Coex stence of Extraskeletal Mesenchymal Chondrosarcoma and Isolated Hem hyperplas a: A Case Report

Introduction: Mesenchymal chondrosarcoma is a malignant tumor which originates from chondrogenic tissue. Agressive surgery is considered the major treatment method for the primary disease but there may be unsatisfactory results of surgery depending on location or extension to unresectable sites of majority of mesenchymal chondrosarcomas. Hemihyperplasia, formerly called hemihypertrophy, is a rare congenital overgrowth disorder in which one or more body parts on one side of the body grows more than other, causing asymmetry. Isolated hemihyperplasia is caused by an underlying abnormality of cell proliferation in individuals with neither any other underlying diagnosis nor vascular or skin anomalies. Isolated hemihyperplasia reveals an increased risk of embryonal tumors such as Wilms tumor, adrenal carcinoma or hepatoblastoma. However no such concomitance has been reported with chondrosarcoma. We report the first case of mesenchymal chondrosarcoma presenting with isolated hemihypertrophy. Case Report: A 64-year-old female presented with a palpable mass in posterolateral aspect of the left lateral malleolus which developed over 5 months. Hemihypertrophy involving the right side of her body was present and the patient had a history of foot shortening operation at adolescent age. Further examination of the patient revealed no vascular or skin anomalies, nor any findings suggesting a syndrome. Isolated hemihyperplasia diagnosis was achieved. After preoperative imaging studies, surgical excision was considered as best treatment option in local musculoskeletal tumor council and marginal resection was performed. The resected mass was sent for histopathological investigation. Immunohistochemical and morphological findings revealed malignant mesenchymal chondrosarcoma. Conclus on: IH is well known to be associated with increased incidence of some embryonal tumors like Wilms' tumor, adrenal carcinoma and hepatoblastoma. But no such association has been reported with chondrosarcoma. In musculoskeletal masses of patients with hemihyperplasia, the possibility of malignancy must be kept in mind.