Radiotherapy in the management of solitary extramedullary plasmacytoma

BACKGROUND: Solitary extramedullary plasmacytoma (SEP) is a rare tumour for which the standard treatment remains local radiotherapy (RT). We present a study of a multi-institutional experience, between 1980 and 1999, in an attempt to better understand the natural history of SEP and to identify possible prognostic factors. METHODS: The records at Radiation Oncology Victoria and Peter MacCallum Cancer Centre, Melbourne, were used for the identification of patients. The inclusion criteria were as follows: (i) histological confirmation of clonal plasma cells involving a single extramedullary site with or without lymph node involvement; (ii) no histological evidence of bone marrow involvement; (iii) no evidence of distant bone lesion on radiographic skeletal survey (bone erosions adjacent to the primary thought to be due to contiguous involvement were permitted) and (iv) no anaemia, hypercalcaemia or renal impairment due to plasma cell dyscrasia. RESULTS: A total of 16 patients was identified, with a median follow up of 66 months (range 12-211 months). The head and neck region accounted for the majority of presentations (88%). Among all patients, a serum mono-clonal paraprotein was found in three patients and bone erosion was identified in seven patients. All patients received local RT, although two patients also received elective nodal irradiation (ENI). The median RT dose was 45 Gy (range 40-50.4 Gy). Local control was achieved in all patients (100%), however, regional recurrence outside the RT fields occurred in 2/16. Multiple myeloma (MM) developed in five patients, all within 5 years. The predicted 10-year myeloma free survival is 75% and 10-year overall survival is 54%. CONCLUSION: RT can achieve excellent local control of SEP. The rate of conversion to MM is 31%. Moderate dose RT of at least 40 Gy using limited radiation fields is recommended, although ENI should be considered if toxicity is not increased.     

Progression of a solitary plasmacytoma to multiple myeloma. A population‐based registry of the northern Netherlands

Plasmacytoma is characterized by a local accumulation of monoclonal plasma cells without criteria for multiple myeloma (MM). The current treatment regimen is local radiotherapy. However, more than 50% of patients develop MM within 2 years after treatment. A population‐based registry was consulted for the diagnosis of solitary plasmacytoma between 1988 and 2011. Progression to MM and prognostic features for progression to MM were scored, including hypoxia inducible factors (HIF), vascular endothelial growth factor (VEGF, also termed VEGFA) and micro‐vessel density (MVD) expression in biopsy material. A total of 76 patients were included, 34% having extramedullary plasmacytoma (EMP) while 66% had a solitary plasmacytoma of the bone (SBP). Median follow‐up was 89 months, (7–293 months). In Seventy per cent of SBP patients developed MM with a median time to progression of 19 months (5–293). Three patients (12%) with EMP developed MM. High expression of VEGF and HIF‐2α (also termed EPAS1) was demonstrated in conjunction with an increased MVD in 66% of the patients. No association could be shown between angiogenesis parameters and progression to MM. In conclusion, this population‐based study demonstrates that SBP patients have a higher risk of developing MM following local radiotherapy, indicating that this group might benefit from added systemic chemotherapy.     

Extramedullary plasmacytoma of the small intestine: clinical features, diagnosis and treatment

Extramedullary plasmacytoma (EMP) of the small intestine is an unusual plasma cell neoplasm in this anatomic region with only 61 cases described so far. Clinical suspicion is infrequent owing to its location and nonspecific manifestations such as abdominal pain, obstructive symptoms or even bleeding. Diagnosis is reached through histopathological examination coupled with immunohistochemistry of the endoscopic biopsy or surgical resection specimens. Nevertheless, the differential diagnosis between EMP, lymphoma and other kinds of tumors can sometimes be troublesome. The managements include surgery, radiotherapy or chemotherapy. Generally, the prognosis of EMP is favorable, but occasionally it may relapse, or progress to a plasma cell myeloma. EMP should therefore be followed-up for a long period after treatment. In this comprehensive review of the current literature, the patients' characteristics, clinical manifestations, diagnosis, differential diagnosis, treatment and outcome were discussed.     

多发性骨髓瘤并发髓外浸润的临床分析

目的:了解多发性骨髓瘤(MM)髓外浸润的发生率及临床特点.方法:回顾分析357例MM患者中并发髓外浸润患者的临床表现、实验室检查、疗效及预后.结果:共有43例患者在初诊或治疗过程中出现髓外浸润,发生率为12.04%.Logistic回归分析,发现仅M蛋白类型与是否浸润相关(P<0.05).最常见的发病部位为软组织(19...     

Multiple myeloma in elderly patients: prognostic factors and outcome

Abstract:  Objectives : Purpose of this study was to compare prognostic factors and outcome of patients with multiple myeloma (MM) aged >70 yr at diagnosis with those of younger patients. We also applied the recently proposed International Staging System (ISS) for MM in these patients. Patients and methods : Among 1,162 newly diagnosed, symptomatic MM patients included in our database, 357 (31%) were >70 yr of age. Clinical and laboratory variables were evaluated in patients >70 yr and in younger patients and were assessed for possible correlation with survival in patients >70 yr of age. Results : Most clinical and laboratory features were similar in the two groups of patients but older patients presented more frequently with advanced ISS ( P  = 0.02). Despite similar response rates to primary treatment, younger patients survived longer than patients >70 yr of age (40 vs. 28 months, P  = 0.001). There was a longer survival of younger patients than that of older patients diagnosed with ISS stage 1 (median 71 vs. 54 months, P  = 0.007) and ISS stage-2 patients (median: 38 vs. 26 months, P  = 0.0008) but for patients with ISS stage 3 median survival was similarly poor in the younger and older age group (21 and 20 months, P  = 0.283). Other variables associated with impaired prognosis were severe anemia, extensive bone marrow plasmacytosis and elevated serum LDH. Conclusions : Older patients with MM present more often with advanced ISS and have significantly shorter survival than younger patients. The ISS retained its prognostic significance within the group of elderly patients.     

Soluble syndecan-1 level at diagnosis is an independent prognostic factor in multiple myeloma and the extent of fall from diagnosis to plateau predicts for overall survival

Syndecan-1 (CD138) is a heparin sulphate proteoglycan that is over expressed on the surface of both normal and malignant plasma cells and actively shed from the cell surface (soluble syndecan-1). Soluble syndecan-1 has been shown to be an independent prognostic factor in myeloma but its role in prognostic classification requires further investigation. We have retrospectively measured soluble syndecan-1 in 324 presentation samples and 154 plateau phase samples from the UK Medical Research Council Myeloma VIth trial. Log-rank analysis showed that the presentation value of soluble syndecan-1 is a highly significant prognostic factor when assessing survival from entry (chi2=14.92, P<0.0001) and remains an important independent prognostic factor when considered in Cox regression models (P相似文献   

Prognostic evaluation in multiple myeloma: an analysis of the impact of new prognostic factors

We have analysed the prognostic information for survival of presenting features in an unselected series of 394 myeloma patients. 15 variables with significant prognostic information were identified, among these were some not previously or only recently reported: serum levels of hepatocyte growth factor (HGF), interleukin-6 (IL-6), C-terminal cross-linked telopeptide of collagen I (ICTP) and soluble interleukin-6 receptor (sIL-6R). In a multivariate Cox analysis six variables were significantly and independently associated with poor survival: high age, low W.H.O.-performance status (PS), high serum levels of calcium, beta-2-microglobulin (beta-2M), IL-6 and sIL-6R. A risk score formed to predict survival for each percentile of the patient population allowed an efficient separation of prognostic groups. The discriminating power of the model compared favourably with three other previously published staging systems applied to the study population. Exclusion of IL-6 and sIL-6R from the model only marginally decreased the efficacy of the separation. The predictive value of some variables (sIL-6R, beta-2M and W.H.O.-PS) decreased significantly over time. We conclude that formation of a risk score based on independent variables is an efficient way to separate prognostic groups, that the contribution of new and not easily available parameters should be thoroughly evaluated before inclusion in prognostic models for clinical use and that the predictive value of parameters may decrease over time.     

Bone metastases from hepatocellular carcinoma:clinical features and prognostic factors

BACKGROUND: Bone metastases (BMs) from hepatocellu-lar carcinoma (HCC) is an increasingly common disease in Asia. We assessed the clinical features, prognostic factors, and differences in outcomes related to BMs among patients with different treatments for HCC. METHODS: Forty-three consecutive patients who were diag-nosed with BMs from HCC between January 2010 and Decem-ber 2014 were retrospectively enrolled. The clinical features were identified, the impacts of prognostic factors on survival were statistically analyzed, and clinical data were compared. RESULTS: The median patient age was 54 years; 38 patients were male and 5 female. The most common site for BMs was the trunk (69.3%). BMs with extension to the soft tissue were found in 14 patients (32.5%). Most (90.7%) of the lesions were mixed osteolytic and osteoblastic, and most (69.8%) patients presented with multiple BMs. The median survival after BMs diagnosis was 11 months. In multivariate analyses, survival after BM diagnosis was correlated with Karnofsky perfor-mance status (P=0.008) and the Child-Pugh classification (P<0.001); BM-free survival was correlated with progression beyond the University of California San Francisco criteria (P<0.001) and treatment of primary tumors (P<0.001). BMs with extension to soft tissue were less common in liver trans-plantation patients. During metastasis, the control of intrahe-patic tumors was improved in liver transplantation and hepa-tectomy patients, compared to conservatively treated patients. CONCLUSIONS: The independent prognostic factors of surviv-al after diagnosis of BMs were the Karnofsky performance status and Child-Pugh classification. HCC patients developed BMs may also benefit from liver transplantation or hepatectomy.     

多发孤立性浆细胞瘤伴维生素D缺乏

多发孤立性浆细胞瘤(MSP)发病率低,相关报道罕见。本文报道1例63岁女性患者,右下肢针刺样疼痛2月余,伴麻木、乏力。实验室检查提示高甲状旁腺激素血症,血清骨源性碱性磷酸酶升高,维生素D缺乏。神经肌电图显示右下肢神经源性损害。骶椎、骨盆CT增强提示骶1椎体右侧见软组织团片灶,呈膨胀性骨质破坏,骨皮质破裂。18 F-FDF PET/CT显示胸骨、胸、腰椎椎体、左侧髂骨及右侧骶骨骨质破坏影。CT引导下经皮穿刺活检免疫组化显示浆细胞骨髓瘤、CD138(+)、MUM-1(+)、Lambda(+)。结合骨髓涂片等结果该病例诊断为多发孤立性浆细胞瘤。     

Ten-year survival and prognostic factors in multiple myeloma

Summary. Among 1119 Japanese patients with symptomatic multiple myeloma diagnosed between 1965 and 1981, 38 (3.4%) survived more than 10 years. Younger age, low tumour mass (absence of severe anaemia, hypercalcaemia, renal failure, and multiple bone lesions), low plasma cell percentage in bone marrow, mature and intermediate myeloma according to Greipp's criteria, and a positive response to subsequent treatment, were related to long-term survival according to univariate analysis. Multivariate logistic regression analysis indicated younger age and low tumour mass as pretreatment characteristics to be related to long-term survival. Prognostic factors proposed applicable to myeloma were also related to 10-year survival.     

Bortezomib in treatment of extramedullary plasmacytoma of the pancreas

BACKGROUND:Extramedullary pancreatic plasmacy- toma treated with bortezomib is rarely reported. METHODS:We admitted a 53-year-old woman with an asymptomatic mass above the left clavicle for over three months,then an asymptomatic swelling of the pancreas was found.A biopsy on the mass and a fine needle aspiration of the pancreas were performed.The diagnosis of extramedullary plasmacytoma(EMP)was made.The patient was initially treated with combination chemotherapy consisting of vincristine,doxorubicin and dex...     

Solitary extramedullary plasmacytoma in the retroperitoneum

Solitary extramedullary plasmacytoma is an uncommon neoplasm and occurs most frequently in the upper respiratory tract. Herein, we reported a solitary extramedullary plasmacytoma in the retroperitoneum. A 28-year-old man presented with obstructive jaundice and a retroperitoneal tumor. Ultrasound-guided biopsy confirmed that the lesion was a plasma cell neoplasm. A detailed investigation showed that no other sites were involved. The tumor got a moderate reduction following local irradiation, and a complete remission was achieved after 12 courses of adjuvant chemotherapy. Therefore, the possibility of a solitary extramedullary plasmacytoma should be considered in the differential diagnosis of obstructive jaundice without a history of multiple myeloma. Am. J. Hematol. 58:235–238, 1998. © 1998 Wiley-Liss, Inc.     

Clinical significance of vascular endothelial growth factor and hepatocyte growth factor in multiple myeloma

Angiogenesis is a crucial process in the progression of multiple myeloma (MM). Vascular endothelial growth factor (VEGF) and hepatocyte growth factor (HGF) are multifunctional cytokines that potently stimulate angiogenesis including tumour neovascularization. Serum levels of VEGF and HGF were measured in 52 patients with MM by enzyme-linked immunosorbent assay (ELISA). Serum levels of VEGF and HGF were elevated in MM patients compared with healthy controls (VEGF: mean 0.31 ng/ml and 0.08 ng/ml respectively, P < 0.01; HGF: mean 2.17 ng/ml and 0.45 ng/ml, respectively, P < 0.001). In serial samples taken after chemotherapy, serum VEGF and HGF levels were correlated with M-protein levels. Serum levels of VEGF were higher in patients with extramedullary plasmacytomas than in patients without them (P < 0.05). They were also significantly higher in a group of patients who showed poor response to chemotherapy (P < 0.01). Serum levels of HGF were higher in patients with complications such as anaemia, hypercalcaemia and amyloidosis than in patients without these complications (P < 0.01, P < 0.05, P < 0.05 respectively). Both serum VEGF and HGF levels were significant predictors of mortality (P = 0.01, P = 0.02, respectively, log-rank test). The present study demonstrated that serum levels of VEGF and HGF are significantly elevated and dependent on the severity of MM, suggesting that measurement of VEGF and HGF may be useful for assessing disease progression and for predicting the response to chemotherapy in MM patients.     

Mediastinal plasmacytoma detected by echocardiography and biopsied with EUS-FNA

Mediastinal ultrasound is currently being performed by a variety of different practioners but all need to be familiar with major pathology outside their narrow fields. We are reporting the first case where echocardiography led to the discovery of a mediastinal mass which upon endoscopic-ultrasound guided fine needle aspiration biopsy (EUS-FNA) was found to be a plasmacytoma. The differential diagnosis of mediastinal masses is briefly reviewed.     

Pulmonary and intracerebral plasmacytomas in a patient without multiple myeloma: a case report

Extramedullary plasmacytoma of the lung or brain parenchyma is rare. We report herein a case of extramedullary plasmacytoma involving both the lung and parenchymal brain. We review the literature, comparing and contrasting the current case.