Ewing’s Sarcoma of the Humerus in a Three year-old boy Requiring Radical En-Bloc resection and Total Humeral Expandable Prosthetic Replacement: A Case Report

Ewing’s sarcoma is a malignant bone tumor that affects children typically in their second decade, with a low incidence in patients less than five years of age. Presenting symptoms may be nonspecific and may include pain, swelling, fever, and weight loss. Definitive diagnosis may be difficult and further investigation may be necessary to differentiate Ewing’s sarcomas with osteomyelitis.We present a case report of a three year-old boy with Ewing’s sarcoma that involved the entire right humerus. Due to his young age, and nonspecific nature of his presentation, the patient was initially misdiagnosed and treated for osteomyelitis. The patient required multi-agent chemotherapy and radical en-bloc resection of the entire right humerus, given the extent of his tumor involvement. A custom expandable modular humeral prosthesis was designed and engineered specifically for this patient. His arm is very functional three years postoperatively, and he is disease free. To our knowledge, this is the youngest report of Ewing’s sarcoma that required total humeral resection and prosthetic replacement presented in the literature. *Corresponding author: Don Young Park, MD, 300 Pasteur Dr. Stanford, CA 94305, USA, Tel: 650 497 8201; Fax: 650-497-8891; E-mail: [email protected] Received September 13, 2011; Accepted November 17, 2011; Published November 18, 2011 Citation: Park DY, Batista E, Rinsky LA (2011) Ewing’s Sarcoma of the Humerus in a Three year-old boy Requiring Radical En-Bloc resection and Total Humeral Expandable Prosthetic Replacement: A Case Report. Surgery 1:101. doi:10.4172/2161-1076.1000101 Copyright: © 2011 Park DY, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Introduction Ewing’s sarcoma, a small round blue cell tumor, is the second most prevalent primary malignant bone cancer in children, following osteosarcoma [1]. It belongs in the Ewing sarcoma family of tumors (ESFT), a group of tumors that also includes primitive neuroectodermal tumor (PNET) and Askin tumor [2]. Small round cell neoplasms and a common chromosomal translocation characterizes ESFT. Approximately 95% of Ewing’s sarcoma patients have a t(11;22) or t(21;22) translocation, resulting in EWS and either FLI1 (chromosome 11) or ERG (chromosome 21) gene fusion [3]. In the United States, the incidence of Ewing’s sarcoma is estimated at 2-3 cases per million children and has not changed over a 30 year time period [4,5]. Typically, Ewing’s sarcoma has a peak incidence in the second decade but has been reported in all age groups [5]. In patients less than 5 years of age, the annual incidence is low, estimated at 0.6 per million [2]. In addition, only 3% of Ewing’s sarcoma occurs in patients younger than 3 years of age [6]. Ewing’s sarcoma can occur in the axial and appendicular skeleton with nearly equal frequency [3]. Tumors of the humerus occur infrequently with approximately 10% of Ewing’s sarcoma found in this location [7]. Presenting symptoms may include pain, swelling, fever, and weight loss, which can be consistent in more common conditions, such as osteomyelitis, [1-3]. The classic radiographic appearance of Ewing’s sarcoma With “onionskinning” periosteal reaction also can be seen in osteomyelitis [8]. Due to the Nonspecific nature of presentation, definitive diagnosis may be difficult and further investigation, including imaging studies and open biopsy may be necessary. We present a case of a three year-old boy with Ewing’s sarcoma that involved the entire right humerus. The patient required multi-agent chemotherapy and radical en-bloc resection of the Entire right humerus and replacement with a custom expandable modular humeral Prosthesis. To our knowledge, this is the youngest report of Ewing’s sarcoma that Required total humeral resection and prosthetic replacement presented in the literature.