INTRODUCTION: Primary pulmonary plasmocytomas are rarely reported tumors with average progression and treatment over multiple years. We describe a rapidly progressive case of the disease in which the patient responded to unorthodox chemotherapy treatment at late stage.
CASE PRESENTATION: A 77 year-old female presented to emergency room complaining of shortness of breath for two days. Patient had experienced intermittent episodes of dyspnea over past two weeks with associated band-like pain across her chest. Pain worsened with inspiration. Patient also complained of epigastric tenderness.Patient had no history of cough or fever. Medical history was significant for atrial fibrillation (on anticoagulation), hypertension, anemia, and rectal bleeding. She had no prior cardiac history. She had elective hip replacement surgery four months prior and had video-assisted thoracoscopy (VATS) for a benign pleural effusion one year prior. During this hospitalization, the patient’s vitals were: blood pressure 114/74 mm of Hg, heart rate 103/minute, respiratory rate 24/minute, afebrile, and saturating 98% on room air. Lung exam was significant for bilateral audible rhonchi.Chest x-ray showed multiple masses in right lung base (7.9 cm), right hilum (7.8 cm), and left mid-lung (5.2 cm). Serum laboratories showed abnormal serum protein (10.4 g/dL) and globulin content (6.8 g/dL) with low-grade chronic anemia.One year prior to this hospitalization, patient had normal chest x-ray, normal serum protein levels, mildly elevated serum globulin levels (4.5 g/dL), and no anemia. During an ER visit for shortness of breath two months prior to present hospitalization, patient’s chest x-ray showed a 4.1 cm density in the right lower lung field. Patient was subsequently lost to followup. During hospitalization, CT chest with contrast showed moderate bilateral pleural effusions, right pleural-based mass (3.7 cm) with associated rib fracture, left pleural-based mass (2.6 cm) with associated rib destruction, mass (10 cm) with destroyed thoracic vertebral body in subcarinal region, and multiple associated hematomas. Concurrently, patient was assessed for multiple myeloma based on clinical presentation and abnormal bloodwork.Four days into hospitalization, patient developed bilateral lower extremity weakness, secondary to metastatic disease. Pathology results from a CT-guided transcutaneous thoracic needle biopsy showed Plasma Cell Myeloma with positive Lambda antigen and positive “M” spike in Gamma region on serum protein electrophoresis (SPEP) consistent with Extramedullary Plasmacytoma (EP).Given the rapidly progressive metastatic plasmacytoma, patient was treated with four cycles of intravenous Bortezomib. Following chest x-ray showed rapidly shrinking thoracic tumors, but over the next few days, patient terminally succumbed to multiple organ failure secondary to tumor lysis syndrome and side effects of Bortezomib.
DISCUSSIONS: EP constitutes 5-10% of plasma cell tumors with 43% of these tumors originating in axillary, mesenteric, pelvic lymphnodes, stomach or pleura. Patients most commonly present with symptoms of cough, dyspnea, and fever. Hemoptysis has also been reported.Pulmonary Parenchynal Plasmocytoma (PP) is rarely reported (7 cases). Even though the biopsy specimen of our patient mainly contained rib tissue, we believe patient also had PP involvement because of absence of fever, non-resolution of lesions with antibiotic therapy, rapid resolution on chemotherapy, and radiographic appearance.Normally, the EP progresses slowly (mean survival of 115 months), allowing treatment with radiation and/or surgery. However, for the unusual rapidly progressive disease in our patient, we resorted to chemotherapy as a late-stage treatment.
CONCLUSION: Pulmonary Parenchynal Plasmocytoma is a rarely-reported tumor, and rapidly progressive disease cases have not been reported. In spite of the rapid disease progression, our patient did respond to the unorthodox chemotherapy treatment as documented by serial chest x-rays showing day-by-day tumor resolution.
DISCLOSURE: Sukriti Singhal, No Financial Disclosure Information; No Product/Research Disclosure Information