Journal of Cancer Research and Clinical Oncology

, Volume 144, Issue 12, pp 2475–2484 | Cite as

Kaposiform hemangioendothelioma without cutaneous involvement

  • Yi JiEmail author
  • Siyuan ChenEmail author
  • Lizhi Li
  • Kaiying Yang
  • Chunchao Xia
  • Li Li
  • Gang Yang
  • Feiteng Kong
  • Guoyan Lu
  • Xingtao Liu
Original Article – Clinical Oncology



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


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


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.


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.


Kaposiform hemangioendothelioma Cutaneous involvement Kasabach–Merritt phenomenon Morbidity Mortality 



This work was supported by Grants from the National Natural Science Foundation of China (Grant nos: 81401606 and 81400862) and the Science Foundation for Excellent Youth Scholars of Sichuan University (Grant no: 2015SU04A15).

Compliance with ethical standards

Conflict of interest

Author Yi Ji declares that they have no conflict of interest. Author Siyuan Chen declares that they have no conflict of interest. Author Lizhi Li declare that they have no conflict of interest. Author Kaiying Yang declares that they have no conflict of interest. Author Chunchao Xia declares that they have no conflict of interest. Author Li Li declares that they have no conflict of interest. Author Gang Yang declares that they have no conflict of interest. Author Feiteng Kong declares that they have no conflict of interest. Author Guoyan Lu declares that they have no conflict of interest. Author Xingtao Liu declares that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study. Written informed consent for publication of this study was obtained from all patients and patient’s parents.

Supplementary material

432_2018_2759_MOESM1_ESM.docx (24 kb)
Supplementary material 1 (DOCX 23 KB)
432_2018_2759_MOESM2_ESM.docx (24 kb)
Supplementary material 2 (DOCX 23 KB)


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Division of Oncology, Department of Pediatric SurgeryWest China Hospital of Sichuan UniversityChengduChina
  2. 2.Pediatric Intensive Care Unit, Department of Critical Care MedicineWest China Hospital of Sichuan UniversityChengduChina
  3. 3.Department of Pediatric SurgeryFujian Provincial HospitalFuzhouChina
  4. 4.Department of RadiologyWest China Hospital of Sichuan UniversityChengduChina
  5. 5.Laboratory of PathologyWest China Hospital of Sichuan UniversityChengduChina
  6. 6.Department of Pediatric SurgeryChengdu Shangjin Nanfu HospitalChengduChina
  7. 7.Department of Pediatric SurgerySichuan Women and Children’s HospitalChengduChina
  8. 8.Pediatric Intensive Care UnitWest China Second University Hospital, Sichuan UniversityChengduChina
  9. 9.Department of Vascular and Interventional RadiologyChengdu Women and Children’s Central HospitalChengduChina

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