Abstract

Extramedullary plasmacytoma (EMP) is a rare variant of plasma cell myeloma that affects soft tissues. The head and neck region are the most affected sites, although others have also been described. Herein we report an uncommon case of EMP of the larynx in a 65-year-old male who presented with a history of progressive dysphonia and hoarseness. Laryngeal fiberscopy evidenced a reddish pedicled voluminous mass in the left false cords and ventricle. Microscopic suspension laryngoscopy was performed under general anaesthesia and a 4 W Acublade CO2 Laser was used for transoral resection of the lesion. This was followed by adjuvant radiotherapy, with the widely recommended doses on the supraglottic region, to achieve better local control. Diagnosis of EMP is based on immunohistochemistry and the exclusion of systemic plasma cell proliferative disorders. Diagnosis of solitary EMP can be made only if studies for disseminated disease and X-ray and/or magnetic resonance imaging of the spine, pelvis, femurs, and humerus and bone marrow biopsy are negative. As there are no internationally established guidelines, treatment of EMP is mainly based on consensus of expert opinion.

Highlights

  • Plasma cell neoplasms represent a spectrum of diseases ranging from benign conditions, such as monoclonal gammopathy, to malignant entities, such as plasma cell myeloma and plasma cell leukemia [1]

  • extramedullary plasmacytoma (EMP) of the larynx is rare, it is on the increase, with 4.5% to 18% of EMP of the head and neck occurring in the larynx [7]

  • The clinical presentation varies depending on the organ involved [8], EMP of the larynx often presents with hoarseness and/or dysphagia [7]

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Summary

Introduction

Plasma cell neoplasms represent a spectrum of diseases ranging from benign conditions, such as monoclonal gammopathy, to malignant entities, such as plasma cell myeloma and plasma cell leukemia [1]. Plasma cell myeloma (multiple myeloma (MM)), a neoplastic proliferation of plasma cells [2], is the most common plasma neoplasm and is characterized by the involvement of the bone marrow at multiple sites [3]. A solitary plasmacytoma is a single localized mass of neoplastic plasma cells occurring in either bone (medullary) or soft tissue (extramedullary). The localized variant may be either the first evidence of generalized myeloma or an independent solitary lesion, characterized by the production of monoclonal immunoglobulins detectable in the serum and/or urine [2]. Extramedullary plasmacytomas have been reported in various sites in the body, such as the airway passages, gastrointestinal tract, and soft tissues [5]. About 80% of extramedullary plasmacytomas occur in the head and neck region [6], mainly in the nasal cavity, paranasal sinuses, or nasopharynx [4], whilst EMP of the larynx is quite rare

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