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

INTRODUCTION AND IMPORTANCE: Chondrosarcoma 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. CONCLUSION: We 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.

The outcome and complications of modern staged repair surgery in newborns with classic bladder exstrophy in different genders: A retrospective study.

INTRODUCTION: Classic bladder exstrophy (CBE) is a rare anterior midline birth defect that remains a challenge for pediatric surgeons. Despite multiple reconstructive methods, outcomes vary widely in various reports. This study aims to compare the success rate and complications of modern staged repair of exstrophy (MSRE) in each gender and compare together. METHODS: This retrospective cross-sectional study included cases of CBE between 2010 and 2020 that underwent MSRE. Short-term follow-up results, including incontinence rate, vesicoureteral reflux (VUR), urinary infections, deformed genitalia, and so on, were measured in each gender, and their differences were reported. RESULTS: Among the 40 newborns with CBE who underwent MSRE, 25 (62.5%) were boys, while the others had non-male genitalia. The rates of incontinence, VUR, dehiscence, and fistulas did not differ significantly between genders. However, chronic urinary tract infections (UTIs) were more frequent in girls, and boys were more likely to have malformed genitalia (p < .05). CONCLUSION: Our findings indicate a similar rate of complications in each gender. However, chronic UTIs and external genitalia deformities were significantly more common in girls and boys, respectively. Further large-sized controlled trials may be needed to corroborate these findings.