Low-grade fibromyxoid sarcoma of the maxilla: A diagnosis not to be missed

Low-grade fibromyxoid sarcoma (LGFMS) is a rare malignant spindle cell neoplasm that characteristically involves the trunk and extremities. The bland morphological appearance of the tumor makes it notoriously difficult for the pathologist to identify in routine microscopic sections especially when the tumor is located at unconventional sites. This ensues significant therapeutic implications as the tumor is known for long-standing distant metastasis. This case involves a 48-year-old lady who was diagnosed with this tumor at an extremely uncommon site as maxilla. The case report highlights the importance of meticulous histological examination and careful selection of immuno-histochemical markers in diagnosing this entity which might otherwise be missed clinico-radiologically.