A 49-year-old Hispanic woman presented to the ambulatory medicine service for worsening oedema of her right upper extremity. Six years prior to presentation, she noticed painless swelling of her right thumb, which she initially attributed to an injury that occurred while working in a chicken-processing plant. She reported receiving repeated local injections of an unknown antibiotic in Honduras over the course of 2 weeks without relief. As time progressed, so did the swelling with extension from her hand to shoulder with eventual involvement of her right chest wall and hemithorax (Figure 1). Her medical evaluation at that time was extremely limited and included a complete blood count and an X-ray, both of which she reported were normal, and hence, no further workup was sought. Eighteen months before presentation to the clinic, she developed dyspnoea on exertion with occasional cough. She reported right arm and back pain from the significant swelling with associated right chest wall discomfort. Apart from postprandial nausea, she denied any fever, night sweats, weight loss, or lower extremity oedema. A computed tomography (CT) scan of the chest with contrast revealed soft tissue oedema without evidence of thrombus or obstruction in the venous system (Figure 2). Further evaluation for multiple infectious aetiologies, including tularaemia, coccidiodes, and malaria, were negative; however, she did have a positive purified protein derivative (PPD) test for which she received and completed latent tuberculosis treatment. Subsequent lymphoscintigraphy showed evidence of lymphatic obstruction at the level of the distal forearm and she was referred to a lymphedema clinic for compressive therapy. Her oedema continued to progress resulting in a large right-sided exudative chylus pleural effusion requiring thoracentesis. Fluid analysis for at that time was negative for malignancy and cultures were negative for acid-fast bacilli. She again presented two months later with dyspnoea and was found to have a re-accumulation of her pleural effusion at which time she underwent pleuroscopy with biopsy and pleurodesis with pleural drain placement. Although repeat pleural fluid analysis was negative for malignancy, thoracoscopy revealed a pleural space that was densely adherent in multiple locations with gelatinous, firm material. Pathological examination showed sections of very nodular neoplastic proliferation of small spindled to ovoid cells with eosinophilic cytoplasm and bland nuclei within a predominantly myxoid stroma. A 'whorled' cellular morphology was identified multifocally without mitotic figures or necrosis. Immunohistochemically, the tumour cells involved were positive for epithelial membrane antigen and progesterone receptor but negative for Ewing sarcoma break point 1 gene translocation, smooth muscle antigen, cytokeratin AE1/AE3, S100, calretinin, microphthalmia-associated transcription factor (MITF), and claudin 1 (Figure 3). The cytogenetics from the cell cultures of the pleural biopsy revealed complex chromosome translocations and deletions 45,XX,-6,inv(9)(p24q32)x2[14]/90,idemx2[3]/44,idem,dic(19;19)(q13.4;q13.4)[1]/90,idemx2,-6,+7,+11,dic(19;19)[1]/46,XX[1]. Surveillance magnetic resonance imaging (MRI) brain showed no intracranial or meningeal masses. Tissue specimens were referred to a sarcoma expert who identified it as an unusual variant of myxoid sarcoma that could not be classified. Chemotherapy was initiated with doxorubicin that she received every 21 days for 4 cycles. After one cycle of chemotherapy, she had a drastic improvement in her symptoms and physical appearance (Figure 4). Due to her poor performance status in addition to side effects from her prior regimen, her chemotherapy regimen was changed to gemcitabine. Unfortunately she continued to decline and ultimately opted for hospice care.