Ann Surg Treat Res.  2021 Mar;100(3):137-143. 10.4174/astr.2021.100.3.137.

Post-resection prognosis of patients with hepatic epithelioid hemangioendothelioma

Affiliations
  • 1Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
  • 2Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea

Abstract

Purpose
Epithelioid hemangioendothelioma (EHE) is a rare borderline vascular tumor. This retrospective, single-center study evaluated the outcomes of hepatic resection (HR) in patients with hepatic EHE.
Methods
Over the 10-year period from 2009 to 2018, 11 patients with hepatic EHE underwent HR, accounting for 0.1% of the 11,979 adults who underwent HR at our center. Diagnosis of hepatic EHE was confirmed by immunohistochemical staining for CD34, CD31, and factor VIII-related antigen.
Results
The 11 patients included 9 females (81.8%) and 2 males (18.2%) with mean age of 43.5 ± 13.6 years. Preoperative imaging resulted in a preliminary diagnosis of suspected liver metastasis or EHE, with 9 patients (81.8%) undergoing liver biopsy. No patient presented with abnormally elevated concentrations of liver tumor markers. The extents of HR were determined by tumor size and location from trisectionectomy to partial hepatectomy. All patients recovered uneventfully from HR. Five patients showed tumor recurrence, with 4 receiving locoregional treatments for recurrent lesions. The 1-, 3-and 5-year disease-free survival rates were 90.9%, 54.5%, and 54.5%, respectively. Currently, all patients remain alive and are doing well. Univariate analysis on tumor recurrence showed that tumor size ≥ 4 cm was significantly associated with tumor recurrence (P = 0.032), but tumor number ≥ 4 was not related to (P = 0.24).
Conclusion
Hepatic EHE is a rare form of primary liver tumor often misdiagnosed as a metastatic tumor. Because of its malignant potential, HR is indicated if possible. HR plus, when necessary, treatment of recurrence yields favorable overall survival rates in patients with hepatic EHE.

Keyword

CD31 antigen; CD34 antigen; Hemangioendothelioma; Hemangioma; Vascular tissue neoplasms

Figure

  • Fig. 1 Preoperative computed tomography findings and gross photographs of the surgical specimens obtained after initial hepatectomy. Numbers denote the patient number. The specimen from patient 5 was obtained during the second hepatectomy for recurrent tumor.

  • Fig. 2 Microscopic findings of percutaneous needle liver biopsy taken from patient 4. (A) Needle liver biopsy specimen is visible (no stain, ×10). (B) Tumor cells show immunopositivity for CD34 (×400).

  • Fig. 3 Microscopic findings of hepatic epithelioid hemangioendothelioma obtained from patient 5, showing (A) few cellular epithelioid or spindle tumor cells on a background of fibromyxoid stroma (H&E staining, ×100), (B) epithelioid tumor cells containing vacuolated cytoplasm (H&E staining, ×400), (C) thin-walled vessels containing clusters of epithelioid tumor cells (arrows; H&E staining, ×400), and (D) tumor cells diffusely positive for CD31 (×400).

  • Fig. 4 Kaplan-Meier analysis of disease-free patient survival.

  • Fig. 5 Kaplan-Meier analysis of tumor recurrence according to the tumor size cutoff of 4 cm (A) and tumor number of 4 (B).


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