Introduction: Chondroid syringoma (CS) is a rare adnexal tumor, exceptionally uncommon in children. To date, only isolated case reports exist, and no systematic synthesis has been performed. Objectives: We conducted a PRISMA-guided systematic review of pediatric CS and present an illustrative case that provides the first high-frequency ultrasound (HFUS) and elastography findings. Methods: PubMed, Scopus, and Web of Science were searched through September 2025. Eligible reports included pediatric patients (<18 years) with histopathologically confirmed CS. Data on demographics, tumor location, diagnostic methods, treatment and outcomes were extracted. In parallel, an 8-year-old girl with CS was evaluated clinically, dermoscopically, and with HFUS and elastography, followed by surgical excision and histopathology. Results: The systematic review identified eight pediatric cases in the literature, three malignant and five benign. Most tumors arose in the head and neck region; two occurred in the extremities, including one in association with eccrine spiradenoma. All cases were managed surgically, with favorable outcomes in benign lesions, while malignant cases demonstrated recurrence, metastasis, and one death. Our case presented as a cheek nodule with dynamic dermoscopic changes and HFUS features of a round, lobulated, hypoechoic lesion with posterior enhancement, vascularity, increased stiffness, and a budding yeast–like appearance. Complete excision achieved an excellent cosmetic result without recurrence at one year. Conclusions: This study provides the first systematic review of pediatric CS, consolidating current diagnostic and therapeutic knowledge. Our case adds the first detailed HFUS and elastography findings, underscoring the role of imaging as a non-invasive adjunct. Complete excision with long-term surveillance remains the cornerstone of management.
Paker et al. (Mon,) studied this question.