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Competing Interests: Conflicts of interest None disclosed.

Purpose: The management of hepatic hemangioendothelioma (HHE) may be challenging. We aimed to review a large cohort of children who presented to our centers with symptomatic HHE in the last 16 years., Methods: We collected age at presentation, clinical features, histology, diagnostic process, management and outcome., Results: Twenty seven patients (male/female 5/22), median age 13 days (1-1530) presented with hepatomegaly (24/27), cardiac failure (10/27), cutaneous hemangiomas (8/27), fever and anemia (6/27 each), vomiting (5/27), splenomegaly (4/27). The lesion was focal, multifocal, or diffuse in 9 patients of each group. The management included medical treatment (8/27), embolization (8/27), resection (3/27), observation (6/27), transplantation (2/27). After 16 months' follow-up (30 days-11 years), 23/27 (85%) were alive. Diffuse lesions (4/4), cardiac failure (4/4), type II histology (4/4), age older than 6 months at diagnosis (3/4) predicted mortality (all p < 0.01). Histology showed type 1 lesion in 3/8, type 2 in 3/8, and type 3 in 2/8 with foci of angiosarcoma., Conclusion: Most patients with symptomatic HHE can be managed successfully with a combination of medical, radiological and surgical treatments. Patients with diffuse lesions, late presentation, cardiac failure and type II histology have a poor outcome., Level of Evidence: Diagnostic level IV. Therapeutic level IV.

This meta-analysis was to evaluate the efficacy of current treatment modalities for kaposiform hemangioendothelioma and tufted angioma. A systematic review was performed using PubMed (Medline), Web of Science and Embase for clinical studies. The outcome was measured by pooled response rate with 95% confidence intervals (CIs), together with heterogeneity, subgroup analysis, sensitivity analysis and publication bias. Fifteen studies with 244 participants were included in this analysis. Vincristine therapy exhibited a relatively higher response rate (0.72; 95%CI, 0.64-0.79) compared with other therapies including systemic corticosteroid (0.27; 95%CI, 0.17-0.36), interferon (0.36; 95%CI, 0.24-0.48), radiotherapy (0.49; 95%CI, 0.26-0.73), embolization (0.66; 95%CI, 0.48-0.83), aspirin/ticlopidine (0.42; 95%CI, 0.06-0.78) and sirolimus (0.57; 95%CI, 0.00-0.10), in treating KHE/TA. Subgroup analysis indicated that the efficacy of systemic corticosteroids therapy was age-related. The pooled response rate was 0.15 (95%CI, 0.08-0.23) for participants 3.5 months of age and older compared with 0.35 (95% CI, 0.26-0.44) for participants less than 3.5 months. Regarding side effects, systemic corticosteroids treatment was 0.32 (95%CI, 0.15-0.50), vincristine modality was 0.16 (95%CI, 0.08-0.24) and interferon therapy was 0.28 (95%CI, 0.13-0.43). In conclusion, as one of the first reviews evaluating the effect of common therapies in the treatment of KHE/TA, our meta-analysis displayed that vincristine was more effective. Thus, vincristine was the most effective, providing evidence supporting the use of vincristine as a first-line therapy for KHE/TA., (© 2016 UICC.)

Kasposiform hemoangioendothelioma (KHE) and tufted angioma (TA) are classifed as vascular tumors with locally aggressive and benign growth potential, respectively, within the classification schema proposed by the International Society for the Study of Vascular Anomalies. A unique feature of these vascular tumors is the risk of Kasabach-Merritt phenomenon (KMP), a severe thrombocytopenia with mild to moderate coagulopathy resulting from intralesional platelet trapping. As with many vascular anomalies, accurate description of clinical course, responses to therapy, and long-term outcomes have been hindered by lesion misidentification, imprecise nomenclature, and lack of prospective, randomized clinical trials to assess therapeutic efficacy. The classic dermatologic features of these lesions can facilitate diagnosis for the astute provider; however, the absence of or unusual integumentary involvement or presentation in a less common age group (adolescents/adults) poses a diagnostic challenge. Current approaches to the management of KHE/TA are often informed by lesion features such as presence of KMP, extent and location of the tumor, and symptomatology. Evidence-based treatment guidelines are limited. Corticosteroids, vincristine, interferon, multi-agent regimens and newer therapies, such as sirolimus, have demonstrated efficacy in patient series. The use of surgical excision and interventional radiology guided therapies have been described with mixed clinical benefit. Collaboration among emerging vascular anomaly centers and an increasing number of providers across subspecialties with interest in this field are facilitating the development of standardized approaches to diagnosis and management. The rarity of KHE-spectrum lesions and the heterogeneity of clinical manifestations necessitate rationally designed, multisite clinical trials to investigate risk stratification schemas and formally evaluate the short and long-term efficacy of available and novel therapies., (©2016 Frontline Medical Communications.)

Background: Retiform hemangioendothelioma (RH) is a locally aggressive vascular tumor. Wide surgical excision with tumor-free margins is the standard treatment strategy. However, surgical excision is not an option for lesions near critical anatomical structures, such as those on the face. We report the case of a patient with retiform hemangioendothelioma that responded to non-surgical treatments., Methods: We describe the case of a 72-year-old male patient with a retiform hemangioendothelioma on the face that responded to a combination of pulsed dye laser, local corticosteroid injection, and application of imiquimod cream. In addition, we reviewed past case reports of patients with retiform hemangioendothelioma., Results: Thirty-five cases that describe the management of RH, including the present case, have been reported. Review of the cases indicate a relatively low disease-free survival rate in cases that underwent surgical excision as the initial therapy for retiform hemangioendothelioma., Conclusion: Surgical excision might not be the best therapeutic option. Local control with conservative therapy is an acceptable strategy for lesions in anatomical regions including the face., (© 2015 The International Society of Dermatology.)

Neonatal vascular tumours present particular difficulties in their diagnosis and treatment. The special behaviour of neonatal hemangioma and the aggressive but benign nature of kaposiform hemangioendothelioma provide examples of a modern need for a conservative approach. Recent advances in their pharmacological management have dramatically changed the therapeutical scope of this group of tumors with surgery playing a minor role in their treatment. Multidisciplinary teams management involving neonatology, pediatric surgery, dermatology, pathology, genetics and radiology specialists among others remains the best option for an optimal outcome.

: Pseudomyogenic hemangioendothelioma is a recently described vascular tumor that occurs predominantly in the distal extremities of young adults. Because of multifocal presentation, epithelioid morphology, and strong cytokeratin expression, the tumor was frequently misdiagnosed as epithelioid sarcoma. However, substantial immunohistochemical studies revealed an endothelial nature. It has been currently considered a tumor of intermediate malignancy with frequent local recurrence but low risk of distant metastasis. In this report, we describe a case of pseudomyogenic hemangioendothelioma occurring in a 22-year-old man who presented with multifocal disease in the lower extremity and developed bilateral pulmonary metastases within a short period.

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular neoplasm that mainly occurs during childhood. It generally originates on the skin, usually affecting deeper tissue by infiltrative growth. It appears as one or multiple masses, and in most cases is associated to consumptive coagulopathy (Kasabach-Merritt syndrome), and lymphangiomatosis. Although visceral involvement is very uncommon, several cases with bone, retroperitoneal, or mediastinal involvement have been described. These tumors tend to be locally invasive, but are not known to produce distant metastases. The development of KHE in adolescents or in adults is very rare, but cases have also been described. Several factors are associated with the outcome of patients with KHE: accessibility to surgical excision, location (cutaneous versus visceral), size of tumoral mass, clinical response to interferon and glucocorticoids, and the absence of lymphangiomatosis and Kasabach-Merritt syndrome, may result in partial remissions. On the other hand, bulk visceral masses lead to a 40-50% mortality rate, mainly due to progressive failure of the infiltrated organ(s), in spite of interferon, glucocorticoids, and combined chemotherapy. In conclusion, the onset of a consumptive coagulopathy following the presence of a vascular tumor, in children as well as in older patients, should spark suspicion of KHE, among other entities.

Objectives: To discuss the current knowledge regarding complicated hemangiomas (cervicofacial, periorbital, lumbosacral, and parotid), including the associated syndromes of diffuse neonatal hemangiomatosis and PHACES (posterior fossa malformations, most commonly of the Dandy-Walker variant; hemangiomas [especially large, plaquelike, facial lesions]; arterial anomalies; cardiac anomalies and coarctation of the aorta; eye abnormalities; and sternal cleft and/or supraumbilical raphe). To discuss 2 newly recognized entities that may be a source of diagnostic confusion with the common hemangioma, the kaposiform hemangioendothelioma and tufted angioma. To discuss the risks and benefits of current treatment options, including the use of systemic corticosteroids and interferon in necessary situations., Data Sources: The pertinent world literature was reviewed and incorporated into experience from our pediatric dermatology practice at the University of Texas Medical School at Houston., Conclusions: The common hemangioma, kaposiform hemangioendothelioma, and tufted angioma, though benign histologically, may cause serious consequences for children. Dermatologists should be able to recognize unique clinical presentations of these lesions and obtain further diagnostic evaluation accordingly. Dermatologists should also be aware of available treatment options, including the use of systemic chemotherapy in life-threatening situations.

Background: Kaposiform hemangioendothelioma is a rare, aggressive vascular proliferation in children that is clinically and histologically distinct from hemangioma of infancy. It often manifests later than infantile hemangioma, and complication by Kasabach-Merritt syndrome is common., Observations: We examined 3 children with kaposiform hemangioendothelioma, all of whom were initially misdiagnosed as having infantile hemangioma. All 3 children developed Kasabach-Merritt syndrome, in association with a rapidly growing cutaneous vascular mass. Treatment with systemic corticosteroids, interferon alfa, vincristine, and radiation therapy appeared to halt progression of the disease. None had evidence of human herpesvirus 8 infection., Conclusions: Cutaneous kaposiform hemangioendothelioma may appear in early infancy but often appears months to years later. It is frequently complicated by Kasabach-Merritt syndrome, has no known association with Kaposi sarcoma related to human immunodeficiency virus infection, and demonstrates aggressive local behavior with invasion but not distant metastasis. Awareness of this entity is important to prevent a mistaken diagnosis of common hemangioma of infancy.

This report describes a neonate with dermal hematopoiesis associated with diffuse hemangiomatosis. The cutaneous lesions consisted of multiple red papules and bluish subcutaneous nodules scattered over his body. The nodules were bluish due to the presence of hematopoietic tissue within the hemangiomas. Although neonatal dermal hematopoiesis has been described with viral infections or hematologic dyscrasias, the association with diffuse hemangiomatosis has not been previously described.

Nine patients with cutaneous malignant hemangioendothelioma (CMHE) were reported in Okinawa. All the patients were elderly, between 75 and 93 years of age. Four patients were males and five were female. The onset of the disease ranged from 1 to 9 months before the first visit. Eight patients had lesions on the scalp, and one, on the face and cheek. The lesions were in the form of exudative erythematous purpura, erythematous purpuric ulcers, and tumors. One patient developed a systematic metastasis involving the lungs, heart and intestine, and two patients had local metastasis to the cervical lymph nodes. Histopathologically, the tumor vessels were proliferated irregularly and showed anastomosis. The lumens were lined by large and atypical endothelial cells. Most of the specimens were infiltrated with large numbers of red blood cells. By electron microscope, Weibel-palade bodies were found inside the tumor cells located at the peripheral part of the lesion. The patients were treated by irradiation, IL-2 injection, and/or surgery. They were treated for 3 months to 2 years. Eight patients died between 4 to 24 months after the onset of disease and one has survived. The prognosis was poor.

A case of malignant haemangioendothelioma of the scalp in an 82-year-old female treated with intralesional, intra-arterial and intravenous recombinant interleukin-2 (rIL-2) is reported. The scalp lesions disappeared after injection of 35,350,000 Japan reference units of rIL-2 and excision of the residual tumour. Immunohistochemical characterization of the cells infiltrating the lesion after rIL-2 administration revealed 30-40% CD4+ and CD8+ T lymphocytes, 20-30% macrophages and 5-10% natural killer cells. These findings are similar to those in other human cancers. Almost all of the cells in the intersticies of the lesion after rIL-2 injection expressed leucocyte function-associated antigen-1, and some of the tumour cells and the infiltrating cells around them expressed intercellular adhesion molecule-1. HLA-DR-positive cells markedly increased in number after rIL-2 administration. This report suggests that rIL-2 administration is the most effective therapy for malignant haemangioendothelioma.

Malignant angioendotheliomatosis is an angiotropic lymphoma that may present with clinical findings suggestive of thrombophlebitis or vasculitis. Examination of a biopsy specimen with special stains for leukocyte common antigen establishes the diagnosis. Although malignant angioendotheliomatosis is usually fatal, early treatment with chemotherapeutic agents is helpful. We review the characteristics and treatment of this uncommon disorder and present an illustrative case.

A case of Malignant Hemangioendothelioma (MHE) effectively treated with intra-arterial continuous infusion of recombinant interleukin-2 (rIL-2) was experienced. The Pt, a 82-year-old women, presented herself in our hospital with a complaint of the tumor in right-frontal region. Based on clinical and pathological findings, the Pt, was diagnosed as MHE. Increase of LAK (Lymphokine activated killer cell) activity was observed during treatment with intra-arterial continuous infusion of rIL-2. In addition, decrease of tumor was started when LAK activity showed high value. We mainly discussed about treatments for MHE and mechanism of these therapies by use of data of this case and other autho's papers.

A malignant hemangioendothelioma (MHE) developed on the frontal scalp of an 83-year-old woman was treated with intralesional injection of recombinant interleukin-2 (rIL-2, TGP-3, Takeda Chemical Industries LTD, Osaka) which had lead the lesion to complete remission for 2 years and 9 months. After the period of the complete remission, the patient developed MHE lesion on the occipital scalp which rapidly enlarged to more than 10 cm in diameter within 7 days, though there was no recurrence of MHE on the frontal scalp at that time. Intra-arterial rIL-2 injection was conducted through the occipital artery which circulates around the tumor lesion, but resulted unsuccessful because of the obstruction of the artery. Continuous intra-arterial rIL-2 injection through bilateral superficial temporal arteries was conducted, and then the tumor lesion came to complete remission on both clinical and histological data. So far, the patient has been showing no local recurrence or metastasis for the period of 8 months. We conclude that continuous intra-arterial rIL-2 injection immunotherapy through superficial temporal arteries is the best way of all the treatment in cases with recurrent MHE located on occipital scalp.

A case of epithelioid hemangioendothelioma presenting as violaceous nodules on the skin of the thigh is described. On histologic examination the tumor exhibited a proliferation of vascular channels lined by plump cuboidal and atypical endothelial cells with an epithelioid appearance. Lesions of epithelioid hemangioendothelioma were subsequently discovered in the adjacent femur. These tumors were successfully treated with chemotherapy and irradiation followed by a limb salvage procedure. To our knowledge, cutaneous presentation of this rare tumor has not been reported previously.