvement with resulting Kasabach-Merritt syndrome than size determinant alone

Carrington, Patrick R.

doi:10.1016/j.jaad.2005.09.016

« Previous Next »Journal of the American Academy of Dermatology
Volume 54, Issue 5 , Pages 922-923, May 2006

Truncal location of hemangioendotheliomas may indicate potentially more serious involvement with resulting Kasabach-Merritt syndrome than size determinant alone

Dermatology Service, Central Arkansas Veterans Healthcare System, Department of Dermatology, University of Arkansas for Medical Sciences, Little Rock, Arkansas

To the Editor: The intriguing article by Gruman et al¹ concludes that a kaposiform hemangioendothelioma 8 cm or less in size is less likely to trap platelets, reducing the chance of Kasabach-Merritt phenomenon. They note that the decision to treat should be based on size and location. As seen in their Table I, 7 of their 9 patients had vascular tumors in nontruncal location and, although size was thought to be a major determinant of better prognosis (meaning no Kasabach-Merritt phenomenon), one might make the case that invasive tumors remain difficult to accurately size or quantify during routine physical examination or even with magnetic resonance imaging and computed tomography because of their invasive growth behavior. The article, “Kasabach-Merritt syndrome with bone involvement: the pseudomalignant sign of Gorham,”² detailed a case that was truncal (mediastinal), but was also associated with lytic changes of vertebral bodies. During clinical conference with family members, pediatricians, radiologists, and pathologists involved in our case, the possibility of malignancy was very strongly considered. The outcome, however, encouraged us to conceive of the pseudomalignant sign of Gorham as an indication of bone lysis with replacement by vascular tumor not associated with malignancy in this age group. This pays homage to Gorham et al³ and their clinical contribution detailing disappearing bone as a manifestation of massive osteolysis, but also highlights the fact that truncal (peritoneal or mediastinal) tumors associated with bony dissolution may represent vascular neoplasms rather than malignancy in the pediatric patient. Our patient had Kasabach-Merritt phenomenon but did well and has grown to a young man.

We would propose that truncal kaposiform hemangioendotheliomas, independent of size, are more worrisome for concomitant platelet trapping, especially in the presence of bony resorption, lysis, or replacement of bone by vascular neoplasm. Malignant childhood neoplasms are disproportionately represented in tumors of the mediastinum or retroperitoneum associated with bone loss, but benign vascular neoplasms may be a simulant of such severe disease.

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References

Gruman A, Liang MG, Mulliken JB, Fishman SJ, Burrows PE, Kozakewich HPW, et al. Kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon. J Am Acad Dermatol. 2005;52:616–622

Carrington PR, Rowley MJ, Fowler M, Megison RP, Meyers P. Kasabach-Merritt syndrome with bone involvement: the pseudomalignant sign of Gorham. J Am Acad Dermatol. 1993;29:117–119

Gorham LW, Wright AW, Shultz HH, Maxon FC. Disappearing bones: a rare form of massive osteolysis–report of two cases, one with autopsy findings. Am J Med. 1954;17:674–682