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腭部髓外浆细胞瘤的序贯治疗:一例罕见病例报告及七年随访,并文献复习。

Sequential therapy for extramedullary plasmacytoma of the palate: a rare case report with seven years of follow-up and literature review.

机构信息

Department of Oral and Maxillofacial Surgery, Shandong Provincial Hospital Affiliated to Shandong First Medical University, No.324, Jing Six Road, Jinan, Shandong, 250021, China.

Department of Stomatology, Zibo First Hospital, Zibo, Shandong, 255200, China.

出版信息

J Cancer Res Clin Oncol. 2024 Sep 28;150(9):431. doi: 10.1007/s00432-024-05958-1.

Abstract

BACKGROUND

Extramedullary plasmacytoma (EMP) is a rare solitary malignancy that accounts for 3% of plasma cell neoplasms, and EMP with a primary occurrence in the palate is extremely uncommon. Owing to the long course of EMP and the limited available data on treatment outcomes, there are no definitive guidelines for its management, especially for high-risk patients who are more susceptible to early progression to multiple myeloma.

CASE PRESENTATION

In this study, we review nine relevant studies and describe a 54-year-old woman who presented with an asymptomatic nonulcerative mass localized in the palate. After initial radical surgical resection of the lesion, the patient was definitively diagnosed with EMP with minimal plasmacytosis in the bone marrow, and adjuvant intensity-modulated radiation therapy with a minimum dose of 39.6 Gy was administrated in the surgical area. There was no evidence of local recurrence, nodal metastasis or progression to multiple myeloma (MM) during the seven-year follow-up period.

CONCLUSION

Given the atypical clinical features of palate EMP reported in the literature and the encouraging results of our patient, sequential therapy involving surgery and adjuvant radiotherapy for primary palatal lesions in high-risk EMP patients without nodal involvement could be an effective treatment modality.

摘要

背景

髓外浆细胞瘤(EMP)是一种罕见的孤立性恶性肿瘤,占浆细胞瘤的 3%,原发于腭部的 EMP 极为罕见。由于 EMP 的病程较长,且治疗结果的可用数据有限,因此其管理尚无明确指南,特别是对于更容易早期进展为多发性骨髓瘤的高危患者。

病例介绍

本研究回顾了 9 项相关研究,并描述了一名 54 岁女性患者,其表现为无症状的非溃疡性腭部局限性肿块。病变初始行根治性手术切除后,患者被明确诊断为 EMP,骨髓中浆细胞增生程度轻微,在手术区域给予至少 39.6 Gy 的强度调制放疗作为辅助治疗。在 7 年的随访期间,患者未出现局部复发、淋巴结转移或进展为多发性骨髓瘤(MM)。

结论

鉴于文献中报道的腭部 EMP 的非典型临床特征以及我们患者的良好结果,对于无淋巴结受累的高危 EMP 患者的原发性腭部病变,采用手术和辅助放疗序贯治疗可能是一种有效的治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3dcf/11793532/9d4a18ec64c8/432_2024_5958_Figa_HTML.jpg

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