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鼻外浆细胞瘤:文献综述及一例罕见病例报告

Extramedullary nasal plasmacytoma: Literature review and a rare case report.

作者信息

Corvo Marco Antonio Dos Anjos, Granato Lídio, Ikeda Felipe, de Próspero José Donato

机构信息

MSc in Otorhinolaryngology. Assistant Professor, Department of Otorhinolaryngology, Santa Casa de Misericórdia de São Paulo.

PhD in Otorhinolaryngology, Federal University of São Paulo. Associate Professor, Department of Otorhinolaryngology, Irmandade da Santa Casa de Misericórdia de São Paulo.

出版信息

Int Arch Otorhinolaryngol. 2013 Apr;17(2):213-7. doi: 10.7162/S1809-97772013000200016.

Abstract

INTRODUCTION

Extramedullary plasmacytoma is a plasma cell tumor that grows within any of the soft tissues of the organism. Similar to all plasmacytic dyscrasia, the disease is extremely rare.

AIM

This study reports a case of an extramedullary plasmacytoma of the nasal cavity and provides a literature review on the topic.

CASE REPORT

A 51-year-old woman presented at our tertiary university hospital with a 6-month history of progressive nasal obstruction, predominantly to the right side, and self-limiting epistaxis. Examination revealed a large pale-reddish tumor within the right nasal cavity. Anatomopathological analysis showed features consistent with a plasmacytoma diagnosis, which was subsequently confirmed by immunohistochemical techniques. Further assessment revealed the solitary nature of the condition, consistent with extramedullary plasmacytoma. Radiotherapy was initiated, which led to partial regression of the symptoms. The tumor was surgically removed by using a mid-facial degloving approach. The patient evolved with a naso-oral fistula and underwent 3 corrective surgeries. No evidence of associated systemic disease was found after 5 years of follow-up.

CONCLUSIONS

This case report, which describes a rare tumor of the nasal cavity, is expected to improve the recognition and referral of this condition by ear, nose, and throat (ENT) specialists for multidisciplinary management and long-term follow-up.

摘要

引言

髓外浆细胞瘤是一种在机体任何软组织内生长的浆细胞瘤。与所有浆细胞发育异常一样,这种疾病极为罕见。

目的

本研究报告一例鼻腔髓外浆细胞瘤病例,并对该主题进行文献综述。

病例报告

一名51岁女性因进行性鼻塞6个月(主要为右侧)及自限性鼻出血就诊于我们的三级大学医院。检查发现右侧鼻腔内有一个大的淡红色肿瘤。解剖病理学分析显示特征与浆细胞瘤诊断相符,随后通过免疫组化技术得以证实。进一步评估显示该病情为孤立性,符合髓外浆细胞瘤。开始进行放疗,症状部分缓解。采用面中部掀翻术手术切除肿瘤。患者出现鼻-口瘘并接受了3次矫正手术。随访5年后未发现相关系统性疾病的证据。

结论

本病例报告描述了一种罕见的鼻腔肿瘤,有望提高耳鼻喉科(ENT)专家对该疾病的认识,并促使其进行多学科管理和长期随访的转诊。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2f39/4423260/5e95338d0429/10-7162-s-1809-97772013000200016-i170216-1.jpg

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