Extramedullary plasmacytoma with the uvula as first affected site: A case report

Abstract

BACKGROUND Extramedullary plasmacytoma (EMP) represents one of the rarer forms of plasma cell malignancies, capable of impacting a variety of tissues and organs throughout the body. The majority of EMP cases are predominantly found in the head and neck region, especially within the laryngopharynx, as well as in the gastrointestinal tract. While there have been documented instances of oropharyngeal involvement in EMP cases in the academic literature, it is important to note that EMP specifically affecting the uvula is exceedingly uncommon. Furthermore, it is noteworthy that over 60% of epithelial carcinomas in the upper respiratory tract and oropharynx tend to metastasize to the cervical lymph nodes, indicating a propensity for regional spread in these types of cancers. In this context, we present a rare case of extramedullary plasmacytoma where the uvula served as the initially affected site. This case emphasizes the need for heightened awareness among clinicians regarding such unusual comorbidities, as early recognition and diagnosis can significantly influence patient management and treatment outcomes. In addition, a review of the relevant literature is included to further educate and inform healthcare professionals about this rare presentation, ultimately aiming to enhance clinical understanding and improve patient care in similar situations. CASE SUMMARY A 51-year-old man was admitted to our hospital because of a slowly enlarging neck mass. A physical examination revealed a palpable left lymph node, and magnetic resonance imaging (MRI) of the oropharynx and the neck showed a soft tissue mass in the oropharynx and enlargement of multiple lymph nodes in the neck. The soft tissue mass was diagnosed as plasmacytoma by immunohistochemical analysis. Monoclonal immunoglobulins and bone marrow biopsy showed normal results. Therefore, we diagnosed that as EMP of the uvula. After four cycles of adjuvant chemotherapy dominated by bortezomib, MRI reexamination showed a significant reduction of the mass in the oropharynx and the cervical lymph nodes. Afterwards, the λ light chain returned to normal levels. There was no evidence of evolution to multiple myeloma. CONCLUSION We have reported a rare case of extramedullary plasmacytoma with the uvula as the first affected site and the relevant literature is reviewed to improve clinicians' awareness of such rare comorbidities.