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Ultraschall Med
DOI: 10.1055/a-2421-6047
Original Article

Kaposiform haemangioendothelioma: ultrasonographic features and risk factors for the Kasabach–Merritt phenomenon

Kaposiformes Hämangioendotheliom: Ultraschallmerkmale und Risikofaktoren für das Kasabach-Merritt-Phänomen
Jing Zhao
1   Department of Ultrasounography, Zhengzhou University People’s Hospital, Henan provincial People's Hospital, Zhengzhou City, China
,
Jian-Jun Yuan
1   Department of Ultrasounography, Zhengzhou University People’s Hospital, Henan provincial People's Hospital, Zhengzhou City, China
,
Chang-Xian Dong
2   Department of Hemangioma and Vascular Malformation, Zhengzhou University People’s Hospital, Henan provincial People's Hospital, Zhengzhou City, China
,
Xiangqin Zhang
3   Department of Cardiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China (Ringgold ID: RIN191599)
,
Chuang Li
1   Department of Ultrasounography, Zhengzhou University People’s Hospital, Henan provincial People's Hospital, Zhengzhou City, China
,
Qi Sun
4   Department of Pathology, Zhengzhou University People’s Hospital, Henan provincial People's Hospital, Zhengzhou City, China
,
Gang Wu
1   Department of Ultrasounography, Zhengzhou University People’s Hospital, Henan provincial People's Hospital, Zhengzhou City, China
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Abstract

Purpose

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor with high morbidity and mortality. The aim of this study was to evaluate ultrasonographic findings associated with KHE.

Materials and Methods

The clinical and ultrasonographic findings of a cohort of 64 cases with pathologically proven KHE were retrospectively reviewed and analyzed between November 2014 and February 2021. Two subtypes were divided according to the presence or absence of the Kasabach-Merritt phenomenon (KMP). The KMP risk factors in patients with KHE were analyzed statistically.

Results

Among the 64 cases of KHE, 43 (67.2%) were accompanied by KMP. There was a positive correlation between the appearance of KMP and tumor size. KHEs had an increased risk of developing KMP if the lesions measured were >6 cm and if they belonged to the deep or mixed subtype. On ultrasonography, all KHE lesions were heterogeneous, and 81.3% were hypoechoic; 93.8% of KHEs exhibited ill-defined margins, 68.7% had strands branching into the adjacent tissue, and 84.4% presented marked hypervascularity. Elastography showed that central hypoechogenic lesion areas were hard, and surrounding hyperechogenic lesion areas were soft.

Conclusion

KHEs can occur in different parts of childrens’ bodies. On ultrasonography, the main findings are heterogeneous low erosions, indistinct margins, branching strangulation into adjacent tissues, and obvious hypervascularity. Patients with lesions larger than 6 cm or belonging to deep or mixed subtypes (musculoskeletal infiltrates) are at risk for developing KMP, and clinicians should be vigilant.

Zusammenfassung

Hintergrund

Das kaposiforme Hämangioendotheliom (KHE) ist ein seltener, lokal aggressiver Gefäßtumor mit hoher Morbidität und Mortalität. Ziel dieser Studie war es, die mit dem KHE verbundenen Ultraschallbefunde zu untersuchen.

Materialien und Methoden

Die klinischen und sonografischen Befunde einer Kohorte von 64 Fällen mit pathologisch bestätigtem KHE wurden zwischen November 2014 und Februar 2021 retrospektiv überprüft und analysiert. Zwei Subtypen wurden nach dem Vorhandensein oder Fehlen des Kasabach-Merritt-Phänomens (KMP) unterteilt. Die KMP-Risikofaktoren bei Patienten mit KHE wurden statistisch analysiert.

Ergebnisse

Von den 64 KHE-Fällen waren 43 (67,2%) von KMPs begleitet. Es bestand eine positive Korrelation zwischen dem Auftreten eines KMP und der Tumorgröße. KHEs hatten ein erhöhtes Risiko, KMP zu entwickeln, wenn die Läsionen >6 cm groß waren und wenn sie zum tiefen oder gemischten Subtyp gehörten. Bei der Ultraschalluntersuchung waren alle KHE-Läsionen heterogen und 81,3% echoarm. 93,8% der KHEs wiesen unscharfe Ränder auf, 68,7% hatten Stränge, die in das angrenzende Gewebe verzweigten, und 84,4 % zeigten eine ausgeprägte Hypervaskularität. Die Elastografie zeigte, dass die zentralen hypoechogenen Bereiche der Läsion hart und die umgebenden hyperechogenen Bereiche der Läsion weich waren.

Schlussfolgerung

KHEs können an verschiedenen Körperstellen von Kindern auftreten. Bei der Ultraschalluntersuchung sind die wichtigsten Befunde heterogene niedrige Erosionen, unscharfe Ränder, Verästelungen, die in angrenzende Gewebe einwachsen, und eine offensichtliche Hypervaskularität. Bei Patienten mit Läsionen, die größer als 6cm sind oder zu tiefen oder gemischten Subtypen (muskuloskelettale Infiltrate) gehören, besteht das Risiko, dass sie ein KMP entwickeln, und die Ärzte sollten besonders wachsam sein.

Keywords

kaposiform hemangioendothelioma - Kasabach-Merritt phenomenon - ultrasound - vascular tumor


Publication History

Received: 15 April 2024

Accepted after revision: 19 August 2024

Article published online:
15 October 2024

© 2024. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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