关键词: case report extramedullary plasmacytoma multiple myeloma radiotherapy surgery

来  源:   DOI:10.3389/fonc.2024.1353943   PDF(Pubmed)

Abstract:
UNASSIGNED: Extramedullary plasmacytoma (EMP) is an uncommon solitary tumor originating from neoplastic plasma cells located outside the bone marrow. Despite its rarity, the occurrence of EMP without a concurrent diagnosis of multiple myeloma (MM) is considered extremely rare. Approximately 80-90% of EMP cases are found in the head and neck region, with a higher incidence in men aged between 50 and 60 years. The current treatment modalities include radiotherapy (RT) as a first-line approach, with surgery or chemotherapy regarded as other therapeutic options. While RT proves effective in the majority of EMP cases, there are instances where the tumor remains refractory to radiation. In this case report, we present an unusual scenario of EMP resistant to RT without concurrent signs of multiple myeloma which was successfully treated with surgery followed by systemic therapy.
UNASSIGNED: A 72-year-old male was admitted to the Head and Neck Cancer Clinic with a 6-month history of swallowing difficulties. He denied experiencing weight loss or pain on swallowing. Basic laboratory tests yielded results within normal limits, except for beta-2 microglobulin. Physical examination revealed an enlarged submandibular lymph node on the right side. Fiberoptic examination identified a soft tissue polypoid mass within the right piriform fossa, slightly protruding into the vocal slit. A CT scan displayed a well-circumscribed 2 cm polypoid, homogeneously enhancing soft tissue mass adjacent to the posterior surface of the epiglottis and the right side of the tongue base. Bone marrow biopsy revealed no abnormalities, and there were no clinical or laboratory signs of multiple myeloma. Based on the tumor biopsy results and imaging studies, a diagnosis of EMP was made. Due to the lack of response to RT, surgical removal of the tumor was pursued, followed by systemic therapy. Ultimately, the patient achieved full recovery with effective disease control.
UNASSIGNED: In conclusion, EMP without concurrent multiple myeloma is an exceedingly rare condition that demands a multidisciplinary approach for both diagnosis and treatment. Moreover, although RT continues to be the primary standard treatment for EMP, in some cases other therapeutic regimens prove to be successful.
摘要:
髓外浆细胞瘤(EMP)是一种罕见的孤立性肿瘤,起源于骨髓外的肿瘤浆细胞。尽管它很罕见,无多发性骨髓瘤(MM)并发诊断的EMP的发生被认为极为罕见.大约80-90%的EMP病例出现在头颈部,在50至60岁的男性中发病率较高。目前的治疗方式包括放疗(RT)作为一线方法,手术或化疗被视为其他治疗选择。虽然RT在大多数EMP病例中被证明是有效的,在某些情况下,肿瘤仍然难以接受辐射。在这个案例报告中,我们提出了一种不寻常的情况,即EMP对RT耐药,但没有并发多发性骨髓瘤的症状,该病例通过手术后进行全身治疗成功治疗.
一名72岁的男性因6个月的吞咽困难史被送往头颈部癌症诊所。他否认体重下降或吞咽疼痛。基本的实验室检查结果在正常范围内,除了β-2微球蛋白.体格检查显示右侧颌下淋巴结肿大。纤维检查发现右侧梨状窝有软组织息肉样肿块,稍微突出到声带狭缝中。CT扫描显示界限清楚的2厘米息肉,均匀增强邻近会厌后表面和舌根右侧的软组织块。骨髓活检未见异常,并且没有多发性骨髓瘤的临床或实验室征象。根据肿瘤活检结果和影像学检查,诊断为EMP.由于缺乏对RT的反应,手术切除肿瘤,其次是全身治疗。最终,患者通过有效的疾病控制实现了完全康复。
总而言之,没有并发多发性骨髓瘤的EMP是一种极为罕见的疾病,需要多学科的诊断和治疗方法。此外,尽管RT仍然是EMP的主要标准治疗方法,在某些情况下,其他治疗方案被证明是成功的。
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