Clinical and imaging features of Kaposiform Hemangioendothelioma

Hu, Peian; Zhou, Zhengrong

doi:10.1259/bjr.20170798

Cited by 33 publications

(39 citation statements)

References 22 publications

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“…Deep KHE with KMP should be considered in patients presenting with an unexplained severe thrombocytopenia and coagulopathy, especially in patients coexisting with cutaneous purpura and severe anemia. MRI scan of the abdomen and chest should be recommended for such patients [84,85].…”

Section: Diagnosismentioning

confidence: 99%

Kaposiform hemangioendothelioma: current knowledge and future perspectives

Chen

Yang

et al. 2020

Orphanet J Rare Dis

120

180

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Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm with high morbidity and mortality. The initiating mechanism during the pathogenesis of KHE has yet to be discovered. The main pathological features of KHE are abnormal angiogenesis and lymphangiogenesis. KHEs are clinically heterogeneous and may develop into a lifethreatening thrombocytopenia and consumptive coagulopathy, known as the Kasabach-Merritt phenomenon (KMP). The heterogeneity and the highly frequent occurrence of disease-related comorbidities make the management of KHE challenging. Currently, there are no medications approved by the FDA for the treatment of KHE. Multiple treatment regimens have been used with varying success, and new clinical trials are in progress. In severe patients, multiple agents with variable adjuvant therapies are given in sequence or in combination. Recent studies have demonstrated a satisfactory efficacy of sirolimus, an inhibitor of mammalian target of rapamycin, in the treatment of KHE. Novel targeted treatments based on a better understanding of the pathogenesis of KHE are needed to maximize patient outcomes and quality of life. This review summarizes the epidemiology, etiology, pathophysiology, clinical features, diagnosis and treatments of KHE. Recent new concepts and future perspectives for KHE will also be discussed.

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Section: Diagnosismentioning

confidence: 99%

Kaposiform hemangioendothelioma: current knowledge and future perspectives

Chen

Yang

et al. 2020

Orphanet J Rare Dis

120

180

View full text Add to dashboard Cite

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“…Schmid et al insisted that meticulous surgical excision must be considered as the first line of treatment for KHE, while Ji et al proposed that sirolimus could be regarded as the first option [4,9]. KHE often manifests as an immovable enlarging mass [10]. Our patient's lesion was located deep inside, alongside the thoracic vertebral bodies.…”

Section: Discussionmentioning

confidence: 82%

Kaposiform Hemangioendothelioma in Adolescent-Onset Scoliosis: A Case Report and Review of Literature

Kim

et al. 2020

Case Reports in Orthopedics

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Background and Objectives. Kaposiform hemangioendothelioma (KHE) is a vascular tumor of very low incidence, which occurs mostly in children and infants. The tumor is recognized for its locally aggressive, yet rarely metastatic behavior. It may cause consumptive coagulopathy known as Kasabach-Merritt phenomenon. We report a distinctive case, where an 11-year-old boy is presented with progressive thoracolumbar scoliosis without any symptom or neurological sign. Case Report. The patient underwent spinal deformity correction via posterior pedicle screw instrumentation and fusion, along with tumor biopsy. The pathology report confirmed KHE. The patient did not show a prominent progression of scoliosis after the surgery without any further treatments. Conclusions. Many of scoliotic patients do not have any apparent cause, thereby regarded as idiopathic scoliosis. The presented case is where kaposiform hemangioendothelioma is likely to be linked to the patient’s scoliosis. We demonstrate the possibility of secondary scoliosis should always be kept in mind of orthopaedic doctors. We also conclude that secondary scoliosis does not show exacerbation after growth completion.

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“…Regarding MRI findings in TA and KHE, previous studies have reported that the imaging characteristics of TA and KHE were similar to other vasoproliferative neoplasms, although KHE tended to be larger, more ill‐defined and infiltrative . Several case reports and series have described imaging findings associated with KHE exhibiting ill‐defined margins, multiplanar involvement, diffuse enhancement, isointensity relative to muscle on T 1 ‐weighted imaging, hyperintensity on T 2 ‐weighted imaging and destruction/remodeling of adjacent bone. In our study, all lesions presented as irregular soft tissue masses in subcutaneous regions, with iso‐ or hypointensity to muscle on T 1 ‐weighted sequences and hyperintense T 2 signal.…”

Section: Discussionmentioning

confidence: 95%

Ultrasonography and magnetic resonance imaging features of kaposiform hemangioendothelioma and tufted angioma

Gong

Ying

Zhang

et al. 2019

The Journal of Dermatology

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Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) primarily occurring in infants are difficult to distinguish. This study evaluated ultrasonography (US) and magnetic resonance imaging (MRI) features of KHE and TA. Pathologically proven TA (n = 21) and KHE (n = 40 [11 KHE + Kasabach–Merritt phenomenon [KMP]]) occurring between January 2015 and December 2017 were reviewed. US (n = 61) and MRI (n = 50) findings were retrospectively evaluated. On US, KHE and TA lesions were subcutaneous, while 40% of KHE exhibited an infiltrative pattern extending into adjacent muscles. Of TA lesions, 42.9% were hyperechoic and 96.15% of KHE lesions exhibited mixed echogenicity. Of TA lesions, 76.2% exhibited well‐defined margins and all KHE lesions exhibited ill‐defined margins. The depth and vascular density of KHE and KHE + KMP were significantly increased compared with TA. The arterial peak systolic blood flow velocity of KHE + KMP was significantly higher than that in TA and KHE. KHE and KHE + KMP were significantly harder than TA on elastography. 3‐D color Doppler revealed branch‐shape blood flow for KHE and KHE + KMP lesions; for TA, it revealed a dot‐like and striped pattern. Considering MRI findings, KHE and KHE + KMP were more likely to exhibit diffuse heterogeneous enhancement after contrast than TA. KHE was infiltrative and more likely to be thick, hypoechoic, ill‐defined, richly vascular and hard than TA on US. KHE lesions were subcutaneous and reticular, with heterogeneous enhancement on MRI. Awareness of these features should prompt radiologists in the differential diagnosis of pediatric masses.

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Clinical and imaging features of Kaposiform Hemangioendothelioma

Cited by 33 publications

References 22 publications

Kaposiform hemangioendothelioma: current knowledge and future perspectives

Kaposiform hemangioendothelioma: current knowledge and future perspectives

Kaposiform Hemangioendothelioma in Adolescent-Onset Scoliosis: A Case Report and Review of Literature

Ultrasonography and magnetic resonance imaging features of kaposiform hemangioendothelioma and tufted angioma

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