Extramedullary plasmacytoma immunoglobulin D (λ) in the chest wall and the subglottic region

Abstract

Clinical Summary A 47-year-old woman visited our hospital with chief symptoms of a fist-sized tumor and invariable pain in the left lateral pectoral region. The tumor was elastic and hard, with radiating pressure pain. The chest radiograph showed an ill-defined 7.0 5.0-cm mass shadow in the left lower lung field (Figure 1, A). The chest radiograph obtained during a mass chest radiograph survey about 10 months before the visit appeared normal. Chest computed tomography demonstrated a heterogeneously enhanced mass without destruction of the ribs in the left chest wall (Figure 1, B). Magnetic resonance imaging showed no osteolytic lesion in the ribs. Preoperative histopathologic diagnosis of the incisional biopsy sample was high-grade sarcoma. The patient was scheduled for a chest wall resection and reconstructive surgery. On the day of the operation, the anesthetist discovered a subglottic tumor while intubating the patient (Figure 2, A). There had been no symptoms of dyspnea or change of voice before the operation. After a tracheotomy, a chest wall dissection was performed, encompassing an area of 15.0 12.5 cm starting from the lower edge of the seventh rib to the upper edge of the 11th rib. The defect of the chest wall was reconstructed with polytetrafluoroethylene mesh and a skin flap. The excised chest wall tumor, measuring 9.0 7.3 5.0 cm, surrounded ribs. Examination of a permanent section revealed that the chest wall tumor was IgD( ) type plasmacytoma. Ki-67 proliferation index (the proportion of proliferating cells in the tumor) of the tumor showed a value of 40%. After the operation, M-protein was not detected in the blood or urine. Bone marrow examination showed normal findings. The results of a radiographic survey did not show any lesion. Although biopsy had been performed several times on the subglottic tumor, we had not been able to achieve a definite diagnosis. The subglottic tumor had regressed spontaneously by about 10 weeks after the chest wall operation (Figure 2, B ). Two months later, the patient was urgently readmitted to our hospital with the symptom of airway stenosis caused by rapid regrowth of the subglottic tumor that reached the vocal cord (Figure 2, C ). The subglottic tumor was identified as IgD( ) type plasmacytoma. Systemic resurvey did not show any other lesions. The subglottic tumor was treated with radiotherapy in doses of 50 Gy and one cycle of chemotherapy with vincristine, doxorubicin, and dexamethasone. The subglottic tumor has been well controlled (Figure 2, D ). The patient was successfully treated and shows no signs of recurrence or development of multiple myeloma 7 years after this treatment.