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Kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon

https://doi.org/10.1016/j.jaad.2004.10.880Get rights and content

Kasabach-Merritt phenomenon is a serious coagulopathy associated with kaposiform hemangioendothelioma (KHE), tufted angioma, and possibly other vascular neoplasms. KHE presenting in the absence of Kasabach-Merritt phenomenon is rare, although tufted angioma frequently occurs without thrombocytopenia. We retrospectively reviewed 10 cases of KHE without Kasabach-Merritt phenomenon. The tumors appeared as soft tissue masses with the overlying skin being either normal, erythematous, or violaceous. There were no radiologic or microscopic differences in noncoagulopathic KHE as compared with coagulopathic KHE. Evidence of platelet trapping and hemosiderin deposition was seen histologically, despite normal serum platelet levels. All KHE were less than 8 cm in diameter, suggesting that tumors that grow no larger than this size are less likely to trap platelets in sufficient quantity to cause thrombocytopenia. Our series confirms that KHE appears with a wide spectrum of behavior and response to treatment. The decision as to whether or not to treat a noncoagulopathic KHE should be based on the size and location of the tumor and the possible side effects of therapy.

Section snippets

Materials and methods

A total of 9 patients with KHE in the absence of thrombocytopenia were culled from the Vascular Anomalies Center at Children's Hospital, Boston, Mass, database by diagnosis. These patients were seen from 1990 to 2003. One patient was included from the Department of Dermatology, University of California at San Francisco. We reviewed the medical records, available radiology, and histology from all 10 patients.

Clinical features

The study group was composed of 10 infants and children (Table I). Tumors were first noted from birth to 2 years of age; they were located on the trunk (n = 2), upper and lower extremities (n = 6), and cervicofacial region (n = 2). The lesions were 1 to 3 cm in diameter at presentation and grew up to 3 to 8 cm in diameter. The most common appearance was a soft tissue mass with cutaneous findings that ranged from an erythematous papule, plaque, or nodule to an indurated, purple, and firm tumor (Fig 1

Discussion

We were curious as to why some infants with KHE develop thrombocytopenia whereas others do not. We looked at clinical, radiologic, and histologic features that might correlate with platelet trapping.

It is generally accepted that KMP is far less likely with TA than with KHE. Wilson Jones et al,7, 11 who were first to describe TA, later described 20 patients with TA without KMP, but they did not document lesional size. These tumors were most commonly located on the neck, upper aspect of chest,

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    Funding sources: None.

    Conflicts of interest: None identified.

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