scholarly journals The lung in amyloidosis

2017 ◽  
Vol 26 (145) ◽  
pp. 170046 ◽  
Author(s):  
Paolo Milani ◽  
Marco Basset ◽  
Francesca Russo ◽  
Andrea Foli ◽  
Giovanni Palladini ◽  
...  

Amyloidosis is a disorder caused by misfolding of autologous protein and its extracellular deposition as fibrils, resulting in vital organ dysfunction and eventually death. Pulmonary amyloidosis may be localised or part of systemic amyloidosis.Pulmonary interstitial amyloidosis is symptomatic only if the amyloid deposits severely affect gas exchange alveolar structure, thus resulting in serious respiratory impairment. Localised parenchymal involvement may be present as nodular amyloidosis or as amyloid deposits associated with localised lymphomas. Finally, tracheobronchial amyloidosis, which is usually not associated with evident clonal proliferation, may result in airway stenosis.Because the treatment options for amyloidosis are dependent on the fibril protein type, the workup of all new cases should include accurate determination of the amyloid protein. Most cases are asymptomatic and need only a careful follow-up. Diffuse alveolar-septal amyloidosis is treated according to the underlying systemic amyloidosis. Nodular pulmonary amyloidosis is usually localised, conservative excision is usually curative and the long-term prognosis is excellent. Tracheobronchial amyloidosis is usually treated with bronchoscopic interventions or external beam radiation therapy.

2017 ◽  
Vol 141 (2) ◽  
pp. 247-254 ◽  
Author(s):  
Andras Khoor ◽  
Thomas V. Colby

Context.—Amyloidosis is a heterogeneous group of diseases characterized by the deposition of congophilic amyloid fibrils in the extracellular matrix of tissues and organs. To date, 31 fibril proteins have been identified in humans, and it is now recommended that amyloidoses be named after these fibril proteins. Based on this classification scheme, the most common forms of amyloidosis include systemic AL (formerly primary), systemic AA (formerly secondary), systemic wild-type ATTR (formerly age-related or senile systemic), and systemic hereditary ATTR amyloidosis (formerly familial amyloid polyneuropathy). Three different clinicopathologic forms of amyloidosis can be seen in the lungs: diffuse alveolar-septal amyloidosis, nodular pulmonary amyloidosis, and tracheobronchial amyloidosis. Objective.—To clarify the relationship between the fibril protein–based amyloidosis classification system and the clinicopathologic forms of pulmonary amyloidosis and to provide a useful guide for diagnosing these entities for the practicing pathologist. Data Sources.—This is a narrative review based on PubMed searches and the authors' own experiences. Conclusions.—Diffuse alveolar-septal amyloidosis is usually caused by systemic AL amyloidosis, whereas nodular pulmonary amyloidosis and tracheobronchial amyloidosis usually represent localized AL amyloidosis. However, these generalized scenarios cannot always be applied to individual cases. Because the treatment options for amyloidosis are dependent on the fibril protein–based classifications and whether the process is systemic or localized, the workup of new clinically relevant cases should include amyloid subtyping (preferably with mass spectrometry–based proteomic analysis) and further clinical investigation.


2013 ◽  
Vol 31 (15_suppl) ◽  
pp. 6530-6530 ◽  
Author(s):  
Ronald C. Chen ◽  
Matthew Edward Nielsen ◽  
Bryce B. Reeve ◽  
Laura H. Hendrix ◽  
Robert P Agans ◽  
...  

6530 Background: NC ProCESS is a population-based cohort of early (non-metastatic) CaP patients followed prospectively from diagnosis. Methods: Patients were identified through Rapid Case Ascertainment of the NC Cancer Registry from all NC counties in 2010-12. Phone survey assessed perceptions regarding treatment options and priorities in treatment selection. Results: 937 (59% of all eligible) completed this survey. Median age was 65; 72% were Caucasian. At time of survey, ~13 weeks from diagnosis, 98% had discussed options with a urologist, 49% with primary care, and 41% radiation oncologist. Many patients had concerns about potential effects of surgery and radiation on ability to perform daily activities, recovery time, and burden to family (Table). Open prostatectomy (ORP) and external beam radiation (RT) were deemed most likely to affect urinary and sexual function; fewer reported concern with robotic prostatectomy (RALP). Only 32% reported hormonal therapy would affect sexual function. Most reported surgery (especially RALP) had the best chance for cure, while 59% worried about recurrence with RT. In almost all questions, patients who consulted only with a urologist had significantly different perceptions about treatment options than those who also consulted with a radiation oncologist. In choosing treatment, 61% reported that cure was the highest priority, and 28% indicated preserving quality of life. Conclusions: Modern CaP patients often have misconceptions about treatment options inconsistent with published evidence, which are partially mitigated by multidisciplinary consultation. Most indicated cure as the highest priority, and surgery offers the best chance of cure. [Table: see text]


2013 ◽  
Vol 31 (6_suppl) ◽  
pp. 99-99
Author(s):  
Gilles Pasticier ◽  
Eduard Baco ◽  
Olivier Rouviere ◽  
Sebastien Crouzet ◽  
Jean-Yves Chapelon ◽  
...  

99 Background: One third of patients treated with External beam radiation therapy (EBRT) for localized prostate cancer (PCa) experience local recurrence. Salvage treatment options include prostatectomy, cryoablation, and High Intensity Focused Ultrasound (HIFU). Whole gland treatment in these patients offers acceptable cancer control, but carries a risk of severe urinary incontinence in at least 20% of cases and reduction of Quality of Life (QoL). In patients with unilateral local relapse, focal HIFU is feasible. The aim of this prospective study was to evaluate the effect of Hemi HIFU in patients with unilateral recurrence after radiotherapy. Methods: Between 2009 and 2012, 48 patients were prospectively included in 2 centers. Inclusion criteria were positive MRI and biopsy in one lobe diagnosing unilateral cancer after EBRT (46 patients) and after brachytherapy (2 patients). Mean age was 68.8 ± 6 years, mean pre HIFU PSA was 5.2 ± 5.2 ng/mL and the repartition of Gleason score was ≤7: 28, ≥8: 18 and not determined: 2. Median follow-up was 16.3 months [range 3-43]. Treatments were performed with Ablatherm® HIFU device. Results: The mean PSA nadir value was 0.69 ± 0.83 (median: 0.4). Disease progression occured in 16 patients (35,5%). Local recurrence was found in 4 patients in the controlateral lobe, and in 4 patients in both lobes. Six patients developped metastases and 2 had rising PSA without local recurence or proven metastasis. Fifteen of these 16 patients received salvage treatments (3 re-HIFU, 11 androgen-deprivation, and 1 re-HIFU plus androgen-deprivation). Thirthy-six patients (75%) were pad-free. Seven patients (14.6 %) required 1 pad a day. Severe incontinence occured in 5 patients (10.4%). One of them received artificial urinary sphincter. Paired results indicated no significant change in QoL and IPSS scores: EORTC-QLC-30: (from 35.7 ± 8.7 to 36.8 ± 8.6, p=0.22) and IPSS: (from 7 ± 5.6 to 8.5 ± 5.1, p=0.13) Conclusions: Hemi-salvage HIFU is efficient in patients with unilateral radio-recurrent PCa with a preserved QoL offering comparable cancer control to whole gland treatment.


2014 ◽  
Vol 32 (30_suppl) ◽  
pp. 305-305
Author(s):  
Christopher J. Inserra ◽  
Nabin Khanal ◽  
Peter T. Silberstein

305 Background: Glioblastoma Multiforme (GBM) is the most common and most deadly type of human glioma. Nearly half of all gliomas are diagnosed as GBM at which point the median survival of patients is approximately one year and the two-year survival rates are approximately 10%. Current treatment options for GBM include surgical resection, external beam radiation, and oral temozolomide chemotherapy. However, the patterns of chemotherapy use in GBM as well as the patient characteristics that determine its use have yet to be investigated. Methods: This is a retrospective study of glioblastoma patients (n = 96,966, making this the largest trial ever on glioblastoma) diagnosed between 2000 and 2011 in the NCDB. The NCDB contains nearly 70% of new cancer cases diagnosed in the United States and consists of data from over 1,500 cancer programs across the country. A chi-squared test was used to determine any differences in the characteristics of patients who did or did not receive chemotherapy. Results: Patients who were younger than 70 years of age, male, white, had private/managed insurance, no comorbidities, household income greater than $49,000, were receiving radiation therapy, and diagnosed between 2004 and 2011 were significantly more likely to have received chemotherapy to treat glioblastoma (see Table). Conclusions: Understanding any potential barriers in the use of chemotherapy to treat glioblastoma can help improve its utilization among people of diverse socioeconomic backgrounds. [Table: see text]


2019 ◽  
Vol 10 (3) ◽  
pp. 37-44
Author(s):  
M. S. Taratkin ◽  
E. A. Laukhtina ◽  
K. I. Adelman ◽  
Y. G. Alyaev ◽  
L. M. Rapoport ◽  
...  

Prostate cancer (PCa) is the most common oncological disease among men. It is important to note that over 50% of the first identified primary malignant neoplasms of prostate are low - risk PCa. Recently, radical prostatectomy and external beam radiation therapy have been the standard treatment options for PCa. According to recent data, patients with low - risk PCa have a favourable prognosis because of the slow progression of the disease. Some studies show no links between 10-year cancer - specific survival and treatment modalities and no progression even in the absence of therapy. Active surveillance (AS) allows avoiding unnecessary treatment in men who do not require immediate intervention but achieves the correct timing for curative treatment in those who eventually need it. According to the guidelines of the European Association of Urology, AS is one of the standard treatment options for low - risk PCa and should be consideredfor all patients in this category. The advantage of AS is to improve the quality of life in men with low - risk PCa and to delay surgical interventions as much as possible. However, despite widespread AS worldwide, there are only a few centres, which use it routinely in Russia. In this review, we would like to shed some light on the most important questions of AS strategy: what criteria should we use for selection of patients for AS strategy? How often should patient visit the urologist, control PSA level, and undergo prostate biopsy? When should a doctor change strategy and turn to active treatment? In this article, we considered indications for AS in men with PCa and showed the most recent data on the efficacy and relevance of this modality.


2015 ◽  
pp. 39-45

Introduction: Retinoblastoma is a devastating autosomal dominant genetic disease usually seen in children. It causes tumors of the retina which can lead to severe visual impairments and in some cases death. Body: A defective or absent pRB protein, from a mutated or deleted RB1 gene, causes genomic instability and allows retinoblasts to undergo rapid mitosis. This can lead to the formation of tumors within the retina. As retinoblastoma usually affects infants and young children, they may not notice any changes in their vision or ocular discomfort. The most commonly observed sign of retinoblastoma is leukocoria, caused by the light colored tumor within the eye. There are many treatment options available, some of which include chemotherapy, enucleation, external beam radiation, and radioactive plaques. If caught early, the prognosis is usually very good in the United States. Unfortunately, in second and third world countries the outlook can be significantly worse. Discussion: Retinoblastoma is a complex disease which can have severe health impacts and endanger the life of the afflicted child. Although this disease is rare, it is the most common primary ocular malignancy in children. Retinoblastoma is caused by a disruption of the RB1 gene. Research has led to the triple hit hypothesis that three mutations are required for retinoblastoma formation. Tumors often appear early as translucent thickenings of the retina and evolve into dome-shaped, white, vascularized masses. The most common treatments include chemotherapy, radiation and enucleation. Early detection is best accomplished when young children receive comprehensive eye examinations. Catching the disease before it metastasizes greatly improves a child’s chance of survival. Early diagnosis and subsequent treatment could ultimately save a child’s vision or even his or her life.


2009 ◽  
Vol 27 (15_suppl) ◽  
pp. e16147-e16147
Author(s):  
G. J. Kubicek ◽  
G. J. Kubicek ◽  
S. Brown ◽  
S. Redfield

e16147 Background: Prostate cancer is the most common male malignancy, and there is no one standard treatment modality. One treatment option is the combination of external beam radiotherapy and permanent transperineal brachytherapy seed implant Methods: Retrospective review of prostate cancer and side effect outcomes at a single institution in the community setting. All patients were treated with a combination of low dose rate transperineal brachytherapy seed placement and external beam radiation. Results: A total of 897 patients were analyzed, 781 had a minimum follow-up of one year. Median pre-treatment PSA was 8.1 (range 0.3 to 106) and the median Gleason score was 6. With a median follow-up of 3.6 years, 33 (3.4 %) patients had biochemical failure based on the phoenix definition of Nadir + 2. Not including impotence, acute toxicity greater than or equal to Grade 2 was seen in 115 patients (102 GU and 13 GI) and 193 patients had late toxicity greater than or equal to Grade 2 (155 GU and 38 GI). 563 patients received hormone therapy prior to or concurrent with the radiation. Conclusions: This is the largest series reporting on the outcome of combination brachytherpay implant and external beam radiation in the treatment of prostate cancer. Combination treatment using brachytherapy and external beam radiation is well tolerated, with a low rate of biochemical failure and should be considered one of the treatment options for prostate cancer. No significant financial relationships to disclose.


2014 ◽  
Vol 32 (4_suppl) ◽  
pp. 269-269
Author(s):  
Koji Okihara ◽  
Kana Kobayashi ◽  
Kazumi Kamoi ◽  
Tsuyoshi Iwata ◽  
Tsuneharu Miki

269 Background: Salvage permanent brachytherapy (SPB) is one of treatment options in recurred men after external beam radiation therapy (EBRT). The accurate 3D correspondence between recurred cancer focus and location of seed implants using transrectal sonography (TRUS) is indispensable to design excellent dosimetry. The application of MRI-TRUS fusion targeted/systematic biopsy (Fusion-B) has enabled us to draw 3D recurred-cancer mapping. We have started SPB in men who recurred 3D-conformal EBRT using 3D-cancer mapping from Fusion-B. We introduce the novel dosimetric criteria for SPB-based on 3D cancer mapping. Methods: The patients with PSA failure (defined by Pheonix criteria) and without lymphnode/distant metastasis were enrolled in this study. In men who had suspicious recurrent focus/foci in MRI, we performed Fuision-B (ultrasound equipment: Image fusion: Koelis in France, and Biopsy: Medison in South Korea). We classified the three dosimetric patterns as follows: (1) Focal pattern: one core positive from target biopsy alone within hemilobe and GS less than 8. (2) Hemi-lobe pattern: positive cores within hemi-lobe from target/ systematic biopsies and GS less than 8. (3) Whole / focused gland pattern c/w hormonal therapy: Positive core(s) with GS greater than seven was identified regardless of positive biopsies location, or positive cores with GS less than eight from bilateral lobes. Prescribed dose to the target lesion, rV100 and uD90 were planned greater than 160Gy, 0% and less than 165Gy respectively. Results: Since August 2012, SPB has underwent in nine cases (PSA range prior to SPB: 2.22- 4.97ng/ml, age 61 to 77). In those cases, prescription dose at the 3D-conformal EBRT was 72 Gy and the duration between the 3D-conformal EBRT and SPB ranged from 41 to 107 months. The biopsy GS (-7/8-) obtained from Fuison-B distributed in 6/3 cases. Biopsy revealed single focus obtained from target biopsy in two cases. Focal and hemi-lobe pattern were chosen in two and one case, respectively. For the present, there is no re-PSA failure and G3-4 GI and GU comorbidities. Conclusions: To reduce the incidence of co-morbidity and maintain reasonable oncological outcome, it is informative to apply the 3D Cancer mapping for the SPB candidates. It is necessary to assess this criteria with the further follow-up.


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