Extramedullary plasmacytoma (EMP): Report of a case manifested as a mediastinal mass and multiple pulmonary nodules and review of literature

<p>Abstract</p> <p>Background</p> <p>Extramedullary plasmacytoma (EMP) is a rare plasma cell neoplasm of soft tissue without bone marrow involvement or other systemic characteristics of multiple myeloma</p> <p>Case presentation</p> <p>A 42 year-old woman presented with intermittent dry cough of 10 months duration. Her breathing sound was slightly coarse without rales or rhonchi on auscultation. CT scan revealed a right anterior mediastinal shadow with multiple pulmonary nodular lesions. A video-assisted thoracoscopic surgery (VATS) was performed. Histopathology showed it to be a myeloma.</p> <p>Conclusion</p> <p>This is the first presentation of EMP with a mediastinal mass with multiple pulmonary nodules.</p

Prognostic factors in solitary plasmacytoma of the bone: a multicenter Rare Cancer Network study

BACKGROUND: Solitary plasmacytoma (SP) of the bone is a rare plasma-cell neoplasm. There are no conclusive data in the literature on the optimal radiation therapy (RT) dose in SP. Therefore, in this large retrospective study, we wanted to assess the outcome, prognostic factors, and the optimal RT dose in patients with SP. METHODS: Data from 206 patients with bone SP without evidence of multiple myeloma (MM) were collected. Histopathological diagnosis was obtained for all patients. The majority (n = 169) of the patients received RT alone; 32 chemotherapy and RT, and 5 surgery. Median follow-up was 54 months (7–245). RESULTS: Five-year overall survival, disease-free survival (DFS), and local control was 70%, 46%, and 88%; respectively. Median time to MM development was 21 months (2–135) with a 5-year probability of 51%. In multivariate analyses, favorable factors were younger age and tumor size < 5 cm for survival; younger age for DFS; anatomic localization (vertebra vs. other) for local control. Older age was the only predictor for MM. There was no dose-response relationship for doses 30 Gy or higher, even for larger tumors. CONCLUSION: Younger patients, especially those with vertebral localization have the best outcome when treated with moderate-dose RT. Progression to MM remains the main problem. Further investigation should focus on adjuvant chemotherapy and/or novel therapeutic agents

Skeletal Plasmacytoma: Progression of disease and impact of local treatment; an analysis of SEER database

<p>Abstract</p> <p>Background</p> <p>Previous reports suggest an as yet unidentifiable subset of patients with plasmacytoma will progress to myeloma. The current study sought to establish the risk of developing myeloma and determine the prognostic factors affecting the progression of disease.</p> <p>Methods</p> <p>Patients with plasmacytoma diagnosed between 1973 and 2005 were identified in the SEER database(1164 patients). Patient demographics and clinical characteristics, treatment(s), cause of death, and survival were extracted. Kaplan-Meier, log-rank, and Cox regression were used to analyze prognostic factors.</p> <p>Results</p> <p>The five year survival among patients initially diagnosed with plasmacytoma that later progressed to multiple myeloma and those initially diagnosed with multiple myeloma were almost identical (25% and 23%; respectively). Five year survival for patients with plasmacytoma that did not progress to multiple myeloma was significantly better (72%). Age > 60 years was the only factor that correlated with progression of disease (p = 0.027).</p> <p>Discussion</p> <p>Plasmacytoma consists of two cohorts of patients with different overall survival; those patients that do not progress to systemic disease and those that develop myeloma. Age > 60 years is associated with disease progression. Identifying patients with systemic disease early in the treatment will permit aggressive and novel treatment strategies to be implemented.</p

RNA interference approaches for treatment of HIV-1 infection

HIV/AIDS is a chronic and debilitating disease that cannot be cured with current antiretroviral drugs. While combinatorial antiretroviral therapy (cART) can potently suppress HIV-1 replication and delay the onset of AIDS, viral mutagenesis often leads to viral escape from multiple drugs. In addition to the pharmacological agents that comprise cART drug cocktails, new biological therapeutics are reaching the clinic. These include gene-based therapies that utilize RNA interference (RNAi) to silence the expression of viral or host mRNA targets that are required for HIV-1 infection and/or replication. RNAi allows sequence-specific design to compensate for viral mutants and natural variants, thereby drastically expanding the number of therapeutic targets beyond the capabilities of cART. Recent advances in clinical and preclinical studies have demonstrated the promise of RNAi therapeutics, reinforcing the concept that RNAi-based agents might offer a safe, effective, and more durable approach for the treatment of HIV/AIDS. Nevertheless, there are challenges that must be overcome in order for RNAi therapeutics to reach their clinical potential. These include the refinement of strategies for delivery and to reduce the risk of mutational escape. In this review, we provide an overview of RNAi-based therapies for HIV-1, examine a variety of combinatorial RNAi strategies, and discuss approaches for ex vivo delivery and in vivo delivery

Abstract P5-08-13: Phyllodes tumours of the breast: The British Columbia cancer agency experience

Abstract Background Phyllodes tumours (PT) of the breast are uncommon fibroepithelial lesions for which optimal management remains unclear. This population-based study reports treatment and outcomes for patients with PT and evaluates characteristics that influence outcome. Methods Data were analysed on 183 patients with newly diagnosed PT referred to the BC Cancer Agency from 1999-2014. Follow-up was obtained from chart and death records and letters to general practitioners. Five-year Kaplan-Meier (KM) local recurrence (LR) and survival were compared between cohorts with benign (n=83), borderline (n=50) and malignant PT (n=49) histology. Subtype was unknown in 1 patient who did not receive surgery due to severe comorbidity. Univariate analysis was performed using Cox regression modeling. Results Median follow-up was 65 (range 0.5-197) months. Median age was 48 (range 14–87) years. Median tumour size was 4 (range 1-23) cm. Heterologous sarcomatous differentiation was seen in 15 and malignant epithelial transformation in 11 patients. Local excision was performed in 163 (89%) and mastectomy in 19 (10%) patients. Margin status after local excision were: negative (&amp;gt;1mm, n=121, 74%), close (≤1mm, n=21, 13%), positive (tumour touching ink, n=20, 12%), or unknown (n=1, 1%). Margin status after mastectomy were negative (n=14, 74%) or close (n=5, 26%). Tumour borders were pushing (n=62, 34%), intermediate (n=22, 12%), infiltrative (n=38, 21%) or unknown (n=60, 33%). Eleven patients with malignant PT received radiation therapy as part of initial treatment. In these cases median tumour size was 8 cm and heterologous sarcomatous differentiation was present in 46% compared to 4 cm and 16% in malignant cases who did not receive RT. LR occurred in 16 cases (5 benign, 4 borderline and 7 malignant). Distant metastases (DM) occurred in 7 patients with malignant PT leading to 6 cause specific deaths. Five-year KM outcomes among women with benign, borderline, and malignant PT were: LR 6% vs 9% vs 21%, P=0.131; overall survival 96% vs 100% vs 82%, P=0.002; and disease free survival 94% vs 91% vs 67%, P&amp;lt;0.001. DM-free and cause specific survival at 5 years for malignant cases were 82% and 88% respectively. Five-year KM LR among women with negative vs close vs positive margins were 8% vs 6% vs 37%, P&amp;lt;0.001. Corresponding rates for intermediate vs pushing vs infiltrative borders were 6% vs 6% vs 33%, P=0.006. On univariate analyses, large tumour size, postmenopausal status, malignant classification, necrosis, positive margins, and infiltrative borders were factors associated with increased risk of any type of relapse (all P≤0.014). Positive margins and infiltrative tumour border were associated with increased LR (all P≤0.006), and the latter remained significant in exploratory analyses after adjusting for margin status and PT classification. Advanced age at diagnosis and large tumour size were predictors of DM (all P≤0.001). Conclusion In this population-based series, 5-year outcomes among women with PT are comparable to those reported in the literature. Exploratory analysis suggests that infiltrative tumour borders may be used in conjunction with margin status to assess LR risk. While close margin was not associated with increased LR, re-excision is warranted for cases with positive margins. Citation Format: Rodrigues MF, Truong PT, Weir LM, Knowling MA, Wai ES. Phyllodes tumours of the breast: The British Columbia cancer agency experience [abstract]. In: Proceedings of the 2016 San Antonio Breast Cancer Symposium; 2016 Dec 6-10; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2017;77(4 Suppl):Abstract nr P5-08-13.</jats:p

Solitary Plasmacytoma of the Mandible: An Uncommon Entity

Introduction: Plasma cell dyscrasias are characterized by a monoclonal neoplastic proliferation of plasma cells. Solitary bone plasmacytoma (SBP) is a local form of the disease with the vertebrae and long bones being the most frequently encountered sites. Its prevalence in the maxillofacial area is extremely rare. Case Presentation: A 70-year-old Caucasian male patient was referred for the extraction of his mobile premolar tooth with a poorly-defined radiolucent lesion. Histopathological analysis revealed an SBP and no distant lesion or serum M protein was noted on radiological and hematological examinations. The patient was under follow-up care with no recurrence at 2 years of follow up. Conclusions: Diagnosis of an SBP is based on local radiological and neurological symptoms and similar systemic manifestations of multiple myeloma that are also distinctive for SBP. Skeletal radiological analysis including CT and PET-CT, bone marrow biopsy, and serum protein electrophoresis are essential for confirmation of the diagnosis. Although surgery, chemotherapy, and radiation, or a combination of these modalities, have been successfully used in the treatment of SBP, it should be managed in relation to its possible long-term evolution