Effect of definitive radiotherapy on the long‐term outcome in patients with solitary extramedullary plasmacytoma

Extramedullary solitary plasmacytoma (EMP) is a rare type of malignancy. This paper presents a retrospective review of the experience with EMP at the Krakow Oncology Center. Records of 17 patients with head and neck EMP, treated with definitive radiotherapy between 1976 and 2009, were analyzed. The total tumour dose ranged from 45 to 70 Gy (median 56 Gy). In four patients with partial response after radiotherapy, adjuvant melphalan‐based chemotherapy was applied. The median follow‐up period was 8.6 years. The treatment was well tolerated. The estimated 10‐year overall survival, disease‐free survival, and multiple myeloma‐free survival were 68.4%, 49.3%, and 55%, respectively. The 10‐year local control rate was 90.9%. No in‐field local recurrence was observed. During the follow‐up, progression into multiple myeloma was observed in five patients, with a mean time to conversion of 24 months. The only factor adversely affecting overall survival on univariate analysis was the age >56 years, whereas a complete tumour regression after radiotherapy was associated with a significant improvement in both disease‐free survival and multiple myeloma‐free survival. Despite the high effectiveness of local radiotherapy, there is still a significant treatment failure risk due to the EMP conversion into generalized disease. An attempt to identify prognostic factors may facilitate selection of patients with a high risk of progression to multiple myeloma. Copyright © 2015 John Wiley & Sons, Ltd.     

孤立性浆细胞瘤预后相关因素探究*

目的：探究孤立性浆细胞瘤患者的临床特点、治疗方法及预后相关因素。方法：搜集2000年6 月至2012年10月就诊于天津医科大学肿瘤医院的644 例恶性浆细胞肿瘤患者的临床资料,并对其临床特点、治疗方案、预后因素等进行分析,其中孤立性浆细胞瘤（solitary plasmacytoma ,SP）患者有66例占10.25% 。结果：根据SP的发生部位可分为骨孤立性浆细胞瘤（solitary bone plasmacytoma ,SBP ）和髓外浆细胞瘤（extramedullary plasmacytoma ,EMP ）。 SBP 大部分发生在椎体,而髓外浆细胞瘤好发于上呼吸道。此外,两者在肿块大小、血清M 蛋白水平、尿本周氏蛋白水平以及是否更易进展为多发性骨髓瘤（multiple myeloma,MM）等方面的差异均具有统计学意义（P < 0.05）。 多因素分析结果表明,SBP 患者的肿块越大（d≥ 5 cm）,其肿瘤的局部控制率（local control ,LC）、无骨髓瘤进展生存期（multiple myeloma-freesurvival,MMFS）、总生存期（overall survival,OS）及无病进展生存期（progression-free survival ,PFS）越差;对于EMP 患者来说,放以及血清β 2- 微球蛋白< 3.5 mg/L 是预后良好的因素。结论：肿块大小、血清M 蛋白、尿本周氏蛋白、血清β 2- 微球蛋白水平可为临床判断SBP 与EMP 预后及指导治疗提供帮助。     

A case report of tracheal extramedullary plasmacytoma

Introduction Extramedullary plasmacytoma (EMP) is an uncommon tumor that often develops outside the bone and arises from clonal proliferation of atypical plasma cells before EMP is diagnosed. Multiple myeloma(MM) must be excluded by performing laboratory tests such as serum protein electrophoresis, bone marrow biopsy and skeletal imaging ex-aminations. A bone marrow biopsy should show no evidence of mul-tiple myeloma, and less than 3% of plasma cells. Monoclonal bands of serum protein and Bence-Jones protein in the urine can sometimes be detected. EMP can involve any extraosseous organs, but it pre-dominantly affects the head and neck areas. Any extra-osseous organ may also be involved[1,2]. Tracheal involvement is a rare finding. Only a few cases of primary tracheal extramedullary plasmcytoma have been reported[2-4], Here we present a rare case of truly localized tra-cheal extram edullary plasmacytoma without evidence of myelomaelsewhere.     

髓外浆细胞瘤的临床病理特征及诊疗进展

在浆细胞瘤中,多发性骨髓瘤（MM）常见,而髓外浆细胞瘤（EMP）相对罕见,易误诊为其它肿瘤。本文对EMP的临床病理特点、生物学行为、诊断、治疗和预后等问题及其研究进展作一综述。     

50例孤立性浆细胞瘤临床分析

目的对孤立性浆细胞瘤(SP)的特点和预后因素进行回顾性分析,以帮助临床诊断、治疗及对预后的判断。方法入组50例SP患者,并对其进行随访,应用Kaplan-Meier法对其临床特点、治疗及预后因素进行回顾性分析。结果髓外浆细胞瘤(EMP)组与骨孤立性浆细胞瘤(SPB)组、放疗组与未放疗组、年龄﹤60岁与年龄≥60岁组的log rank检验结果提示,生存曲线差异均无统计学意义(P均>0.05)。50例SP患者与168例多发性骨髓瘤(MM)患者的生存曲线差异有统计学意义(P<0.05)。结论 SP患者中,EMP与SPB的生存状况无明显差异,SP好发于中老年人,预后较好,部分可转化为MM。     

Imaging features of extramedullary plasmacytoma

Extramedullary plasmacytomas are rare and can occur at any site. The imaging results are often nonspecific. This report highlights the radiographic, sonographic and cross‐sectional imaging features of extramedullary plasmacytomas.     

Solitary bone plasmacytoma and extramedullary plasmacytoma

Opinion statement Solitary bone and extramedullary plasmacytomas are rare plasma cell proliferative disorders. Their diagnosis is based on histologic confirmation of monoclonal plasma cell infiltration of a single disease site and on the exclusion of systemic myeloma. For both entities, the treatment of choice is localized radiotherapy. With modern radiotherapy and with a total dose of at least 4000 cGy, the risk for local recurrence is less than 5%. There is no role for systemic chemotherapy in the management of these disorders. Approximately 30% of patients with solitary bone plasmacytoma (SBP) remain disease-free for several years; some of these patients may be cured. Patients with the best prognosis are those in whom the monoclonal protein disappears by 1 year after radiotherapy. The prognosis of patients with solitary extramedullary plasmacytoma (SEP) appears to be better than for patients with SBP because approximately 70% of patients with SEP remain disease-free at 10 years. With more sensitive staging procedures, the diagnosis of SBP and SEP may become less common, but the number of patients with prolonged stability and cure may increase.     

SOLITARY PLASMACYTOMA OF BONE AND EXTRAMEDULLARY PLASMACYTOMA

ＳＯＬＩＴＡＲＹＰＬＡＳＭＡＣＹＴＯＭＡＯＦＢＯＮＥＡＮＤＥＸＴＲＡＭＥＤＵＬＬＡＲＹＰＬＡＳＭＡＣＹＴＯＭＡＹａｎｇＤｉｓｈｅｎｇ杨迪生；ＦａｎＳｈｕｎｗｕ范顺武；ＴａｏＨｕｉｍｉｎ陶惠民；ＨｅＲｏｎｇｘｉｎ何荣新；ＹｅＺｈａｏｍｉｎｇ叶招明；Ｚｈ...     

Solitary plasmacytoma of bone and extramedullary plasmacytoma

A small proportion of patients with plasma cell myeloma have a solitary plasmacytoma of bone. Strict staging criteria, including normal MR imaging studies of the axial skeleton and the long bones and absence of monoclonal plasma cells detected by flow cytometry or PCR, are required for diagnosis. Radiotherapy at a dose of 4500 cGy is required to eradicate the local tumor. Many patients enjoy prolonged disease-free survival, but the incidence of systemic relapse is high. It is expected, however, that if strict diagnostic criteria are applied some patients may be cured. Extramedullary plasmacytoma is an even rarer plasma cell disease which usually occurs in the head and neck area. Careful microscopic and immunohistochemical studies are required for the correct diagnosis, because this disease can be confused with other malignancies, particularly lymphomas. The treatment of choice is radiotherapy which, in cases of head and neck plasmacytomas, should encompass the adjacent lymph nodes. Most patients with extramedullary plasmacytoma can be cured, and fewer than 30% develop a distant failure in the form of multiple myeloma or multiple extramedullary tumors.     

Pancoast syndrome caused by extramedullary plasmacytoma

A 61-year-old patient had the clinical findings of Pancoast syndrome with shoulder pain, weakness and atrophy of band muscles, and an opacified area in the apical lung field. Unlike most cases with Pancoast syndrome in which the tumors are primary carcinomas of the lung, the present case was shown to have a plasmacytoma involving the apical parietal pleura and the adjacent chest wall. The need for histologic diagnosis before treating patients with Pancoast syndrome is emphasized.     

Solitary extramedullary plasmacytoma - larynx

Solitary Plasmacytoma of the larynx is a rare tumor with male predominance and most patients are between 50-70 years of age. The paper reports a case of Solitary Extramedullary’ Ipasmacytoma. (EMP) ljirynx in subglottic region, in a 65-year-old man successfully treated with complete response to radiotherapy.     

Early extramedullary plasmacytoma confined to the lamina propria of the gastric mucosa: case report.

A case of early gastric plasmacytoma in a 51-year-old woman is reported. The demonstration of monotypic IgM-kappa immunoglobulin in biopsy specimens by an immunoperoxidase technique contributed to the histological diagnosis. The patient had non-ulcerative lesions (of approximately 15 x 16 cm) of a superficially spreading type of gastric plasmacytoma. Extensive histological analysis of the resected stomach showed the IgM-kappa monoclonal proliferation of plasmacytic tumor cells to infiltrate merely into the lamina propria of the gastric mucosa and not to extend deeply into the submucosa, proper muscle and serosa of the stomach.     

髓外浆细胞瘤临床分析

目的探讨髓外浆细胞瘤的生物学行为和临床特点,以帮助临床诊断、治疗及对预后的判断。方法对40多年来笔者所在科室收治的28例髓外浆细胞瘤进行回顾性分析,其中单纯放射治疗16例(57.1%),综合治疗11例(39.3%),单纯手术1例(3.6%)。结果中位随访90个月(4～280个月),2例(7.1%)局部复发,2例(7.1%)转化为多发性骨髓瘤。3、5、10年总生存率分别为92.6%、75.6%和75.6%;单纯放射治疗组与综合治疗组3、5、10年总生存率分别为93.8%、77.3%、77.3%和90.9%、72.7%、72.7%(P>0.05)。结论单纯放射治疗是治疗髓外浆细胞瘤最有效的方法之一,局部放射治疗DT40Gy～50Gy后,可获得良好的局部控制率和长期生存率。部分髓外浆细胞瘤可向多发性骨髓瘤转化,故需密切观察。     

Solitary plasmacytoma of bone and soft tissue

Between 1962 and 1978, 15 patients presenting with a solitary plasmacytoma were treated with curative intent by radiotherapy alone at the University of Florida. Criteria for admission to this study were: 1) a biopsy-proven, apparently solitary focus of plasmacytoma; 2) bone marrow biopsy showing less than 10% plasma cells; and 3) no evidence of disseminated disease. In 9 patients the primary site was osseous and in 6 patients extramedullary; 5 of the 6 extramedullary lesions were located in the upper respiratory passages. Two of the 6 extramedullary plasmacytomas progressed to multiple myeloma at 2 and 7 months. The remaining 4 patients have been disease free for periods ranging from $\text{2}\text{2}\text{3}$ years to over 16 years. Of the 9 patients with osseous lesions, 3 developed multiple myeloma in 3–23 months and one developed a solitary second bone lesion at 9 years. One patient with a large sacral lesion developed a local recurrence following an initial radiation dose of 3000 rad. The recurrence was re-treated with radiation, and local control was obtained. No other local failures occurred. This study presents a detailed analysis of the time-dose relationship required for local control, based on a study of our own patients and a review of the literature.     

孤立性浆细胞瘤2例报道并文献复习

目的探讨孤立性浆细胞瘤(SP)的诊断和治疗。方法分析2例SP病例的临床资料。结果1例SPB已随访2年,无局部复发及远处播散。1例EMP经术后放疗,局部疗效达CR,现存活。结论单纯放疗是治疗SP最有效的方法之一,局部放疗DT40Gy~50Gy可获得良好的局控率。SP可向多发性骨髓瘤转化,故需密切随访观察。     

1例锁骨近端孤立性骨浆细胞瘤的临床报告

孤立性骨浆细胞瘤是一种以浆细胞单克隆异常增殖为特征的恶性肿瘤。临床上主要以单一骨破坏常见,约占所有浆细胞恶性肿瘤的5%。发生于锁骨近端的孤立性骨浆细胞瘤临床上少见,诊断较困难且容易发生误诊误治。     

Solitary bone plasmacytoma: outcome and prognostic factors following radiotherapy

Purpose: To clarify the natural history of solitary plasmacytoma of bone (SBP) after radiation treatment.

Methods and Materials: Between 1965–1996, we identified 57 previously untreated patients with a SBP. A serum myeloma protein was present in 33 patients (58%) and Bence Jones proteinuria was present in an additional eight patients (14%). The median radiotherapy dose was 50 Gy (range, 30–70 Gy). Overall survival, cause-specific survival, and freedom from progression to multiple myeloma were calculated actuarially.

Results: Local control was achieved in 55 of 57 patients (96%). For those 29 patients (51%) who subsequently developed multiple myeloma, the median time to progression was 1.8 years. There was a direct correlation between persistence of abnormal protein following radiotherapy and the likelihood of developing multiple myeloma. Among 11 patients with disappearance of myeloma protein, only two developed multiple myeloma after 4 and 12 years, in contrast to progression in 57% of patients with a persistent protein peak and 63% of those with nonsecretory disease (p = 0.02). Among 23 patients with thoracolumbar spine disease, 7 of 8 patients staged with plain radiographs alone developed multiple myeloma in comparison with 1 of 7 patients who also had magnetic resonance imaging (MRI) (p = 0.08). For all patients, the median survival from radiotherapy was 11.0 years. The median cause-specific survival of patients with disappearance of myeloma protein was significantly longer than that of the remaining patients (p = 0.004).

Conclusion: Results supported the importance of precise staging that includes MRI of the spine for optimum patient selection and the application of definitive radiotherapy. Those patients with myeloma protein that disappears following radiotherapy represent a category with a high likelihood of cure.     

Plasmacytoma of the parotid gland

Extramedullary plasmacytoma of the parotid gland is an extremely rare lesion of which there have been only four other reported cases. The importance of these lesions rests in the possibility of their being a harbinger of multiple myeloma or their subsequent transformation to multiple myeloma. Because of its extreme rarity, the proper management remains unclear.     

Solitary extramedullary plasmacytoma in the oropharynx: advantages of intensity-modulated radiation therapy

We report a case of a 59-year-old man with solitary extramedullary plasmacytoma in his oropharynx. Because the diagnosis is rare and there is only limited experience in the literature based on retrospective data, the optimal planning target volume and optimal dose of radiation therapy (RT) are still controversial. The frequently discussed problem is the necessity of first echelon lymph node irradiation because it is associated with a higher rate of complications such as xerostomia caused by damage to salivary glands. In order to prevent late toxicity, intensity-modulated RT with the use of simultaneous integrated boost and parotid salivary gland sparing was used in this patient's treatment. The RT was performed in 23 identical fractions, the primary tumor region was irradiated with a dose of 46 Gy and the first echelon lymph node region with the risk of subclinical disease with a dose of 41.4 Gy; the dose per fraction was 2 Gy and 1.8 Gy, respectively. The patient is alive and well > 20 months after the irradiation, without any evidence of disease. Parotid gland function remained intact, and no xerostomia occurred. This is the first report of the use of intensity-modulated RT with parotid gland sparing in the treatment of solitary extramedullary plasmacytoma in the head and neck region.