Search

Kaposiform hemangioendothelioma (KHE) is presently classified as a vascular neoplasm of intermediate malignant potential. The clinical course of large, deep-seated tumors is frequently complicated by consumptive coagulopathy and life-threatening hemorrhage, while superficial tumors tend to behave in an indolent manner, with no known reports of distant metastasis. We describe an unusual example of KHE occurring as an incidental microscopic finding, within a background of extensive lymphangioma-like changes. The patient underwent 4 intralesional excisions over a period of 6 years, and the Kaposiform component accounts for less than 5% of the overall tissue excised. The patient remains clinically well with residual disease 5 years after conservative surgery, and there has been no evidence of regional or distant metastasis. Based on existing literature, it appears doubtful that KHE has any metastatic potential at all, which calls into question the appropriateness of its place in the spectrum of malignant vascular neoplasms. [ABSTRACT FROM AUTHOR]

Abstract: The kaposiform hemangioendothelioma (KHE) is an uncommon vascular neoplasm of borderline or intermediate malignant potential in which involvement of the head and neck region is rare. A 5-year-old girl was admitted to the emergency room with abrupt massive hemoptysis. Transoral laryngoscopy revealed a round, reddish mass with active bleeding on the anterior portion of the left vocal fold region. Since re-hemoptysis and large amount of aspiration caused breathing difficulties for the patient, suspension exam was performed under general anesthesia. Mass excision and thorough bleeding control was performed with laser assisted laryngo-micro system. Postoperatively, there was no further bleeding. The pathologic diagnosis of the tumor was KHE. To the best of our knowledge, this is the first report of a KHE in the larynx presenting as recurrent hemoptysis in childhood. [Copyright &y& Elsevier]