Kaposiform Hemangioendothelioma: Case Report and Literature Review

Abstract

We report the identification of a kaposiform hemangioendothelioma (KH) in the oropharynx of a 3-year-old boy. This is a rare endothelial-derived spindle cell neoplasm affecting children and early adolescents with features common to capillary hemangioma and Kaposi sarcoma. Nine cases of head and neck KH have been reported, this being the first in the otolaryngology literature. Our patient underwent wide local excision and has remained tumor free for over 1 year. KH should be considered in the differential diagnosis of a vascular lesion demonstrating unexpected behavior from that of a hemangioma.

Case Report: Kaposiform Hemangioendothelioma in Multiple Spinal Levels Without Skin Changes

“…KMP describes a syndrome of profound thrombocytopenia and coagulopathy that may be associated with KH [41]. KH generally occurs in the soft tissues, often with KMP, and only rarely has been reported to invade bone [16,22,31,32,34,35]. Of the cases of KH invasion into bone, only two have been reported in extracraniofacial sites [31,35].…”

“…We identified six patients with KH with bone involvement reported in the literature [16,22,31,32,34,35] (PubMed search using keyword ''kaposiform hemangioendothelioma'') ( Table 1). All of these patients presented with accompanying cutaneous changes.…”

“…Although our patient lacked the associated skin changes and swelling, these radiographic changes mimic closely those found in her thoracolumbar spine. Other patients with reported bony changes had a large, soft tissue mass associated with the bone lesion [16,22,31,32,34,35]. In the absence of adjuvant therapy, the bone lesions that were surgically unresectable continued to grow and invade adjacent structures [22,31,32].…”

“…Numerous reports recommend primary wide local excision followed by supportive therapy for associated symptoms [13,32,33,40,43,47]. Several nonsurgical treatments, such as radiation [24], embolization [16,41], interferon [15,22,29,34], and the chemotherapeutic regimen of actinomycin D, cyclophosphamide, and vincristine [22,26] have proved successful in managing KH. In cases with KH associated with KMP, no reports in the literature recommended either observation alone or steroids alone.…”

“…San Miguel et al [40] published a thorough review of 158 patients with KH in which 28 of 29 patients treated with surgical excision and some type of initial nonoperative therapy had eradication of their disease. In the six cases reporting primary bony involvement, as is present in this case, four cases involved surgical excision, whereas prednisone and interferon were used in the other two cases [16,22,31,32,34,35] ( Table 1). Our patient had multilevel spine involvement that was surgically unresectable lesions without associated KMP.…”