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Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options

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Abstract

Background

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor affecting infants and young children. Although benign, it can be associated with an aggressive locally growing tumor and/or a life-threatening Kasabach–Merritt phenomenon (KMP). To date, only reviews of limited cases have been performed. We, therefore, conducted a comprehensive literature search to collect relevant data and make recommendations for future treatment trials.

Methods

Review of the available literature between 1993 and 2017 revealed a total of 105 publications involving 215 patients of less than 21 years of age. To this, we added 12 from our department and 4 from the Cooperative Weichteilsarkomstudie database.

Results

We found that KMP was present in 79% of the infants, in 47% of the 1–5-year olds, in 43% of the 6–12-year olds, and in 10% of the 13–21-year-old patients. KMP was present in nearly all (94%) patients with retroperitoneal tumors and in all patients with extra-regional tumors. The median size of a KHE without KMP was 12 cm2 as compared to 49 cm2 when associated with a KMP. With complete (not further classifiable if R0 or R1) resection, all patients were cured. If inoperable, response regarding KMP/regression of tumor size was seen in 29/28% with steroid-, 47/39% with vincristine-, 44/43% with interferon alpha-, 65/61% with anti-platelet agents-, and in 97/100% with sirolimus-containing therapies.

Conclusions

Patients with progressive KHE should undergo resection whenever it is considered a safe option. If inoperable, sirolimus should be the first choice for treating KMP and reducing tumor size.

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Authors and Affiliations

  1. Division of Pediatric Hematology and Oncology, Dr. von Hauner Children’s Hospital, Ludwig-Maximilians University of Munich, LMU, Lindwurmstr. 4, 80337, Munich, Germany

    Irene Schmid & Anne K. Klenk

  2. Klinikum Stuttgart-Olgahospital, Stuttgart Cancer Center, Zentrum für Kinder-, Jugend- und Frauenmedizin, Pediatrics 5 (Oncology, Hematology, Immunology), Stuttgart, Germany

    Monika Sparber-Sauer & Ewa Koscielniak

  3. Department of Pediatric Hematology and Oncology, Children’s Hospital, Tübingen, Germany

    Ewa Koscielniak

  4. Pediatric Surgery, Dr. von Hauner Childen’s Hospital, Ludwig-Maximilians University of Munich, Munich, Germany

    Rebecca Maxwell & Beate Häberle

Authors
  1. Irene Schmid
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  2. Anne K. Klenk
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  4. Ewa Koscielniak
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Contributions

IS wrote the manuscript, collected patient data and contributed to the analyses and interpretation of the data. AKK wrote the manuscript, collected patient data and contributed to the analyses and interpretation of the data. MSS collected patient data and contributed to the analyses and interpretation of the data. EK collected patient data and contributed to the analyses and interpretation of the data. RM wrote the manuscript, collected patient data and contributed to the analyses and interpretation of the data. BH wrote the manuscript, collected patient data and contributed to the analyses and interpretation of the data. All authors reviewed and approved the final manuscript.

Corresponding author

Correspondence to Irene Schmid.

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Not necessary since the article is a literature review and retrospective analysis.

Conflict of interest

None of the authors have any competing interests to disclose.

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Schmid, I., Klenk, A.K., Sparber-Sauer, M. et al. Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options. World J Pediatr 14, 322–329 (2018). https://doi.org/10.1007/s12519-018-0171-5

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  • DOI: https://doi.org/10.1007/s12519-018-0171-5

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