Clinical analysis on 13 cases of Kaposiform hemangioendothelioma

• DOI: 10.3760/cma.j.issn.2095-428x.2017.11.012
• Publication Date: 2017-06-05
• Authors: Pengfei Gao; Man Shu; Juntao Xie; Qigen Xie; Wenzong Gao; Li Zhou
• Keywords: Histopathology

Objective To explore the diagnosis and treatment of Kaposiform hemangioendothelioma (KHE), deepen understanding KHE Kasabach-Merritt phenomenon (KMP), discuss optimal for KHE.

Methods From January 2008 to August 2016, 13 cases were confirmed by surgery or biopsy pathology admitted First Affiliated Hospital Sun Yat-Sen University, clinical analyzed retrospectively.

Results There patients(7 males 6 females) with a median age 1.0 years(0.2-10.0 years), 84.6%(11/13 cases) infants young children, 76.9% (10/13 involved deep tissue, 23.1% (3/13 associated KMP they younger than 1 year old, 15.4% (2/13 coexisted hemangioma lymphangioma.The location, extent infiltration depth lesion observed imaging examinations histopathology showed nodule shaped spindle tumor cells.Radical resection was considered if possible.Dose Vincristine (0.5 mg/m2 weekly) Propranolol [1 mg/(kg·d)] administered.The prognosis different in thirteen undergoing treatments.After 3 months 9 years follow-up, 41.7% (5/12 survived after treatment.

Conclusions KHE happens mostly varying manifestations high recurrence rate.The is based on histological examination, computed tomography magnetic resonance while it still need explicit pathological needed.KHE may be accompanied lymphangioma.Prognosis affected many factors comprehensive required.KMP should remedied preferentially, individual protocol long term follow-up are necessary.

Key words: Kaposiform hemangioendothelioma; Kasabach-Merritt phenomenon; Diagnosis; Treatment; Analysis