Published online Feb 6, 2020. doi: 10.12998/wjcc.v8.i3.614
Peer-review started: November 21, 2019
First decision: December 23, 2019
Revised: December 25, 2019
Accepted: January 2, 2020
Article in press: January 2, 2020
Published online: February 6, 2020
Erdheim-Chester disease (ECD) is a rare multi-system or multi-organ histiocytic proliferative disease with diverse clinical manifestations, and the development of the disease is complex, which makes clinical diagnosis and treatment difficult. The characteristic clinical manifestations include multi-organ involvement, especially in the symmetrical diaphysis and metaphysis of the bilateral extremities. ECD with a unilateral talus lesion is extremely rare. Here, we report an unusual case of ECD invading the asymmetric talus and tibia without involving other organs. The patient had good outcome after surgery.
We report a case of a 67-year-old man who was referred to our outpatient department because of left ankle chronic pain for 5 years, which exacerbated after a foot sprain 6 mo previously. We discovered multiple sclerotic lesions of the tibia and talus on his previous X-ray films, which were initially missed in a local hospital. Therefore, enhanced computer computed tomography (CT) and magnetic resonance imaging were performed. These examinations showed multiple lesions in the bone marrow cavity of the left tibia, and cortical sclerosis and osteonecrosis of the left talus. Specimens were collected via bone puncture from the two lesions, and a final diagnosis of ECD was confirmed by pathological and immunohistochemical examinations. In addition, other auxiliary examinations including head CT, pulmonary CT, spinal CT, abdominal CT, cardiac ultrasound and thyroid ultrasound showed no obvious abnormalities. The patient underwent surgery for the tibia lesion scraping and talus lesion scraping combined with cement casting. The patient started on a progressive rehabilitation at 4 wk, and felt no pain after surgery. During a 2-year follow-up period, the patient exercised normally without pain, and there were no signs of recurrence.
This study shows that surgery treatment may also achieve good results for ECD patients with only bone involvement.
Core tip: Erdheim-Chester disease (ECD) is a subtype of non-Langerhans’s cell histiocytosis, for which diagnosis is difficult and no treatment guidelines have been available. In addition, ECD is a systemic disease with multi-organ involvement, and especially affects the symmetrical diaphysis and metaphysis of the bilateral extremities. We report the first case of ECD invading the unilateral tibia and talus without involvement of any other organs. The patient was treated with surgery, and had a good prognosis, which added evidence to the diagnosis and treatment of this disease.