Diagnosis and therapy of bone epithelioid hemangioendothelioma: a report of 9 cases

Abstract

Objective To analyze nine cases of bone epithelioid hemangioendothelioma (B-EHE) , retrospectively, and discuss the diagnosis and treatment. Methods Retrospective analysis of 9 patients of B-EHE in Eastern Theater General Hospital of PLA from February 2002 to September 2015 was performed, 6 males and 3 females, ranged from 15 to 60 years. The surgical method of three cases was entire surgical excision, internally fixating with bone graft. One patient that lesion was located in lumbar vertebra was chosen scraped for decompression and stabilization. The 3 cases of adjacent joints lesions were en bloc excision with a modular resection prosthesis, one adopted chemotherapy after surgery. One case who presented as multifocal with pulmonary metastasis, conducted chemotherapy when identified by biopsy. One case occurred pathological fracture and hemorrhage and accepted comprehensive therapy after amputation. Results In 9 cases, 1 case was lost to follow-up. The follow-up time of the rest was from 8 to 72 months. Seven cases survived. Three patients underwent bone grafting and internal fixation; two had local recurrence, and were cured after the second operation. One case that the tumor locate lumbar was resected and decompression fixation, and had good function after operation. Three patients underwent tumor resection and prosthesis replacement, and 2 patients were cured postoperatively with good joint function. Among them, 1 patient received postoperative adjuvant chemotherapy without local recurrence and metastasis, and 1 patient lost follow-up. In 1 case, multiple lesions with pulmonary metastasis were treated with chemotherapy, with no pulmonary progression and no local progression. One patient received comprehensive treatment after amputation, and the tumor recurred and extended bleeding, and finally died of organ failure. Conclusions B-EHE is a low-to-moderate malignant tumor, which has no specificity in preoperative imaging and is difficult to diagnose. It mainly depends on postoperative pathological results for diagnosis. The preferred treatment is boundary resection, combined with chemotherapy or radiotherapy, local recurrence and distant metastasis. Key words: Bone neoplasms; Bone epithelioid hemangioendothelioma; Diagnosis; Comprehensive treatment