Case Report
Copyright ©The Author(s) 2025. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Oncol. Jan 24, 2025; 16(1): 96131
Published online Jan 24, 2025. doi: 10.5306/wjco.v16.i1.96131
Extramedullary plasmacytoma with the uvula as first affected site: A case report
Jing Yang, Hui Peng, Sheng-Ke Tu, Min Li, Kui Song
Jing Yang, Hui Peng, Sheng-Ke Tu, Kui Song, Department of Hematology, The First Affiliated Hospital of Jishou University, Jishou 416000, Hunan Province, China
Min Li, Department of Pharmacy, The First Affiliated Hospital of Jishou University, Jishou 416000, Hunan Province, China
Author contributions: Yang J, Tu SK, Li M, Peng H, and Song K conceived and designed the study, collected and interpreted all the relevant clinical and laboratory data, and prepared the manuscript; all authors read and approved the final manuscript; JY, Tu SK, Li M, Peng H, and Song K confirm the authenticity of all the raw data in this study.
Supported by Hunan Innovative Province Construction Special Task Book, No. 2021SK4050; and Innovation Platform and Talent Program of Hunan Province, No. 2023JJ30609.
Informed consent statement: Written informed consent was obtained from the patient for publication of this case report and the accompanying images.
Conflict-of-interest statement: All authors declare that they have no competing interests to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Kui Song, MD, Chief Doctor, Department of Hematology, The First Affiliated Hospital of Jishou University, 26 Century Avenue, Qianzhou, Jishou 416000, Hunan Province, China. js_hematology@163.com
Received: April 27, 2024
Revised: September 3, 2024
Accepted: September 23, 2024
Published online: January 24, 2025
Processing time: 185 Days and 18.1 Hours
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.

Keywords: Extramedullary plasmacytoma; Plasmacytoma; Uvula; Multiple myeloma; Case report

Core Tip: The high-risk characteristics of extramedullary plasmacytoma in clinical manifestations stem from its pathogenesis and the high-risk trait of cytogenetics. There are limited recommendations and guidelines for this type of tumor owing to the rarity of extramedullary plasmacytoma (EMP) of the uvula. Radiotherapy is the preferred treatment after diagnosis. Chemotherapy is ideal for cases with multiple recurrence, extensive local invasion, and poor histological differentiation. The current chemotherapy regimen can refer to the chemotherapy regimen for multiple myeloma. We report a rare case of EMP with the uvula as the first affected site; therefore, the relevant literature is reviewed to enhance clinicians' awareness of such rare comorbidities.