Semaine de Rhumatologie Aix Les Bains

Congrès national de rhumatologie

Ewing’s sarcoma of the radius metastatic to bone marrow in an adult patient

Ewing’s sarcoma of the radius metastatic to bone marrow in an adult patient

B. Deschamps 1, 2, A. Mazouyes 3, F. Coury 1, 2

1. Department of Rheumatology, Centre Hospitalier Lyon Sud, 69495 Pierre-Bénite, France / 2. University of Lyon I, 69007 Lyon, France 3. Department of Rheumatology, Centre Hospitalier Alpes Leman, 74130 Contamine-sur-Arve, France

 Introduction

Ewing’s sarcoma is a rare tumor, far more frequent among children, but that can begin in adulthood. Most cases of Ewing’s sarcoma are localized disease and affect the diaphysis of long bones. However, metastases are present at diagnosis in about 20 to 30 % of cases. The course of this pathology, therefore best known in children, remains blurry in adults. Presentation, prognostic factors and treatment strategies still have to be assessed in order to improve the poor prognosis of this disease.

 Case report

A 31-year-old female with no significant past medical history was admitted to the rheumatology department through the emergency room with a 3-week history of severe inflammatory low back pain and diffuse pain of the lower limbs. The pain started suddenly, associated with an alteration of her general condition including asthenia and weight loss. She also mentioned profuse sweating, without documented fever.
The initial physical examination did not find any significant sign, besides a five-centimeter lump located at the distal end of the right arm, slightly sensitive, with no local sign of inflammation, appeared a few months ago. There was a diffuse back pain, no sciatica, no sign of infection and no sign of bone fracture. Blood test showed inflammation, with an elevated C-Reactive Protein (CRP) at 46,9mg/L. Complete Blood Count (CBC) was normal, except for a moderate leukocytosis to 12,6/mm3.
Magnetic Resonance Imaging (MRI) of the spine demonstrated a heterogeneous aspect of the medullar compartment, consistent with a post-partum state (she gave birth 18 months ago). A chest and abdomino-pelvic CT were then performed and revealed no abnormality. During hospitalization, the situation got worse. The patient became febrile (40°C), with intense sweating and a major alteration of the general state. Laboratory findings showed an important elevation of CRP at 600mg/L, of erythrocyte sedimentation rate at 90mm, of Lactate Dehydrogenase (LDH) to 2241U/L (superior to ten-fold the normal rate), of ferritin at 2994ug/L and a progressive anemia with hemoglobin at 8g/dl, compatible with a macrophage activation syndrome. A comprehensive search of infection was processed, but no microbiological agent was identified. The interferon-gamma release assay was negative and the transthoracic echocardiography found no sign of endocarditis. A Fluorodeoxyglucose positron emission tomography (PET) / computed tomography (PET/CT) showed an intense metabolism in the medullar compartment, suspect of blood disease, but without lymphadenopathy and without hepatic or spleen impairment (figure 1).
A medullar aspiration and bone marrow biopsy were performed and revealed a massive bone marrow infiltrate made of round, basophilic, poorly differentiated tumor cells expressing the Cluster of Differentiation 99 (CD99+). Proliferation index was increased, greater than 90 %. There was no sign of hemophagocytic syndrome. The other immune histochemical studies concerning cluster of differentiation in order to type tumor cells were negative. Identification of fusion transcript type PNET/Ewing: fusion transcript EWSR1(e7)-ERG(e6) confirmed the diagnosis of Ewing’s sarcoma.
MRI of the lump of the right arm was performed, showing a highly suspicious mass of the radius, suggestive of the primitive location of the Ewing’s sarcoma (figure 2). No other metastases additional to the bone marrow encroachment were found.
The patient started a chemotherapy program with VDCIA (EURO Ewing program 2012, Vincristine, Doxorubicin, Cyclophosphamide, Ifosfamide, Etoposide) in a specialized care center with a good response.

ewing

Figure 1 : PET scanner showing a diffuse and intense medullar hyper metabolism, matching with the macrophage activation syndrome.

Copyright net-partenaires® © 2005. Tous droits réservés.