Infantile Kaposiform hemangioendothelioma in a female patient complicated with severe obstructed jaundice: a case report

Surgical Case Reports

Case Report

Takanobu Maekawa¹  Noriyuki Nakano²  Takako Yoshioka²  Osamu Miyazaki³  Akihiro Fujino⁴  Michimasa Fujiogi⁴  Naoki Hashizume⁴  Eiichiro Watanabe⁴  Takeshi Saito⁴  Genta Ozeki⁴  Yutaka Kanamori⁴  Motohiro Kano⁴  Yuki Yamamoto⁴  Akihiro Yoneda⁵  Mureo Kasahara⁶ 

[1] Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development, Setagaya-Ku, Tokyo, Japan;Department of Pathology, National Center for Child Health and Development, Setagaya-Ku, Tokyo, Japan;Department of Radiology, National Center for Child Health and Development, Setagaya-Ku, Tokyo, Japan;Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, Setagaya-Ku, Tokyo, Japan;Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, Setagaya-Ku, Tokyo, Japan;Division of Pediatric Surgical Oncology, National Center for Child Health and Development, Setagaya-Ku, Tokyo, Japan;Organ Transplantation Center, National Center for Child Health and Development, Setagaya-Ku, Tokyo, Japan;

关键词: Kaposiform hemangioendothelioma;    Hepatoduodenal ligament;    Sirolimus;   

DOI  :  10.1186/s40792-022-01581-9

 received in 2022-11-30, accepted in 2022-12-20,  发布年份 2022

来源: Springer

【 摘 要 】

BackgroundKaposiform hemangioendothelioma (KHE) is a rare locally aggressive vascular neoplasm that occurs mainly in the pediatric population. KHE usually originates just underneath the skin and affects deeper tissues through infiltrative growth; however, visceral tissue involvement is quite rare.Case presentationAn 8-month-old girl with jaundice and acholic stool was referred to our hospital for further evaluation of a hepatoduodenal ligament tumor. A blood examination revealed high bilirubin and liver enzyme levels, but no signs of coagulopathy. The first attempt at a diagnostic surgical procedure did not provide sufficient diagnostic information. However, the histopathological diagnosis of the cystic duct excised in the second surgery indicated KHE. Therefore, in our case, KHE was considered a cause of obstructive jaundice. Sirolimus (rapamycin) was initiated, and the patient was discharged 7 months after admission.ConclusionsIn cases of atypical hypervascular lesions in the abdominal cavity, especially in the pediatric population, it is important to consider the possibility of KHE, and surgical intervention with proper strategies is required for diagnosis, followed sequentially by promising treatments.

【 授权许可】

CC BY   
© The Author(s) 2022

【 图 表 】

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