Low grade fibromyxoid sarcoma: problems in the diagnosis and management of a malignant tumour with bland histological appearance

SummaryAimsTo review the clinicopathological features and highligh the problems in the diagnosis and management of low grade fibromyxoid sarcomas (LGFMS).MethodsThree cases of LGFMS were studied with histology and immunohistochemistry, and cytogenetics in one. The features and problems were compared with those in the literature.ResultsTwo LGFMS had typical fibrotic and myxoid patterns showing abrupt transition from one to the other. Cellularity was low to moderate. Nuclei were medium sized and regular In one of these the correct diagnosis was not made in the original needle biopsy resulting in inappropriate manage ment. In the third tumour only myxoid areas were seen and the diagnosis was supported by cytogenetics showing a complex previously unreported translocation, t(7;18;16). One tumour recurred, one metastasised, and one has possible metastasis on imaging of the lungs.ConclusionLGFMS is a tumour with low grade histologica features but a high risk of local recurrence and a significan risk of metastasis which can be very late. There should be a high index of suspicion for this rare tumour and a low threshold for sending tissue for cytogenetics and/or mole- cular genetics. Special precautions should be exercised ir the interpretation of small biopsies of a spindle cell lesion with bland cytological features in children. If the diagnosis is unclear there must be a detailed follow up plan with a person responsible for monitoring the plan.