Primary intracranial epithelioid hemangioendothelioma: a low-proliferation tumor exhibiting clinically malignant behavior
Journal of Neuro-Oncology, 08/21/2012
Zheng J et al. – Intracranial epithelioid hemangioendothelioma is a rare, low-proliferation tumor, but it exhibits some clinically malignant behaviors, such as local invasion, recurrence and metastasis. Total resection is mandatory where possible, and radiotherapy and/or chemotherapy are otherwise required. Preoperative feeding-artery embolization is recommended.
Methods- We described two new cases of primary intracranial epithelioid hemangioendothelioma and performed a search of MEDLINE (PubMed) using the words “epithelioid hemangioendothelioma”.
- The tumor in case 1 originated from the right temporal bone and invaded the surrounding cranium, dura and temple muscles.
- The tumor in case 2 was located in the petroclival bone and had also invaded the surrounding cranium.
- Both tumors were well vascularized.
- The tumors were totally (case 1) or subtotally (case 2) removed with moderate blood loss.
- A total of 36 cases of intracranial epithelioid hemangioendothelioma were found in the literature.
- The tumor was typically diagnosed in young adults and infants.
- There was no sex predominance in adult patients, while in children, males were more frequently affected (M/F ratio, 3.5:1).
- Surgical removal was the main therapeutic protocol, and adjuvant therapy included radiotherapy or chemotherapy.
- Including the 2 patients presented here, a total of 38 patients were analyzed: 32 % showed local invasion, 24 % suffered a recurrence, 15 % had metastases, and the mortality rate was 15 %.
