Metastatic lipoblastoma-like tumour

Introduction Lipoblastoma-like tumours (LLT) are uncommon and their biologic nature is not well-understood. Complete resection of the primary lesion is usually curative. This is the first reported case of metastatic LLT. Case presentation and management This patient originally had a large primary resected from the vulva. She subsequently developed tumours in multiple areas including her right pleura and lung. These were resected and pathology was consistent with LLT. On follow-up imaging, there was evidence of metastatic disease. Conclusion The patient is currently undergoing detailed molecular analysis in the hope of detecting a molecular target. Given the infrequent occurrence of LLT, its behaviour is not well understood. Longer duration of follow up is required and prognostic markers need to be identified to better direct treatment.