Epithelioid hemangioendothelioma of the bone

Abstract

Introduction: Epithelioid hemangioendothelioma (EHE) of the bone is a very rare malignant vascular neoplasm, with biologic behavior between that of locally aggressive epithelioid hemangioma and malignant epithelioid angiosarcoma. We report a case of a patient with EHE who underwent bone scintigraphy, try to identify the characteristics of it, to highlight the clinical importance of whole-body bone scintigraphy and single-photon emission computed tomography/computed tomography (SPECT/ CT) in the diagnosis and treatment of EHE. Patient concerns: A 67-year-old man with no history of trauma who reported pain in both lower limbs for 6 months, which had been worsening over the last 20 days. Anteroposterior and lateral radiographs of both lower limbs revealed numerous osteolytic lesions in the tibia and fibula bilaterally. 99m Tc-methylene diphosphonate (MDP) bone scintigraphy demonstrated increased tracer uptake in the pelvic and bilateral lower limb bones. SPECT/CT bone imaging showed numerous osteolytic lesions cluster in the same anatomic region, with high tracer uptake in lesion margins. Diagnosis: EHE of the bone. Interventions: Two months after the diagnosis was confirmed, the patient was rehospitalized. Nonopioid analgesic use had not provided pain relief. Magnetic resonance imaging (MRI) of both thighs showed the bone cortex was destroyed with numerous irregular lesions, and soft-tissue was involved. A second bone scintigraphy did not show any new lesions. He was administrated with recombination human endostatin injection 15 mg ivgtt qd for 14 days, combined with apatinib mesylate tablets 500 mg po qd for 18 days. Outcomes: He was discharged voluntarily and died 2 months later. Conclusion: EHE of the bone is a very rare malignant vascular neoplasm with no specific radiographic imaging features. Whole-body bone scintigraphy, especially SPECT/CT bone imaging, significantly reduces ambiguous diagnoses and is recommended before treatment.

Epithelioid hemangioendothelioma of spine: A case report with review of literatures

“…But the low prevalence and variable presentation of EHE often makes it misdiagnosed [15] . On X-ray or CT scan, the typical characteristic of bone EHE is an osteolytic lesion with well-defined margin and without matrix mineralization [5 , 16 , 17] . On MRI, it also shows the lytic lesion with a sclerotic rim [2] .…”

18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography in a Rare Case of Epithelioid Hemangioendothelioma – A Diagnostic Challenge

“…[ 7 ] While, skeletal EHE involvement is often misdiagnosed as multiple myeloma, metastatic tumor, or brown tumor. [ 8 ] Xu et al . observed increased tracer uptake mostly in the lesion margins and defect inside lesions on technetium 99m-methyl diphosphonate bone scintigraphy with single-photon emission computed tomography/CT which they attributed to active bone dissolution and concluded high sensitivity of whole-body bone scintigraphy can help detect silent lesions.…”

A primary solitary vascular tumor of calcaneum: case report and review of literature

“…Mean age at presentation is 43 years. (Jang JK) It has propensity to occur in the second and third decades of life [7] .…”

“…The differential diagnosis for a solitary EHE depends on the patient's age and bone destruction pattern. It can range from benign lesions, like intraosseous lipoma, simple bone cyst, fibrous dysplasia and to malignant lesions, such as osteogenic sarcoma, fibrosarcoma, and multiple myeloma [7] . EHE is a very rare condition, and one should consider EHE after excluding more common benign lesions found in calcaneum like lipoma and simple bone cyst.…”