Coexistence of Extraskeletal Mesenchymal Chondrosarcoma and Isolated Hemihyperplasia: A Case Report

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Demirkıran N. D., Akdeniz O., Hapa O., Havıtçıoğlu H.

Journal of Orthopaedic Case Reports, vol.62, pp.28-30, 2016 (Peer-Reviewed Journal)

  • Publication Type: Article / Case Report
  • Volume: 62
  • Publication Date: 2016
  • Journal Name: Journal of Orthopaedic Case Reports
  • Journal Indexes: Directory of Open Access Journals
  • Page Numbers: pp.28-30
  • Kütahya Health Sciences University Affiliated: No


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.

Keywords: chondrosarcoma; hemihyperplasia; mesenchymal tumor.