Cantone Elena, Di Lullo Antonella Miriam, Marano Luana, Guadagno Elia, Mansueto Gelsomina, Capriglione Pasquale, Catalano Lucio, Iengo Maurizio
Department of Neuroscience, Reproductive and Odontostomatologic Science, ENT Section, "Federico II" University of Naples-Italy, S. Pansini, 5, 80131, Naples, Italy.
Università degli Studi di Napoli "Federico II", Via Pansini, 5, Naples, Italy.
J Med Case Rep. 2017 Aug 10;11(1):219. doi: 10.1186/s13256-017-1382-4.
Extramedullary plasmacytoma is a rare neoplasm characterized by monoclonal proliferation of plasma cells outside bone marrow. It accounts for 4% of all non-epithelial sinonasal tumors. According to the literature, radiotherapy is the standard therapy for extramedullary plasmacytoma. However, the conversion rate of extramedullary plasmacytoma to multiple myeloma is reported to be between 11 and 33% over 10 years. The highest risk of conversion is reported during the first 2 years after diagnosis, but conversion has been noted up to 15 years after diagnosis. Once conversion to multiple myeloma is complete, less than 10% of patients will survive 10 years.
We present three cases of sinonasal extramedullary plasmacytoma who underwent radiotherapy: a 61-year-old white man, a 60-year-old white man, and a 37-year-old white woman. We found long-term survival with stable disease in all three cases.
The management of solitary extramedullary plasmacytomas of the sinonasal tract is not well established yet. However, the possibility of recurrence and progression to multiple myeloma requires a thorough follow-up protocol. Due to the absence of a standardized protocol for these tumors, we tried to design a tailored long-term follow-up scheme.
髓外浆细胞瘤是一种罕见的肿瘤，其特征是骨髓外浆细胞的单克隆增殖。它占所有非上皮性鼻窦肿瘤的4%。根据文献，放射治疗是髓外浆细胞瘤的标准治疗方法。然而，据报道，髓外浆细胞瘤在10年内转化为多发性骨髓瘤的转化率在11%至33%之间。据报道，诊断后前2年转化风险最高，但诊断后长达15年也有转化的情况。一旦完全转化为多发性骨髓瘤，不到10%的患者能存活10年。
我们报告了3例接受放射治疗的鼻窦髓外浆细胞瘤病例：1名61岁白人男性、1名60岁白人男性和1名37岁白人女性。我们发现所有3例患者均长期存活且病情稳定。
鼻窦孤立性髓外浆细胞瘤的治疗方法尚未完全确立。然而，复发和进展为多发性骨髓瘤的可能性需要完善的随访方案。由于这些肿瘤缺乏标准化方案，我们试图设计一个定制的长期随访方案。
