Kaposiform Hemangioendothelioma

Abstract Background Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumor of intermediate malignant potential which shows locally aggressive growth but only rarely metastasizes. It is mostly considered to be a tumor of pediatric population but its occurrence in the adults is not uncommon as once considered. Histologically, KHE can mimic other soft tissue neoplasms of different behaviors (e.g. Kaposi Sarcoma, hemangioma) and establishing the correct diagnosis is important for appropriate treatment. Herein, we describe the clinicopathological features of 8 cases of KHE which will be helpful in making their diagnosis. Methods We reviewed pathology reports, microscopy glass slides and obtained follow up information about 8 cases of KHE which were diagnosed at our institution from January 2008 till June 2020. Immunohistochemical stain for HHV8 was also performed. Results Age ranged from 7 months to 25 years. Seven patients were less than 20 years of age and one patient was 25 years old. Equal gender distribution was observed. Extremities were the most common sites of involvement, followed by head and neck, pancreas and ischiorectal region. 2 cases were resection specimen and all others were incisional biopsies. The largest tumor size was 5.5 cm in one of the resections. The incisional/fragmented tissues were all less than 5 cm in aggregate. Most cases showed predominance of nodular growth and a minor component of spindle cell population along with lymphangiomatosis like vascular channels, with evidence of microthrombi in 2 cases. Few multinucleated giant cells were observed in 2 cases. None of the cases exhibited significant nuclear atypia or mitotic activity. One of the cases arising in dermis showed underlying bone involvement. HHV8 was negative in 7/7 cases. Conclusions KHE can also involve adult population and it should always be considered in the differential diagnoses of a vascular lesion. Presence of multinucleated giant cells is a rare finding. Knowledge about histological features and potential mimics is helpful in avoiding misdiagnosis.

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Kaposiform Hemangioendothelioma in a 3 Months Old Infant

Indian Journal of Hematology and Blood Transfusion ◽

10.1007/s12288-016-0674-y ◽

2016 ◽

Vol 32 (S1) ◽

pp. 370-371 ◽

Cited By ~ 1

Author(s):

Ramya Uppuluri ◽

Vimal Kumar ◽

Deenadayalan Munirathnam ◽

Hemalatha Doss ◽

Sreejith Ramachandrakurup ◽

...

Keyword(s):

Kaposiform Hemangioendothelioma

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Kaposiform Hemangioendothelioma and Kasabach-Merritt Phenomenon: Management of Coagulopathy and Treatment Options

Vascular Anomalies ◽

10.1007/978-3-030-25624-1_6 ◽

2020 ◽

pp. 63-88

Author(s):

Taizo A. Nakano ◽

Ilona J. Frieden

Keyword(s):

Treatment Options ◽

Kaposiform Hemangioendothelioma

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Comparison of Corticosteroid and Vincristine in Treating Kaposiform Hemangioendothelioma and Tufted Angioma: A Systematic Review and Meta-Analysis

European Journal of Pediatric Surgery ◽

10.1055/s-0038-1673708 ◽

2018 ◽

Vol 29 (05) ◽

pp. 401-407 ◽

Cited By ~ 5

Author(s):

Wei Yao ◽

Kai Li ◽

Zuopeng Wang ◽

Jiahao Pei ◽

Xiangqi Liu ◽

...

Keyword(s):

Recurrence Rate ◽

Adverse Reactions ◽

Meta Analysis ◽

Case Series ◽

Research Quality ◽

Cochrane Library ◽

Kaposiform Hemangioendothelioma ◽

Systemic Corticosteroids ◽

Tufted Angioma

Objective To evaluate the outcome and safety of corticosteroids and vincristine (VCR) in the treatment of kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). Materials and Methods Clinical studies involving corticosteroids and VCR therapies in treating KHE/TA were identified by using PubMed, Cochrane Library, OVID, EBSCO, CNKI, VIP, and Wanfang databases from their establishment date to December 2017. Randomized controlled trials, case–control, or case series with more than five cases were included. The following data were extracted: study sample, demographics, responses rate, recurrence rate, and adverse reactions. Two reviewers completed screening and extraction. Methodological quality was evaluated with quality appraisal tool. Results A total of 266 studies were found, and 27 studies were finally included in this research; quality of all studies was low. Seven studies with a total of 123 participants, which compared the effect of systemic corticosteroids with that of VCR, were performed for the meta-analysis. The results indicated that the effect of VCR was significantly higher than that of corticosteroids (relative risk [RR] = 2.08, 95% confidence interval [CI]: 1.38–3.16). The recurrence rate of VCR (11.1%) was lower than that of corticosteroids (50%), but there was no statistical difference between the two therapies (p = 0.1312). The result of pooled adverse reactions response rate for VCR was 18.2%, significantly lower than that for corticosteroids, which was 52.0%. Conclusion The present profile shows that VCR is relatively more effective and safer in treating KHE/TA than corticosteroids are. So, we believe VCR could be used as a first-line medication agent in the treatment of KHE/TA.

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