|Year : 2018 | Volume
| Issue : 4 | Page : 295-297
Ewing's sarcoma of calcaneum masquerading as chronic osteomyelitis: A case report
Siva K Prasad1, K Meher Lakshmi1, Venkateswara Rao Pydi1, Shantveer G Uppin2
1 Department of Medical Oncology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
2 Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
|Date of Web Publication||10-Jan-2019|
Dr. K Meher Lakshmi
Department of Medical Oncology, Nizamís Institute of Medical Sciences, Hyderabad, Telangana
Source of Support: None, Conflict of Interest: None
Ewing's sarcoma is the second most common malignancy of bone among children. It commonly affects the diaphysial region of long bones, while small bones of hands and foot are rarely affected. We report a case of calcaneal Ewing's sarcoma which was misdiagnosed as fungal chronic osteomyelitis and treated inappropriately. High index of suspicion is required in diagnosing tumors at rare sites to avoid undue delay in initiating appropriate therapy.
Keywords: Calcaneum, Ewing's sarcoma, osteomyelitis
|How to cite this article:|
Prasad SK, Lakshmi K M, Pydi VR, Uppin SG. Ewing's sarcoma of calcaneum masquerading as chronic osteomyelitis: A case report. J NTR Univ Health Sci 2018;7:295-7
|How to cite this URL:|
Prasad SK, Lakshmi K M, Pydi VR, Uppin SG. Ewing's sarcoma of calcaneum masquerading as chronic osteomyelitis: A case report. J NTR Univ Health Sci [serial online] 2018 [cited 2020 Apr 5];7:295-7. Available from: http://www.jdrntruhs.org/text.asp?2018/7/4/295/249822
| Introduction|| |
Ewing's sarcoma/primitive neuroectodermal tumors (PNETs) of the bone are part of the Ewing's family of tumors. After osteosarcoma, they are the second most common tumors of the bone in children and young adults with male predilection. The peak age of incidence is in the first two decades. Pathologically, they are categorized as small round blue cell tumors. They characteristically exhibit chromosomal translocation between chromosomes 11 and 22 which leads to the formation of EWS-FLI protein in approximately 80%–90% of patients. They are a heterogeneous group of tumors affecting long bones of extremities, axial skeleton, and rarely flat bones and small bones of hand or foot. We report a case of calcaneal Ewing's sarcoma masquerading as chronic osteomyelitis.
| Case Details|| |
A 27-year-old man, without any comorbidities or history of trauma, presented with pain in the right heel of 2 years duration. On general examination, his Eastern Cooperative Oncology Group performance status was 1 and vital parameters were stable. On local examination, there is mild, diffuse, hard, globular swelling around the heel without any local increase in temperature or tenderness [Figure 1]a. Distal pulses were normal, and neural examination was unremarkable.
|Figure 1: (a) Clinical image showing right ankle and swelling; (b) plain X-ray of right ankle showing sclerosis of calcaneum; (c) magnetic resonance imaging showing signal intensity changes in calcaneum; (d) histopathological image showing round, blue cell tumor in sheets|
Click here to view
Eighteen months back, he was evaluated for the above complaints at his local place. X-ray was suggestive of calcaneal sclerosis and osteomyelitis was suspected. As there was no improvement in his symptoms with antibiotics, fine needle aspiration was done. It was suggestive of chronic inflammatory changes with a possibility of chronic fungal osteomyelitis. He received antifungal therapy for a couple of months, but his pain was persistent. He was then referred to our institute for further management.
In our institute, X-ray was repeated which showed ostesclerosed right calcaneum. Other small joints of foot appeared normal [Figure 1]b. Magnetic imaging resonance (MRI) of the right ankle was performed which showed signal intensity changes in the calcaneum [Figure 1]c. Bone biopsy was done for conclusive diagnosis which was suggestive of small round cell tumor. On immunohistochemistry, CD99 was positive, and Leucocyte common antigen (LCA) and Tdt were negative [Figure 1]d. Further testing with fluorescent in situ hybridization showed FLI-EWS positivity. Staging work-up with contrast-enhanced computed tomography (CT) of chest, bone scan, and bone marrow biopsy revealed no evidence of metastasis. A final diagnosis of localized Ewing's sarcoma of right calcaneum was arrived at.
He was given 12 weeks of neoadjuvant chemotherapy (NACT) with vincristine, doxorubicin, and cyclophosphamide (VAC) alternating with ifosfamide and etoposide (IE). After discussion with the patient regarding surgery and radiation, an above-ankle amputation was done. Postoperative histopathology showed grade 1 response to chemotherapy, and margins were free from tumor. He is currently undergoing the remaining cycles of chemotherapy and is able to carry on all his activities without any difficulty in view of prosthesis placement.
| Discussion|| |
Ewing's sarcoma/PNETs are round cell sarcomas with varying neuroectodermal differentiation. Ewing's sarcoma family of tumors consists of Ewing's sarcoma of bone, extraosseous Ewing's sarcoma, Askin's tumor (chest wall), peripheral neuroepithelioma, and atypical Ewing's sarcoma. All these tumors characteristically harbor specific chromosomal translocation. The classical teaching of Ewing's sarcoma is that it arises from diaphysis or metaphysio-diaphysial portion of long bones. Other bone involvement is less common. Ewing's sarcoma is often associated with systemic symptoms such as anemia, weight loss, fever, leucocytosis, and high sedimentation rate.
On plain X-ray, Ewing's sarcoma appears as osteolytic lesion with permeative or moth-eaten destruction of the bone with soft tissue component. In some cases, large lesion with expansile bone lesion with soap bubble appearance can be seen. MRI and CT scan helps delineate the extent of the tumor.
Pathologically, these tumors are composed of sheets of small, round cells with PAS-positive cytoplasm and occasional Homer–Wright rosettes. On immunohistochemical staining, they are positive for CD99 and neuron-specific enolase. Recurrent translocation (11; 22) (q24; q12) is seen in approximately 85% of the cases. Chromosomal abnormalities involving 1, 8, and 12 are seen in some cases.
Although Ewing's sarcoma principally arises from long bones, it can arise from any bone. Ewing's sarcoma intergroup study had 3% of tumors affecting hand or foot. Similarly, Dahlin et al. reported involvement of foot in 2.4% of patients. Italian Association of Pediatric Hematology and Oncology and Italian Sarcoma Group published their 30-year experience with unusual sites of 112 cases of Ewing's sarcoma. In their analysis, hand and foot small bone involvement was seen in 33% of patients. Adkins et al. reported 16 cases of Ewing's sarcoma of foot. They noted poor survival in their study and a delay in diagnosis due to atypical site and imaging features. Prognostic factors affecting the survival are age, site, stage, response to NACT, and completeness of resection. Prognosis is thought to be worse in cases of foot involvement.
Treatment of Ewing's sarcoma requires multimodality approach. NACT for 12 weeks with VAC/IE followed by surgical excision is the standard practice. For unresectable tumors, radical radiotherapy is advocated. Adjuvant radiation is required for tumors with incomplete surgical resection or intraoperative spill, chest wall involvement, and pathologically involved lymph nodes. Addition of local therapy to systemic therapy has had a dramatic improvement in survival, and the reported 5- and 10-year survival rates are now approximately 70% and 50%, respectively.,
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Burchill SA. Ewing's sarcoma: Diagnostic, prognostic, and therapeutic implications of molecular abnormalities. J Clin Pathol 2003;56:96-102.
Picci P, Rougraff BT, Bacci G, Neff JR, Sangiorgi L, Cazzola A, et al
. Prognostic significance of histopathologic response to chemotherapy in nonmetastatic Ewing's sarcoma of the extremities. J Clin Oncol 1993;11:1763-9.
Beth McCarville M, Chen JY, Coleman JL, Li Y, Li X, Adderson EE, et al
. JOURNAL CLUB: Distinguishing osteomyelitis from Ewing sarcoma on radiography and MRI. Am J Roentgenol 2015;205:640-51.
Aurias A, Rimbaut C, Buffe D, Dubousset J, Mazabraud A. Chromosomal translocations in Ewing's sarcoma. N Engl J Med 1983;309:496-7.
Reinus WR, Gilula LA, Shirley SK, Askin FB, Siegal GP. Radiographic appearance of Ewing sarcoma of the hands and feet: Report from the Intergroup Ewing Sarcoma Study. Am J Roentgenol 1985;144:331-6.
Dahlin DC, Coventry MB, Scanlon PW. Ewing's sarcoma: A critical analysis of 165 cases. J Bone Joint Surg Am 1961;43-A: 185-92.
Berger M, Fagioli F, Abate M, Riccardi R, Prete A, Cozza R, et al
. Unusual sites of Ewing sarcoma (ES): A retrospective multicenter 30-year experience of the Italian Association of Pediatric Hematology and Oncology (AIEOP) and Italian Sarcoma Group (ISG). Eur J Cancer 2013;49:3658-65.
Adkins CD, Kitaoka HB, Seidl RK, Pritchard DJ. Ewing's Sarcoma of the foot. Clin Orthop 1997;342:173-82.
Craft A, Cotterill S, Malcolm A, Spooner D, Grimer R, Souhami R, et al
. Ifosfamide-containing chemotherapy in Ewing's sarcoma: The Second United Kingdom Children's Cancer Study Group and the Medical Research Council Ewing's Tumor Study. J Clin Oncol 1998;16:3628.
Paulussen M. Localized Ewing tumor of bone: Final results of the cooperative Ewing's Sarcoma Study CESS 86. J Clin Oncol 2001;19:1818.