髓外浆细胞瘤与骨单发性浆细胞瘤17例

1977年1月至1988年1月治疗12例髓外装细胞瘤(EMP),5例骨单发性浆细胞瘤(SPB)。单纯放疗10例,手术加放疗5例,手术加化疗2例。照射剂量41～70Gy.60％的SPB在治疗后平均22.3个月发展为多发性骨髓瘤,EMP无发生。16.7％的EMP疗后局部复发,25％的EMP和20％的SPB有区域淋巴结转移。全组3年生存率13/16(81％),EMP与SPB生存率无差异。     

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。     

髓外浆细胞瘤和骨的孤立性浆细胞瘤9例

在浆细胞瘤中，多发性骨髓瘤（mm）常见，而髓外浆细胞瘤（EMP）及骨的孤立性浆细胞瘤（SPB）则罕见。1985—1995年间，作者治疗髓外浆细胞瘤7例，骨的孤立性浆细胞瘤2例。单纯放疗4例，手术加放疗5例。全组3年生存率6／8（7％）。1例SPB在治疗后2年发展为mm，而无1例EMP发生。EMP及SPB治疗后，预后较好。对于预测发展为mm的指标，尚需进一步研究。     

骨的孤立性浆细胞瘤和髓外浆细胞瘤

在浆细胞病中，孤立性浆细胞瘤（骨的孤立性浆细胞瘤和髓外浆细胞瘤）比多发性骨髓瘤罕见，然而两者间关系仍不清楚。１９６０～１９９４年间，作者治疗骨的孤立性浆细胞瘤２４例，髓外浆细胞瘤２０例。诊断标准为：（１）临床与放射学检查未发现另外病变；（２）活检证实为浆细胞瘤；（３）骨髓检查阴性（浆细胞＜１０％）；（４）无贫血、高血钙或肾受累证据。随访平均１１２个月，骨的孤立性浆细胞瘤中５４％、髓外浆细胞瘤中４０％发展为多发性骨髓瘤，两者差异无显著意义（Ｐ＞０．０５）。作者建议将孤立性浆细胞瘤分为潜伏型与侵袭型：肿瘤细胞分化良好者病变趋向潜伏静止，低分化者病变具侵袭性，容易发展为多发性骨髓瘤。治疗选择广泛切除或彻底刮除，瘤腔用液氮或酚等灭活后植骨或骨水泥充填，术后辅以放疗。侵袭型者需辅以化疗，以延缓播散为多发性骨髓瘤。     

单发性骨和骨髓外浆细胞瘤及其与多发性骨髓瘤的关系

单发性浆细胞瘤罕见,其本质及与多发性骨髓瘤的关系也不清楚。本文报告单发性骨和骨髓外浆细胞瘤各12例,分析二者的差别、与多发性骨髓瘤的关系及放疗情况。24例患者中,男18例,女6例,年龄17～80岁,均经放疗或手术+放疗。放疗每周5天,每天200rad.大部分患者(22/24例)总肿瘤量在4000rad以上。为确定患者所患确为单发性浆细胞瘤而非多发性骨髓瘤,著者以3年无瘤生存率为统计单发性浆细胞瘤的基线,因为多发性骨髓瘤有50%患者在确诊后两年内即已死亡。     

原发性肺内髓外浆细胞瘤18F-FDG PET/CT显像1例报告并文献复习

<正>0 引言浆细胞肿瘤可分为多发性骨髓瘤(累及骨髓及其他全身)、孤立性骨髓瘤(骨浆细胞瘤)、髓外(软组织)浆细胞瘤和浆母细胞肉瘤^[1]。髓外浆细胞瘤(extramedullary plasmacytoma,EMP)是一种罕见的不累及骨髓的软组织浆细胞瘤,约占所有浆细胞肿瘤的3%～5%,以上呼吸道及上消化道多见,约占80%～90%,且多见于鼻腔、鼻窦及口鼻咽部^[2]。该病的病因尚不清楚,但病毒发病机制和慢性刺激被认为是主要因素^[3]。     

髓外浆细胞瘤的诊断和治疗

目的 探讨髓外浆细胞瘤（EMP）的诊断和治疗。方法 4例髓外浆细胞瘤的临床资料。结果 1例疗后失访。2例分别随访74月和56月，无局部复发、远处转移，无进展证据。1例治疗后6个月复发，18个月时死于多发性骨髓瘤（MM）。结论 EMP的诊断确立必须有组织学结果，并经系统检查除外MM；放疗和手术是EMP的主要治疗手段。     

髓外浆细胞瘤8例临床分析及文献复习

[目的]探讨髓外浆细胞瘤（EMP）的临床特点与治疗方法。[方法]回顾性分析8例EMP的临床资料,并复习相关文献。[结果]EMP好发于头颈部,男性多见,预后相对较好,较易转化为多发性骨髓瘤。[结论]EMP是罕见的低度恶性肿瘤,确诊依靠病理并排除多发性骨髓瘤等,局部治疗是其主要的治疗手段,化疗对于肿瘤分化较差及复发患者有一定的作用。     

产生IgM的胃浆细胞瘤

浆细胞瘤由成群或成片排列的浆细胞所组成,分为四类:(1)骨髓瘤病或多发性骨髓瘤;(2)骨的单发性骨髓瘤;(3)浆细胞白血病;(4)髓外浆细胞瘤。髓外浆细胞瘤大部分发生在上呼吸道的管壁内。胃浆细胞瘤约占全部髓外浆细胞瘤的5%。自从1928年Vasiliu第一个报告胃浆细胞瘤以来,至今已有60多例。但经免疫学方法证实的为数甚少,本文报告一例缺乏19S—IgM而产生7S—IgM的胃浆细胞瘤,并经免疫荧光及免疫电泳证实。患者为一81岁高龄妇女,因上腹部疼痛而入院,腹部无包块、也无肝、脾肿大及周围淋巴腺病,实验室检查除梅毒血清阳性反应外,其余均无异常。骨髓检查除正常形态的浆细胞稍显增生外,其余均无异常。骨骼X线和闪烁扫描检查正常。钡餐和内窥镜检查,提示胃窦部粘膜弥漫增厚及多发溃疡。活检     

腹壁髓外浆细胞瘤1例报告

浆细胞肿瘤是单克隆性浆细胞异常增殖性疾病。按照WHO分类,浆细胞肿瘤被分为浆细胞骨髓瘤〔多发性骨髓瘤(MM)〕、骨孤立性浆细胞瘤(solitary plasmacytomaofbone,SPB)和髓外浆细胞瘤(extramerdullary plasmacytoma,EMP)。EMP占全部浆细胞肿瘤的5%～10%。其中约80%的EMP发生于上呼吸道,胃肠道约占10%,发生于腹部皮下的EMP报道较少。本研究将确诊的1例EMP病例报道如下。     

Clinical course of solitary extramedullary plasmacytoma.

BACKGROUND AND PURPOSE: Solitary extramedullary plasmacytoma (EMP) represents a rare category of malignant disease on which there are limited data in regard to diagnosis, staging and natural history. This study attempted to clarify the clinical course of solitary extramedullary plasmacytoma after radiation or surgical therapy given with curative intent. MATERIALS AND METHODS: The diagnosis was based on a mass of clonal plasma cells separate from bone or bone marrow without evidence of occult disease elsewhere. Between 1963 and 1996, 22 previously untreated patients with an EMP were diagnosed. Disease presented in the head or neck in 86%, usually in the nasal cavity (NC) or maxillary sinus (MS), and in these areas local bone destruction was found in 10 of 11 patients. Among all patients, serum myeloma protein was present in three patients (14%) and Bence Jones protein alone was found in two patients (9%). Radiation therapy was the sole treatment for 18 of 22 patients, and the median radiotherapy dose was 50 Gy (range, 40-60 Gy); five of seven patients with an EMP of oral cavity (OC), oropharynx (OP), nasopharynx (NP), parotid or larynx also received elective neck irradiation. Two patients underwent surgery plus postoperative irradiation of a plasmacytoma of the sigmoid colon or pleura, and two patients had resection alone of a plasmacytoma of the colon or cervical lymph node. RESULTS: Local control was achieved in 21 of 22 patients (95%), and disease never recurred in regional nodes. Disappearance of myeloma protein occurred in three of five patients with an evaluable abnormality. Multiple myeloma developed in seven patients (32%), all within 5 years. The 5-year rate of freedom from progression to multiple myeloma was 56% and the median survival was 9.5 years. CONCLUSION: Radiation therapy achieved excellent locoregional control of EMP with an approximate cure fraction of 50%.     

Primary isolated extramedullary plasmacytoma of mesentry: A rare case report

Extramedullary plasmacytoma (EMP) is an uncommon entity that most commonly involves nasopharynx and upper repository tract. Involvement of GIT occurs in approximate 10% of cases. According to WHO plasma cell tumors have been classified into two main groups: Multiple myeloma and plasmacytoma. Plasmacytoma includes solitary plasmacytoma of bone and solitary extramedullary plasmacytoma. EMP can be either primary without evidence of bone marrow involvement or may occur simultaneously with multiple myeloma representing extramedullary spread of the disease. It may occur in association with multiple myeloma and it may precede, accompany or follow the onset of multiple myeloma. Diagnosis of primary EMP requires the exclusion of associated multiple myeloma as shown by negative Bence Jones Proteins in urine, normal serum electrophoresis, normal bone marrow biopsy, normal skeletal survey and normal calcium levels. Here we present a case of 55-year male who came to Nephrology Department for urinary tract infection and pain abdomen. Patient was referred to Radiology for ultrasonography which revealed bilateral renal parenchymal disease with a well-defined mass in the mesentry which was further confirmed on computed tomography. Patient was surgically operated and diagnosis of primary EMP of mesentry was made on histopathological examination. Only three cases have been reported so far in the literature. Keywords: Extramedullary plasmacytoma, mesentry, plasma cell dyscrasias.     

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.     

Localised plasmacytomas in Taiwan: comparison between extramedullary plasmacytoma and solitary plasmacytoma of bone.

The clinical features and response to therapy of 32 Chinese patients with localised plasmacytoma are presented, and a comparison between extramedullary plasmacytoma (EMP) and solitary plasmacytoma of bone (SPB) is made. Twenty-two patients had SPB and ten had EMP, accounting for 9% of all of our plasma cell neoplasms. Both groups had a male predominance with a median age of 54 years for SPB and 63 years for EMP. The common sites of SPB included vertebral bodies (15) and the skull (4). Most EMPs occurred in the oronasopharynx (6) and paranasal sinuses (2). An M-protein was detected in eight patients with SPB and in six with EMP. Seventeen patients with SPB and seven with EMP received radiation therapy, and all achieved initial local control. The pattern of failure in 22 patients with SPB manifested as local recurrence in two, multiple bone metastases without bone marrow plasmacytosis in two, multiple EMP progression in two, and development of multiple myeloma (MM) in one. There were two local recurrences, one further solitary bone involvement and one MM conversion in the EMP group. Local recurrence or dissemination was associated with the appearance of M-protein or an increase in the M-protein level in both groups. There was no significant difference in M-protein status or incidence and patterns of failure between the two groups. Patients with EMP had a more favourable overall survival than those with SPB (P = 0.03). The 5 year disease-free survival rate was 79% for EMP and 58% for SPB (P = 0.53). Patients aged less than 60 years had a better overall survival in the SPB group, but location of tumour, presence of M-protein, radiation dose and chemotherapy did not influence prognosis in either group. Our results indicate that adequate local therapy can result in long-term survival with a low frequency of MM progression for patients with localised plasmacytomas, and both EMP and SPB appear to be similar in terms of frequency and patterns of failure.     

Renal extramedullary plasmacytoma—One case report

Extramedullary plasmacytoma (EMP) is rare in multiple myeloma, especially kidneys are involved. We report one case, including diagnosis and treatment. The purpose of this paper is to provide an attention on the extramedullary plasmacytoma disease, reducing the misdiagnose with this disease.     

Renal Extramedullary Plasmacytoma——One Case Report

T Extramedullary plasmacytoma (EMP) is rare in multiple myeloma, especially kidneys are involved. We report one case, including diagnosis and treatment. The purpose of this paper is to provide an attention on the extramedullary plasmacytoma disease, reducing the misdiagnose with this disease.     

Successful treatment of extramedullary gastric plasmacytoma with the combination of bortezomib and dexamethasone: first reported case

We report a case of a 68-year-old man presented with upper-gastrointestinal bleeding. Endoscopy showed a large ulcerated gastric mass. Histological examination of the gastric biopsies revealed a k monoclonal extramedullary plasmacytoma (EMP). Further staging was negative for multiple myeloma. The patient was managed with bortezomib at a dose of 1.3mg/m2 on days 1, 4, 8 and 11 of a 21-day cycle in combination with dexamethasone 20mg p.o. on days 1, 2, 4, 5, 8, 9 and 11, 12 of each cycle. After 4 cycles of treatment, no endoscopic or histological findings of EMP were found. Thirteen months after diagnosis the patient is in complete remission with no evidence of local relapse or evolution to multiple myeloma. This is the first reported case of EMP successfully managed with the combination of bortezomib and dexamethasone.     

Plasma cell neoplasms. Review of disease progression and report of a new variant

Plasma cell neoplasms are generally categorized into four groups; multiple myeloma (MM), solitary plasmacytoma of the bone (SPB), plasma cell leukemias, and extramedullary plasmacytomas (EMP). A plasmacytoma is defined as any discrete, usually solitary mass of neoplastic plasma cells, either in the bone marrow or in various soft tissue sites. Each manifestation of a plasma cell neoplasm differs in terms of tumor recurrence and progression to MM. A gastric plasmacytoma (GP) is a rare presentation of extramedullary plasmacytoma and has not been previously reported as a site of recurrence for a SPB. This pattern of tumor recurrence is unique and the management of gastric plasmacytoma as part of this complex disease is discussed. The continuum of progression between various sites and manifestations of plasma cell manifestations is reviewed including a previously undiscovered sequence of bone disease, gastric disease, and finally multiple myeloma.