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Clinical Note |

Kaposiform Hemangioendothelioma of the Tongue in an Adult

Jeremy B. White, MD; James Pullman, MD; Bruce Wenig, MD; Richard V. Smith, MD
Arch Otolaryngol Head Neck Surg. 2009;135(8):824-826. doi:10.1001/archoto.2009.104.
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Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor that arises during early childhood and has an equal prevalence in males and females. This lesion typically presents as a bluish-red mass in the superficial or deep soft tissue of the extremities, although it may also appear in the abdominal cavity and retroperitoneum.1 More rarely, KHE has been diagnosed in the head and neck, as reported in a series of 14 patients in the English literature.2 All of these patients were diagnosed at 4 years of age or younger. The other important entity that enters the differential diagnosis is the common infantile hemangioma, which eventually involutes spontaneously. Unlike common infantile hemangioma, however, KHE persists and is strongly associated with the Kasabach-Merritt phenomenon (KMP), over 90% of which present as emergencies caused by KHE.3,4 This phenomenon is characterized by a consumptive coagulopathy that can result in severe thrombocytopenia and hemorrhage, which is often life threatening. In predicting the severity of the lesion, 1 case series of 8 KHE lesions indicates that tumors with a diameter smaller than 8 cm will not trap platelets.5 Metastasis is not considered to be part of the natural history of this lesion, although it has been reported to involve regional lymph nodes and to invade local bony structures such as the temporal bone and temporomandibular joint.6 The following is the first case report, to our knowledge, of KHE presenting in a tongue.

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Figure 2.

Representative microscopic views of the resected tumor. A, Low power (hematoxylin-eosin, original objective magnification ×2.5; image width represents 4.45 mm). Arrows point to submucosal tumor nodules, the largest of which bulges into the mucosa. Upper and lower rectangular boxes in this nodule correspond to areas of different morphologic characteristics shown at high power in B and C, respectively (hematoxylin-eosin, original objective magnification ×40; image width represents 290 μm). B, Larger and smaller interconnected vessels, similar to what is seen in a capillary hemangioma. C, Solid area composed entirely of slitlike vascular spaces similar to those seen in Kaposi sarcoma.

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Figure 1.

Intraoperative image of the tongue lesion at the time of resection, following an initial outpatient biopsy.

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Kaposiform hemangioendothelioma of the tongue in an adult. Arch Otolaryngol Head Neck Surg 2009;135(8):824-6.