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头颈部髓外孤立性浆细胞瘤。一项临床病理研究。

Extramedullary solitary plasmacytoma of the head and neck. A clinicopathological study.

作者信息

Hotz M A, Schwaab G, Bosq J, Munck J N

机构信息

Department of Head and Neck Surgery, Institut Gustave-Roussy, Villejuif, France.

出版信息

Ann Otol Rhinol Laryngol. 1999 May;108(5):495-500. doi: 10.1177/000348949910800514.

Abstract

Monoclonal extramedullary plasmacytoma (EMP) is a rare, low-grade lymphoma found predominantly in the head and neck region. Only since the introduction of immunophenotyping techniques 2 decades ago has it been possible to differentiate EMP from benign polyclonal plasma cell proliferation. The purpose of this study was to trace the evolutionary profile of the disease under consideration of monoclonality assessment. The records of 24 patients with morphologically diagnosed EMP treated in a single institution underwent clinical and pathological review. Only 14 patients had true monoclonal plasmacytoma. No EMP-related deaths occurred. Two patients had local recurrence, and 2 patients developed multiple myeloma. Review of the literature confirms the low-grade malignancy of EMP. Diagnostic procedures must exclude benign polyclonal plasmacytoma, multiple myeloma, and solitary bone plasmacytoma. The slow natural progression of the disease and the rarity of secondary multiple myeloma favor nonmutilating local surgery whenever possible to avoid the long-term sequelae of radiotherapy.

摘要

单克隆髓外浆细胞瘤(EMP)是一种罕见的低度淋巴瘤,主要见于头颈部区域。仅在20年前引入免疫表型分析技术之后,才有可能将EMP与良性多克隆浆细胞增殖区分开来。本研究的目的是在考虑单克隆性评估的情况下追踪该疾病的演变情况。对在单一机构接受治疗的24例经形态学诊断为EMP的患者的记录进行了临床和病理复查。只有14例患者患有真正的单克隆浆细胞瘤。未发生与EMP相关的死亡。2例患者出现局部复发,2例患者发展为多发性骨髓瘤。文献回顾证实了EMP的低度恶性。诊断程序必须排除良性多克隆浆细胞瘤、多发性骨髓瘤和孤立性骨浆细胞瘤。该疾病自然进展缓慢且继发性多发性骨髓瘤罕见,因此只要有可能,就应选择不致残的局部手术,以避免放疗的长期后遗症。

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