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Extramedullary Plasmacytoma Of The Nasal Cavity

机译:鼻腔的髓外浆细胞瘤

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Extramedullary Plasmacytomas(EMP) are rare tumors accounting for 0.4% of all head and neck malignancies and 4% of all nonepithelial tumors of nasal cavity. The absence of multiple myeloma must be confirmed with bone marrow examination and skeletal surveys, before arriving at a diagnosis of EMP. We report a case of extramedullary plasmacytoma of the nasal cavity in a 60 year old female, who was a chronic tobacco sniffer. The case demonstrates the multidisciplinary approach required for the optimal diagnosis and management of such tumors and proposes chronic irritation of the nasal mucosa as one of the etiological factors for EMP. Introduction Extramedullary Plasmacytomas(EMP) are rare tumors accounting for 0.4% of all head and neck malignancies and 4% of all nonepithelial tumors of nasal cavity.1 About 80% of the extramedullary plasmacytomas are localized in the submucosa of the upper respiratory tract, with a large proportion occurring in the sinonasalasopharyngeal area.1-3 The absence of multiple myeloma must be confirmed with bone marrow examination and skeletal surveys ,before arriving at a diagnosis of EMP. The therapy consists of combined surgery and radiotherapy. The main prognostic indicator for EMP is progression to multiple myeloma.10-32% of all patients develop multiple myeloma which reduces the mean survival time from 8.3 years to 20 months .1,4 We report a case of EMP of the nasal cavity in a 60 year old female who was a chronic tobacco sniffer. The patient was managed by endoscopic removal of the tumor followed by radiotherapy. A 6 month follow up of the patient revealed neither recurrence of the tumor nor a progression to multiple myeloma. Case Report A 60 year old female, with unremarkable past medical history, presented with right sided nasal obstruction, mucoid nasal discharge and intermittent epistaxis for the past 5 months. There was history of occasional headaches .The patient had been sniffing tobacco for the past 35 years. The general physical examination did not reveal any abnormality, nor was there any evidence of lymphadenopathy. Anterior rhinoscopy revealed a pinkish, fleshy mass in the right nasal cavity which was firm in consistency, bled easily and was insensitive to probing. Endoscopic examination confirmed the presence of a dark red sub mucosal lesion with a smooth surface. The CT scan of the patient revealed a hypodense mass localized to the right nasal cavity showing contrast enhancement with no bony erosion (figure 1 ).A nasal biopsy was subsequently taken which revealed a dense infiltrate of plasma cells ( figure 2 ). A histological diagnosis of plasmacytoma was made. Routine blood tests and serum biochemistry including calcium, phosphorus, blood urea nitrogen, urea, uric acid and creatinine were all within reference range. Both serum myeloma proteins and urine Bence- Jones proteins were negative. Serum and urine electrophoresis did not reveal M component. Bone marrow biopsy revealed a plasma cell infiltrate of less than 3% of all nucleated cells .Chest radiograph, total body skeletal survey and Tc99 scintigram, showed no systemic lesion. The finding confirmed a diagnosis of stage 1 extramedullary plasmacytoma. The patient underwent complete surgical removal of the tumor endoscopically. This was followed by radiotherapy of 5000 cGy, delivered over a period of 6 weeks. A 6 month follow- up of the patient did not reveal any recurrence.
机译:髓外浆细胞瘤(EMP)是罕见的肿瘤,占所有头颈部恶性肿瘤的0.4%,占所有鼻腔非上皮性肿瘤的4%。在诊断为EMP之前,必须通过骨髓检查和骨骼检查来确认是否存在多发性骨髓瘤。我们报道了一名60岁女性的鼻腔髓外浆细胞瘤病例,该女性为慢性吸烟者。该病例证明了对此类肿瘤进行最佳诊断和处理所需的多学科方法,并提出对鼻粘膜的慢性刺激是EMP的病因之一。前言髓外浆细胞瘤(EMP)是罕见的肿瘤,占所有头颈部恶性肿瘤的0.4%,占鼻腔所有非上皮肿瘤的4%。1约80%的髓外浆细胞瘤位于上呼吸道粘膜下层, 1-3在诊断为EMP之前,必须通过骨髓检查和骨骼检查确认没有多发性骨髓瘤。该疗法包括手术和放射疗法的结合。 EMP的主要预后指标是进展为多发性骨髓瘤。所有患者中有10-32%会发展为多发性骨髓瘤,这将平均生存时间从8.3年缩短至20个月。1,4我们报告了1例鼻腔EMP病例。 60岁的女性,她是一名慢性吸烟者。通过内窥镜切除肿瘤,然后进行放射治疗来治疗该患者。对患者进行的6个月随访未显示肿瘤复发或进展为多发性骨髓瘤。病例报告一名60岁女性,既往病史不明显,在过去5个月内出现右侧鼻塞,鼻粘液样分泌物和间歇性鼻epi。有偶尔头痛的病史。患者在过去的35年中一直在闻烟。总体身体检查未发现任何异常,也未发现任何淋巴结肿大的迹象。前鼻腔镜检查发现右鼻腔有粉红色的肉质肿块,结实牢固,容易流血,对探测不敏感。内窥镜检查证实存在具有光滑表面的暗红色粘膜下病变。患者的CT扫描显示低密度肿块位于右鼻腔,显示对比度增强,无骨侵蚀(图1)。随后进行了鼻腔活检,发现浆细胞密集浸润(图2)。进行浆细胞瘤的组织学诊断。常规血液检查和血清生化包括钙,磷,血尿素氮,尿素,尿酸和肌酐均在参考范围内。血清骨髓瘤蛋白和尿液Bence-Jones蛋白均为阴性。血清和尿液电泳未显示M成分。骨髓活检显示浆细胞浸润少于所有有核细胞的3%。胸片,全身骨骼检查和Tc99闪烁图显示无全身病变。该发现证实了1期髓外浆细胞瘤的诊断。内窥镜对患者进行了彻底的手术切除。随后进行为期6周的5000 cGy放射治疗。对患者进行的6个月随访未发现任何复发。

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