A 32-year-old female presented to emergency department with worsening shortness of breath and fatigue. Patient was 1 week postpartum, and while she was pregnant, it was noted that she had left-sided pleural effusion which was drained. Pleural effusion was bloody and culture and cytology were negative at that time. Decision was made to follow up the patient postpartum. Patient ended up presenting to the emergency room (ER) on two separate occasions during pregnancy complaining of shortness of breath, and each time it was noted then that she had recurrent pleural effusions.
After delivery, patient presented again complaining of shortness of breath and fatigue. On examination, vitals were as follows: temperature, 36.8 C; pulse, 93 bpm; respiratory rate, 18/min; blood pressure, 119/81 mmHg; O2 sat, 95% on room air. Chest examination revealed absent breath sounds on left lung base on auscultation. Rest of the physical examination was unremarkable. Following evaluation in the ER, she was found to have recurrence of large left-sided pleural effusion on CXR (Figure 1), so she was admitted on the floor for further evaluation and treatment. Patient did not have any headache, chest pain, palpitations, cough, hemoptysis or weight loss. She also denied any lumps or mass anywhere on her body. Also there was no history of musculoskeletal deformity or pains. Past medical history was significant for gastroesophageal reflux disease (GERD), asthma and depression. The only medications she was on were off and on ibuprofen, prenatal vitamins and zantac. Patient denied use of alcohol and illicit drugs but did have a history of cigarette smoking for 17 years. Family history was positive for breast cancer in sister and diabetes and hypertension in father. There was no history of tuberculosis and exposure of asbestos.
A pigtail catheter was placed for drainage of left pleural effusion. Pleural fluid was again bloody and repeat pleural fluid analysis did not reveal any infectious or malignant etiology. Computed tomography (CT) scan of chest with contrast showed large partly loculated left pleural effusion increasing in volume as compared to previous CT chest done 1 month ago (Figure 2). Diffuse ground-glass opacities and non-specific mediastinal and hilar lymphadenopathy were also seen (Figure 2). Patient was started on empirical levofloxacin and vancomycin on the suspicion of possible pneumonia. Sputum, blood and pleural fluid cultures were all negative. In the mean time, patient developed acute blood loss anemia due to non-resolving hemorrhagic pleural effusions for which multiple blood transfusions were given. During the course of hospital stay, patient developed worsening atelectasis of left lung with large hemothorax (Figure 3). In the setting of above findings, a suspicion of left lung mass obstructing the bronchus was made and patient was scheduled for left video-assisted thoracoscopic (VATS) exploration for hemothorax evacuation and biopsy of possible mass. During the procedure, the left lobe appeared to be completely collapsed with a large tumor appearing to compress and occupy the entire left lower lobe space. Due to the size, extent and location of the tumor being unknown and both the previous imaging scans being negative for a tumor, the procedure was converted to posterolateral thoracotomy as determining the extent and the type of this lesion was of utmost priority. A stat frozen biopsy was taken from the tumor and sent for pathologic examination, which confirmed the specimen was consistent with sarcoma but was not able to definitively define it without further testing with an additional specimen, which was provided. Significant vascularity was noted and the tumor appeared to be bleeding even with partial manipulation. Due to the large size of the tumor, extension of the tumor into mediastinum as well as the preliminary diagnosis, the decision was made not to proceed with any further attempts at resection due to low chances of successfully completely resecting the tumor and the potential for significant bleeding and complications. Chest cavity was evacuated and hemostasis was achieved.
Pathology results confirmed the diagnosis of high-grade myxoid lung sarcoma (Figure 4). Immunohistochemical staining was positive for Vimentin and negative for S100, desmin, keratin, MUC4 and CD34. CT of abdomen and pelvis and skeletal survey of extremities were done to find any primary sarcoma, but all radiological investigations were normal. Patient was counseled about the inoperable nature of tumor, and discussion of chemotherapy and radiotherapy as possible alternatives was made with the patient. She was started on multi-drug chemotherapy and regularly followed up with an oncologist. After 1 month, the patient presented again with shortness of breath, nausea, vomiting and fever. Repeat CT chest was done which showed progressive mediastinal shift to the right and interval development of nodular hypertrophy or pleural thickening along the left major fissure, suggesting progression of malignant disease (Figure 5). There was also mass effect on heart and trace pericardial effusion (Figure 5).