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06.10.2018 | Original Article – Clinical Oncology | Ausgabe 12/2018

Journal of Cancer Research and Clinical Oncology 12/2018

Kaposiform hemangioendothelioma without cutaneous involvement

Zeitschrift:
Journal of Cancer Research and Clinical Oncology > Ausgabe 12/2018
Autoren:
Yi Ji, Siyuan Chen, Lizhi Li, Kaiying Yang, Chunchao Xia, Li Li, Gang Yang, Feiteng Kong, Guoyan Lu, Xingtao Liu
Wichtige Hinweise

Electronic supplementary material

The online version of this article (https://​doi.​org/​10.​1007/​s00432-018-2759-5) contains supplementary material, which is available to authorized users.
Yi Ji, Siyuan Chen and Lizhi Li contributed equally to this work.

Abstract

Purpose

We sought to characterize the clinical features and management of patients diagnosed as Kaposiform hemangioendothelioma (KHE) without cutaneous involvement.

Methods

The electronic patient chats at six Triple A hospitals in China were searched to find all patient diagnoses with KHE without cutaneous involvement.

Results

Of 30 patients (mean age at diagnosis, 55.6 months), 17 (56.7%) were male. Fourteen (46.7%) patients were associated with Kasabach–Merritt phenomenon (KMP). Patients with KMP were significantly more likely to have lesions involving truck compared to patients without KMP (odds ratio 10.000; 95% confidence interval 1.641–60.921; P = 0.011). Other common complication included severe anemia and decreased range of motion. In the majority of cases (93.3%), the lesions were highly infiltrative and locally invasive with ill-defined margins. Histological examination was required in all patients without KMP for precise diagnosis. In all, 16 (53.3%) patients received corticosteroid treatment, 19 (63.3%) received oral sirolimus treatment, 7 (23.3%) received intravenous vincristine, and 5 (16.7%) patients used propranolol. Patients had varied responses to conventional drugs, whereas all patients receiving sirolimus treatment had better response. In all, three patients (10%) died of disease, all presented with KMP. Feature of these recalcitrant cases (death) included young age, visceral location, extensive involvement, and lack of improvement with high-dose corticosteroids.

Conclusions

Our study clearly demonstrated that KHE without cutaneous involvement could be associated with important complication, which might result in death or severe morbidity. Increased awareness of KHE without cutaneous involvement is required for early diagnosis and aggressive therapy in an attempt to prevent complication.

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Zusatzmaterial
Supplementary material 1 (DOCX 23 KB)
432_2018_2759_MOESM1_ESM.docx
Supplementary material 2 (DOCX 23 KB)
432_2018_2759_MOESM2_ESM.docx
Literatur
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