Abstract
Lipoblastoma is an extremely rare disease and mostly affects the infant to pediatric age group. Primarily, it involves the extremities, head and neck region, and rarely, the retroperitoneal region. Retroperitoneal lipoblastoma mainly presents with either abdominal pain or distension. We report a case of a 24-year-old male who presented with acute cord compression and underwent decompression and fixation with biopsy. Histopathology established the presence of lipoblastoma. Further staging workup showed no metastasis. Immunostaining and cytogenetics were positive for CD34 and negative for desmin, DNA damage-inducible transcript 3 (DDIT3), mouse double minute 2 (MDM2)(12q15), and pleomorphic adenoma gene 1 (PLAG1). The patient underwent resection of the retroperitoneal mass with intraoperative radiation therapy to the tumor bed. The histopathology identified the mass as retroperitoneal lipoblastoma. The postoperative course was uneventful and CT images showed no recurrence or metastasis. This was a unique case of retroperitoneal lipoblastoma in an adult with a unique clinical presentation.