A Clinical Sign of Chronic Osteomyelitis in Ewing’s Sarcoma

Already published in Sari Pediatri 2009;11(1):52-5

 A Clinical Sign of Chronic Osteomyelitis in Ewing’s Sarcoma

Sri Mulatsih, Vicka Farah Diba
Pediatric Health Department, Medical Faculty, Gadjah Mada University
Dr Sardjito General Hopital, Yogyakarta.

Background

Ewing’s sarcoma is the second most common cancer of bone in children and adolescents and it is the most lethal bone tumor, with an incidence of 2.1 per million children in the United States. Its cause is unknown.2 Most frequently, the tumor is diagnosed as a monostatic lesion in the metaphysis or diaphysis of the long bones of the extremities. The tumor may also occur, although less frequently, in the pelvic area, ribs, and scapulae. In fact, any bone may be involved.1

Occasionally, the clinical picture may be similar to that of acute or chronic osteomyelitis, include remittent fever, mild anemia, leukocytosis, elevated erythrocyte sedimentation rate (ESR) and increased serum lactic dehydrogenase (LDH).1,4 Eventually, most patients have a large palpable mass, which grows rapidly, with a tense and tender local swelling. Symptoms usually last a few weeks to a few months.1 The diagnosis of acute osteomyelitis is confirmed by the presence of two of the following criteria: pus aspirated from the bone, a positive blood or bone culture, local signs of inflammation, and radiographic or bone scan changes.3,4 No single morphologic or functional imaging method provides findings for a specific diagnosis of Ewing’s sarcoma. Appropriate tumor samples, including fresh tissue,should be obtained for routine pathological evaluation.2

Ewing sarcoma merupakan tumor tulang pada anak yang sering dijumpai. Gambaran klinis maupun radiologis tumor ini sangat mirip dengan infeksi tulang yaitu osteomielitis akut maupun kronik, dengan keluhan benjolan pada tulang yang dirasa nyeri. Dengan demikian, sering kali pasien datang terlambat karena di diagnosis infeksi tulang. Diperlukan pemeriksaan patologi anatomi pada pasien dengan osteomielitis kronis untuk menyingkirkan adanya keganasan tulang. (Sari Pediatri 2009;11(1):52-5). Kata kunci: tulang, nyeri, benjolan, sarkoma, ostomielistis. 

Case report

A 5 year old girl came with chief complain swelling in her right ankle joint, accompanied with pain, fever and inability to walk. The complain started since 9 months ago. First she was taken to HI Hospital and performed rontgent of chest, vertebral and ankle joint and the result she was suffered from pulmonary TBC, then got treatment for pulmonary TBC.

4 months after the treatment the complain of swelling and pain in right ankle joint still persisted, then she was taken to DSGH and performed laboratory examination and aspiration of the ankle joint fluid with the results no infection of gram positif or negatif bacteria nor acid fast baccil and no malignancy, based on the results, she was diagnosed as JIA and got therapy Ibuprofen and also still continue pulmonary TBC treatment.

After completed TBC treatment and two months treatment of JIA, the complain still persisted then she was hospitalized for the second time in DSGH.

From the physical examination the nutritional status is undernourished, we found swelling in right ankle joint with fever, redness, pain and limitation of movement. (fig. 1)

Fig.1 Progressive swelling of ankle joint in 1 month

TB score was three and from the follow up in the ward found lymphadenopathy, abdominal mass, paraperesis inferior flaccyd, retentio urine, but no enlargement of liver and spleen an no uveitis found in the eyes.

From laboratory finding found anemia hypochrom micrositer and leucocytosis and from peripheral blood smear showed anemia hypochrom micrositer with infection process.

Fig. 3 X-ray of Right Ankle joint Widening of ankle joint and thickening of soft tissue showed chronic infection process due to suspect Osteomyelitis TB

From the x ray evaluation of right ankle joint showed widening of ankle joint and thickening of soft tissue showed chronic infection process due to suspect Osteomyelitis TB. From chest X–ray found resolving of pulmonary TBC and from vertebral x-ray found compression in V Th 11 with paravertebral abcess in V Th 9 – 12

Fig. 4. Histology finding found mesenchymal malignancy cell

From histopatology finding of ankle joint fluid found mesenchymal cell malignancy showed the appearance of Ewing’s sarcoma.

Fig. 5 Bone Marrow Aspiration found metastatic malignancy cell

From bone marrow aspiration found the appearance of metastatic cel. From CT scan abdomen showed urinoma due to chronic retentio urine. The child now confirmed suffer from Ewing’s sacoma and plan to have chemotherapy of Ewing’s sarcoma and plan to get biopsy from abcess in vertebral Th 9 – 12.

Discussion
The most common early sign of osteomyelitis during the first few months of life is swelling of the soft tissue and decreased spontaneous motion of the affected limb.3 In Ewing’s sarcoma the most common initial symptom is also pain or swelling in a bone or joint, eventually most patients have a large palpable mass, which grows rapidly, with a tense and tender local swelling,1 The presence of systemic symptoms such as weight loss and fever may initially lead to an missed diagnosis (e.g., osteomyelitis).2 In this case from history taking, physical examination and laboratory examination is similar to the sign and symptom of chronic osteomyelitis that can occur in the history of Ewing’s sarcoma.

The differential diagnosis of acute localized bone pain in a child who has systemic signs includes osteomyelitis, septic arthritis, acute rheumatoid arthritis, acute rheumatic fever, malignancy (eg, leukemia, Ewing sarcoma, and metastatic neuroblastoma), bone infarction (ie, sickle cell disease), and toxic synovitis. The cardinal rule is that any child who has fever and bone tenderness should be considered to have acute osteomyelitis until proven otherwise. 3,4

The diagnosis of acute osteomyelitis is confirmed by the presence of two of the following criteria: pus aspirated from the bone, a positive blood or bone culture, local signs of inflammation, and radiographic or bone scan changes.3,4 It may take up to 2 weeks for bony changes to appear on conventional radiographs. They are useful initially to exclude fracture or malignancy from the differential diagnosis. 3,4 Isolation of a pathogen from the blood, bone, or subperiosteal pus is the cornerstone of the diagnosis of osteomyelitis. Blood cultures are positive in only 50% to 60% of cases. Performance of cultures of both blood and material obtained by needle aspiration of the involved bone will yield a positive culture in 75% to 80% of cases. Bone aspiration should be performed even if the child has already been started on antibiotics because the culture may be positive for several days after the start of therapy, especially if the pathogen is S aureus.4. There is no single morphologic or functional imaging method provides findings for a specific diagnosis of Ewing’s sarcoma.2 The tumor originates in the shaft of the bone, and the earliest radiographic findings may appear as a roughening of the peniosteum, which may be confused with osteomyelitis.5 Eventually, a Ewing sarcoma tends to be extensive, sometimes involving the entire shaft of the long bone. Lytic destruction is the most common finding, but there may be regions of density with new bone formation.5,7 As the tumor bursts through the cortex, it often elevates the peniosteum, gradually producing multiple layers of subpeniosteal reactive new bone to give an “onion skin” appearance.5,6 Appropriate tumor samples should be obtained for pathological evaluation. 2
From this case, radiologic finding was found widening of ankle joint and thickening of soft tissue showed chronic infection process due to suspect Osteomyelitis TB and found compression in V Th 11 with paravertebral abcess in V Th 9 – 12, but unresponsive of TBC treatment and from histologic finding of ankle joint fluid found mesenchymal cell malignancy showed the appearance of Ewing’s sarcoma. From this data is strongly supported to the present of Ewing’s sarcoma.

Approximately 20 to 25 percent of patients with Ewing’s sarcoma have metastatic disease at presentation. Of patients who present with metastatic disease, the sites of metastases are the lungs in 50 percent of cases, bone in 25 percent of cases,and bone marrow in 25 percent of cases of Ewing’s sarcoma Because Ewing’s sarcoma often metastasize to bone marrow, patients in whom these tumors are suspected must undergo bone marrow aspiration and biopsy at two or more sites.2 In this case we also found metastatic cell in bone marrow aspiration.

From this case we can learn that earlier histologic finding is needed to be done in case of chronic swelling and bone pain especially in the case that suspected osteomyelitis but unresponsive to treatment so an earlier diagnosis of bone malignancy can be done.

Conclusion
The clinical sign of Ewing’s sarcoma may be similar to chronic osteomyelitis. Earlier histologic finding is needed to be done in the case that suspected to osteomyelitis but unresponsive to treatment so an earlier diagnosis of bone malignancy can be done.

REFFERENCES
1.Strauss G Ludwig, MD., 2007, Ewing Sarcoma, http//www.emedicine.com/topic275. Html.
2.AS Carola., Arndt., M William Crist., 1999, Common Musculoskeletal Tumors of Childhood and Adolescence, N Engl J Med; 30 (5); 342 – 350
3.Roy R Dennis, 1995, Osteomyelitis, Pediatr. Rev.;16;380-384
4.Gold Ronald, 1999, Diagnosis of Osteomyelitis, Pediatr. Rev. 1;12;292-296
5.J Norman., 1991, Osteosarcoma, Pediatric Review;12; 333-343
6.Hevroni Avigdor, M.D., Koplewitz Z Benjamin., M.D, 2007, Bone within Bone — Chronic Osteomyelitis, N Engl J Med; 356;8; e7
7.Horowitz M. E., Tsokos M. G., DeLaney T. F., 1992, Ewing’s sarcoma, CA Cancer J Clin; 42; 300-320