A trimodality approach in the management of metastatic low-grade epithelioid hemangioendothelioma of the bone

Abstract

SUMMARYA 29-year-old man presented with a 2-week history of severe pain in the left foot with no preceding history of trauma. A left foot radiograph demonstrated a cortical lucency in the mid-distal shaft of the third metatarsal bone. MRI of the left foot showed an expansile lesion in the same location. A staging bone scan showed a focal uptake in the known lucency in the left third metatarsal and in the proximal left femur. A subsequent left hip radiograph demonstrated a lucency in the intertrochanteric region. CT scan of the chest, abdomen and pelvis was unremarkable. A biopsy of the left third metatarsal expansile lesion performed during an incision and curettage procedure revealed an epithelioid haemangioma (EHE) of the bone. MRI of the left hip performed in response to the findings on the bone scan showed metastatic disease in the left intertrochanteric region. A prophylactic left hip fixation surgery with an interlocking intramedullary femoral nail was therefore undertaken to avoid a pathological fracture of the left hip from the metastatic disease. Simultaneously, a left hip biopsy was performed, which also revealed an EHE. The patient underwent external beam radiation to the left femoral head and neck. This was followed by fractionated radiosurgery to the left third metatarsal. Once the left foot wound had healed, the patient subsequently received four cycles of doxorubicin and ifosfamide. A restaging positron emission tomography CT carried out after completion of therapy showed no metabolic evidence of residual primary tumour or metastasis. More than 2 years after completing his trimodality therapy, the patient remains fully functional and symptom free. BACKGROUND

Metastatic Epithelioid Hemangioendothelioma of the Bone: A Case Report and Literature Review

“…No single chemotherapy regimen has been found to be 100% successful, and the chemotherapy methods employed are based on a succession of EHEs involving many locations. In numerous documented case series, anthracyclines were the most commonly used first-line chemotherapeutic agents [24][25][26]. A series of 29 patients with multicentric or localized bone EHE was published in 1986, with a follow-up of 33 months after surgery or adjuvant radiotherapy.…”

Epithelioid hemangioendothelioma of the distal lower extremity and the role of radiotherapy: A report of two cases

“…2 , 5 , 10 Saste et al described success in using radiation therapy in combination with chemotherapy to treat a multifocal lower extremity EHE on a young male patient. 11 Albakr et al reviewed the current literature of spinal EHE management and describes how post-operative RT with doses ranging from 40 to 60 Gy obtained local pain control and better quality of life by at least 1-year follow-up. 12 Kleer et al reported on a series of 40 cases EHE of bone, however was unable to conclude the efficacy of RT given the metastatic disease present in the majority of patients at the time of diagnosis.…”

Application of Ultrahigh Doses of Morphine in Systemic Multiple Epithelioid Hemangioendothelioma without Addiction: A Case Report

“…Lesions are frequently found in bone, lung, liver, soft tissue, and skin [1] [2]. Most EHE patients are asymptomatic at the time of the diagnosis, but as the disease advances, severe burning pain is the most common symptom [3]. Currently, various therapies, including drug treatment [4] [5], interventional therapy [6], radiotherapy [7], and surgical treatment [8] have been described, however, none of these therapies can completely cure this disease, and pain may become more and more refractory [9].…”