Gorham-Stout disease of the pelvis: Seven years follow up with complete radiological evaluation

A 22-year old female presented in 2008 at the outpatients clinic of our department for evaluation of an extended pelvic osteolysis, being diagnosed accidentally after a fall fifteen days before administration. The patient reported inability to weight bearing and intense pain at her left hip. On clinical examination, edema of the proximal femur was obvious, while the patient could not perform any passive or active movement. No chronic diseases, weight loss or previous injury at the pelvis were documented at the patient’s past medical history. Her blood tests were within normal range, as well as inflammatory markers, while kidney and liver function was not disturbed. Tests regarding thyroid, parathyroid and steroid hormones were within normal values, while insufficiency of vitamin D was as 11.9 ng/ ml. Also no signs of autoimmune disease were detected. The urine calcium test was high as 323.82 mg/24hr (normal range 50-250 mg/24hr) and total urine proteins were 0.03 g/24hr (normal range 0.04-0.15 g/24hr). Markers of bone metabolism where within normal range. Simple anteroposterior x-ray of the pelvis (Figure 1A) showed extensive bilateral osteolysis of the pelvic ring. Lymphangiography with Technetium 99 showed increased uptake on right inguinal and popliteal lymph nodes. Absence of the middle and posterior wall of the right acetabulum was diagnosed by Computed Tomography (CT) scan (Figure 1B) and Magnetic Resonance Imaging (MRI) (Figure 1C), while arthritic changes of the right sacroiliac joint were revealed (Figure 1D). Biopsy under CT scan was performed and diagnosis of Gorham Stout disease was suggested, due to intramedullary destruction of bone architecture, as bone tissue was replaced by lymphatic tissue and osteoblastic activity was absent. Neoangiogenesis was also aggressive and extended widely in the bone marrow. The patient went under transcutaneous radiotherapy of the pelvis, receiving a total dose of 45 Gy, one year after the onset of the symptoms. Osteolysis was progressive and biphosphonates were administered. She had a yearly follow up with CT and MRI scan. On last examination (Figure 2A-C) osteolytic destruction of the posterior wall and the roof of the right acetabulum, edema of the right hip flexors and progressive arthritis of the involved right hip were demonstrated. The patient complained of complete inability to weight bearing on her right hip. The patient denied amputation of the extremity and nowadays she continues her daily activities by the use of crutches.