Abstract Background: Renal cell carcinoma (RCC) is rare in children, and extrarenal RCC is extremely uncommon, with very few cases reported. Atypical presentations pose a significant diagnostic challenge. Clinical Description: A 15-year-old girl who presented with multiple progressively enlarging scalp swellings. On evaluation, she was found to have multiple hard, nontender, fixed bony scalp lesions, left supraclavicular lymphadenopathy, and a firm epigastric mass. The overlying skin was normal, and there were no features of raised intracranial pressure. Management and Outcome: Blood investigations revealed a normal blood picture and tumor markers. Imaging revealed a retroperitoneal mass encasing the inferior vena cava and widespread bone metastases, while both kidneys were normal. Biopsy and immunohistochemistry of the supraclavicular lymph node confirmed translocation-type extrarenal RCC. She received sunitinib followed by lenvatinib, but therapy was complicated by skin discoloration, refractory thrombocytopenia, and thyroiditis. Conclusion: Extra-renal RCC in children is rare and may present with unusual manifestations. This case underscores the importance of considering metastatic malignancy in a child presenting with atypical scalp swellings, which could be due to extra-renal RCC.
Munusamy et al. (Wed,) studied this question.