Extraskeletal conventional chondrosarcoma of genitalia in a child- An unusual pathology for a rare tumor.

introduction and importanceChondrosarcoma is a rare malignant tumor considered as the second common sarcoma of bone following osteosarcoma. Less than 5 % of chondrosarcomas occur in children. Conventional chondrosarcoma is the most common type observed as skeletal tumors. Extraskeletal chondrosarcomas account for about 1 % of chondrosarcomas. They are almost always of myxoid or mesenchymal subtypes. CASE PRESENTATION: A 4-year-old girl was referred with pain and palpable mass in the labia majora since 2 years of age for which she had undergone twice biopsies, reported as soft tissue enchondroma. At this time, complete excision of the lesion with negative margins was carried out. The pathology was in favor of extraskeletal chondrosarcoma of conventional type without any evidence of bone involvement. CLINICAL DISCUSSION: Our case was a very young child with extraskeletal conventional chondrosarcoma, not reported yet in the literature. The vast majority of extraskeletal myxoid chondrosarcomas arise in the soft tissues of the extremities. The lesion in this case was located within the subcutaneous tissue of the labia majora; however, the tumor was not compatible with myxoid chondrosarcoma. Mesenchymal chondrosarcoma is another type of extraskeletal chondrosarcoma but the histopathologic patterns observed in our patient were not compatible even with the mesenchymal chondrosarcoma.

conclusionWe present a unique case of extraskeletal chondrosarcoma in a child with conventional subtype which has not been reported in the literature. In addition, the lesion was assumed to arise from a previous soft tissue enchondroma of the labia majora since infancy which makes the case distinctive.