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Kaposiform hemangioendothelioma is a rare, potentially life-threatening vascular tumor often associated with a coagulopathy known as Kasabach–Merritt phenomenon (KMP). We report the case of an infant with satellite lesions around a large vascular tumor and associated KMP.

Human herpes virus (HHV)-8 is associated with causation of Kaposi's sarcoma (KS). An HIV-positive male with multiple nodules on the body was clinically and histopathologically diagnosed as a case of KS; however, immunohistochemistry was negative for HHV-8. This peculiar scenario of HHV-8-negative tumor with typical clinical and histopathologic findings was labeled as "Atypical spindled endothelial proliferation suspicious of Kaposi sarcoma", hitherto unreported novel entity. [ABSTRACT FROM AUTHOR]

Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm that can be potentially low malignant and mainly affects infants and adolescents. This tumor usually is seen in the skin, soft tissue, and retroperitoneum. This study is a case of KHE in the long bone of the ulna. The subject was a 7-year-old female patient with osteolytic lesion in the forearm without cutaneous lesions. Histologically, the neoplasms comprised of nodules of spindle-to oval-shaped cells that grew in an infiltrative fashion. The results of 2-year follow-up of the patient after enblec resection were desirable and no recurrence was observed. This is the first study to report a case of KHE of the bone in Iran. [ABSTRACT FROM AUTHOR]

Vascular anomalies (VAs) comprise a large variety of individual diagnoses that in different phases of treatment require a diverse number ofmedical specialists to provide optimal care. Medical therapies include agents usually associated with cancer chemotherapy, such as vincristine, as well more immunomodulatory types of drugs, such as glucocorticoids and sirolimus. These immunomodulating drugs are being successfully applied in cases that are typically categorized as vascular tumors, including kaposiform hemangioendothelioma (KHE) and tufted angioma (TA), as well as some of the more invasive types of vascularmalformations (i.e., microcystic lymphatic malformations and blue rubber bleb nevus syndrome (BRBNS). These therapies need to be combined with good supportive care, which often involves anticoagulation, antimicrobial prophylaxis, and comprehensive pain and symptom-relief strategies, as well as appropriate drug monitoring and management of side effects of medical treatment. The optimal care of these patients frequently involves close collaboration between surgeons, interventional and conventional radiologists, medical subspecialists, and nurses. [ABSTRACT FROM AUTHOR]

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]

Kaposiform hemangioendothelioma is a rare, potentially life-threatening vascular tumor often associated with a coagulopathy known as Kasabach-Merritt phenomenon (KMP). We report the case of an infant with satellite lesions around a large vascular tumor and associated KMP. [ABSTRACT FROM AUTHOR]

Background. Epistaxis is very common during childhood. It occurs primarily in boys and is usually self-limiting. Trauma and nose picking are among the most common causes. In general, epistaxis can be easily treated with anterior nasal packing or electrocoagulation. Methods. We report a case of an 8-year-old girl with severe unilateral epistaxis. Results. The bleeding originated from a kaposiform hemangioendothelioma arising in the left nasal cavity and ethmoid sinus. The feeding vessels originating from the maxillary artery were first embolized. The tumor was then surgically removed through a combined external ethmoidectomy and endonasal approach. The postoperative course was uneventful. MRI at 6 months after surgery showed no tumor recurrence. Conclusions. We report a previously undescribed cause of epistaxis in children, namely, a kaposiform hemangioendothelioma. To our knowledge, this is the first such case in the English-language literature. The differential diagnosis of severe unilateral nasal bleeding among the pediatric population should include the possibility of a kaposiform hemangioendothelioma. © 2006 Wiley Periodicals, Inc. Head Neck, 2006 [ABSTRACT FROM AUTHOR]

Sixty percent of vascular anomalies in children are found in the head and neck. These lesions can present throughout antenatal, perinatal and childhood development. They broadly fall into two categories: vascular tumours and vascular malformations. Their clinical and, often, psychological impact is determined by both pathological type and location: many lesions follow an uncomplicated natural course and other more complex, extensive or progressive lesions can present a threat to life from mass effect, haemorrhage or large volume arteriovenous shunting. Vascular tumours include infantile haemangioma (IH), congenital haemangioma (CH) and kaposiform hemangioendothelioma (KH); of which IH is the most common. Management options for vascular tumours include conservative approaches, oral medications and surgical intervention as determined by tumour type, location and associated complications. Vascular malformations can be categorised into low flow and high flow lesions. Low flow lesions include capillary, venous and lymphatic malformations (LMs). High flow lesions describe the arteriovenous malformations (AVMs), a highly heterogeneous group of lesions which can present in a variety of ways-the mainstay of treatment for these dynamic lesions is endovascular or surgical obliteration. We provide a practical framework for clinical classification of vascular anomalies of the head and neck in children. We also explore principles of their clinical and radiological assessment along with management, highlighting the importance of a multi-disciplinary approach.