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INTRODUCTION: The adrenal gland is a frequent site of metastases in different types of cancer. The aim of this study was to assess the results of metastatic adrenalectomy in a single institution and to identify factors for survival. MATERIAL AND METHODS: A retrospective, single-centre analysis of outcomes of 39 patients (22 male, 17 female) with adrenal metastases who underwent surgery within 14 years (2004-2017) was performed. The median age at the time of adrenal surgery was 64.8 years (range 49-79 years). RESULTS: In our study group non-small cell lung cancer (NSCLC) was the most frequent primary tumour type (15 pts), followed by renal cell carcinoma (RCC) (14 pts) and colon cancer (6 pts). Most of the metastases - 36 (92%) - were metachronous (> 6 months). All synchronous metastases were NSCLC. The mean time from primary cancer to adrenalectomy was 42.3 months (range 1-176) and was statistically longer for RCC. In 3 patients (8%) metastases were bilateral and both adrenal glands were removed. In all patients, surgery was limited to the adrenal gland, and no major complications of surgery were observed. The median overall survival after metastasectomy was 18 months (3-81) and was statistically longer for colon cancer - 29.5 months (p = 0.012). In patients who died, tumours were significantly bigger than in survivors, 76.5 mm vs. 52.5 mm (p = 0.026). CONCLUSIONS: Surgery for adrenal metastasis is safe and indications for this procedure should be individualized. In selected patients, surgical removal of adrenal metastasis was associated with longer survival.
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Adrenal ganglioneuroma is a rare benign neuroblastic tumor. Most of adrenal ganglioneuromas are asymptomatic and discovered incidentally during imaging examinations performed for many different indications. Proper preoperative diagnosis is challenging and most of this masses are described as being poor lipid adenomas or pheochromocytomas. AIM: The aim of the study was to present experience of referral center with this rare adrenal pathologies. MATERIALS AND METHODS: Analysis of our data - 374 adrenalectomies performed over last 13 years (2004-2016) in patients referred to our department from different endocrinological centers. Date of all patients were reviewed retrospectively in order to focus on adrenal ganlioneuroma. RESULTS: Among this patients histopathological report confirmed 10 adrenal ganglioneuromas (2,67% of cases, median age 51 years, range 39-74), diagnosed in 4 men and 6 women. Preoperative CT tumors were described as homogenous masses with mean attenuation on unenhanced images 28 (19-39 HU). In 5 patients (50%), progressive enhancement on delayed-phase postcontrast imaging was observed. 7 right and 3 left adrenalectomies was performed (laparoscopic approach in 50% of cases) Mean size of the resected tumors in histopatogical report was 47mm ( 5-85 ). CONCLUSIONS: Proper preoperative diagnosis of adrenal ganglioneuroma is challenging. In our series all diagnosis was made by histopathological examination. Surgery is indicated because it's difficult to distinguish adrenal gangioneuroma from other adrenal malignances. Radical excision is a definitive cure and may be done safely by laparoscopy.
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Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia , Ganglioneuroma/cirurgia , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Adulto , Idoso , Feminino , Ganglioneuroma/diagnóstico por imagem , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
Primary adrenal leiomyosarcomas are very rare adrenal mesenchymal tumours, only few dozen has been reported in the English language literature so far. We present two cases of patients with primary adrenal leiomyoosarcomas. 80 year-old female admitted with left adrenal tumour with presumptive diagnosis of nonadenoma, laparoscopic adrenalectomy was performed and 85 year-old man who underwent surgical resection by laparotomy of adrenal tumour suspected for adrenal carcinoma. Native density on unenhanced CT was 30 (HU) and 28 (HU) respectively and absolute and relative contrast washout was non-characteristic. Pathologic report reviled thinly encapsulated tumours composed of neoplastic cells with strong cytoplasmic reactivity for smooth muscle actin, desmin and vimentin. Male patient 20 months after surgery was diagnosed with multiple nonresectable metastases, systemic treatment was introduced. Female patient 28 months after surgery has no signs and symptoms of recurrence. We describe the clinical course and a brief review of clinical and histological features , biologic behaviour and diagnostic and therapeutic strategies. The extremely rare occurrence of primary adrenal leiomyosarcoma and a scientific literature based on single cases present challenges in establishing any firm conclusions on management and prognosis. Obviously it's impossible to have proper diagnosis based on clinical symptoms and diagnostic imaging. Radical surgery with free margins seems to be treatment of choice.
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Neoplasias das Glândulas Suprarrenais/diagnóstico , Leiomiossarcoma/diagnóstico , Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia , Idoso de 80 Anos ou mais , Feminino , Humanos , Leiomiossarcoma/cirurgia , Masculino , PrognósticoRESUMO
Rectal cancer constitutes over one-third of all colorectal cancers (CRCs) and is one of the leading causes of cancer-related deaths in developed countries. In order to identify high-risk patients and better adjust therapies, new markers are needed. Systemic inflammatory response (SIR) markers such as LMR, NLR, and PLR have proven to be highly prognostic in many malignancies, including CRC; however, their roles in locally advanced rectal cancer (LARC) are conflicting and lack proper validation. Sixty well-selected patients with LARC treated at the Maria Sklodowska-Curie National Research Institute of Oncology in Warsaw, Poland, between August 2017 and December 2020 were prospectively enrolled in this study. The reproducibility of the pre-treatment levels of the SIR markers, their correlations with clinicopathological characteristics, and their prognostic value were evaluated. There was a significant positive correlation between LMR and cancer-related inflammatory infiltrate (r = 0.38, p = 0.044) and PD-L1 expression in tumor cells, lymphocytes, and macrophages (combined positive score (CPS)) (r = 0.45, p = 0.016). The PLR level was correlated with nodal involvement (p = 0.033). The SIR markers proved to be only moderately reproducible and had no significant prognostic value. In conclusion, the LMR was associated with local cancer-related inflammation and PD-L1 expression in tumor microenvironments. The validity of SIR indices as biomarkers in LARC requires further investigation.
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Neuroendocrine neoplasms of the small intestine (SI-NENs) are one of the most commonly recognized gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs). Carcinoid heart disease (CHD) is the primary cause of death in patients with the carcinoid syndrome (CS). The aim of this retrospective study was to evaluate possible factors impacting upon overall survival (OS) in subjects with both neuroendocrine tumors (NETs) G1/G2 of the small intestine (SI-NET) and CHD. Enrolled in our study of 275 patients with confirmed G1/G2 SI-NET, were 28 (10%) individuals with CHD. Overall survival was assessed using the Kaplan-Meier method. The Cox-Mantel test was used to determine how OS varied between groups. A Cox proportional hazards model was used to conduct univariate analyses of predictive factors for OS and estimate hazard ratios (HRs). Of the 28 individuals with confirmed carcinoid heart disease, 12 (43%) were found to have NET G1 and 16 (57%) were found to have NET G2. Univariate analysis revealed that subjects with CHD and without resection of the primary tumor had a lower OS. Our retrospective study observed that patients who presented with CHD and without resection of primary tumor had worse prognosis of survival. These results suggest that primary tumors may need to be removed when feasible, but further research is needed. However, no solid recommendations can be issued on the basis of our single retrospective study.
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Purpose: Adrenal gland is a common site of metastasis and on the other hand, metastases are the most frequent malignant adrenal tumors. The aim of this study was to estimate the risk of malignancy in suspicious adrenal mass in patients with a history of cancer. Methods: This is a single-center retrospective analysis of patients with adrenal tumors treated previously for different types of cancers. Between 2004 and 2021 a hundred and six such patients were identified. Mean age of patients was 62.6 years (30-78), and mean time from oncologic treatment was 55.8 months (0-274). The most common primary cancer was kidney (RCC): 29 (27.4%), colon/rectum (CRC): 20 (18.9%) and lung (NSCLC): 20 (18.9%). Results: Of 106 patients, 12 had hormonally active (HA) (11,3%) and 94 (88,7%) non active (HNA) tumors In group of patients with HA tumours 4 had hypercortisolaemia and 8 had elevation of urinary metanephrines. In the first group of HA patients pathology confirmed preoperative diagnosis of adrenocortical cancer and no metastasis was found. In all patients from the second group pheochromocytomas were confirmed. Primary (PM) and secondary (SM) malignancies were found in 50 patients (47.2%). In hormone inactive group only SM - 46/94 (48.9%) were diagnosed. The odds that adrenal lesion was a metastasis were higher if primary cancer was RCC (OR 4.29) and NSCLC (OR 12.3). Metastases were also more likely with high native tumor density, and bigger size in CT. The cut-off values for tumor size and native density calculated from receiver operating characteristic (ROC) curves were 37mm and 24, respectively. Conclusion: Risk of malignancy of adrenal mass in a patient with a history of cancer is high (47,2%), regardless of hormonal status. 47,2% risk of malignancy. In preoperative assessment type of primary cancer, adrenal tumour size and native density on CT should be taken into consideration as predictive factors of malignancy. Native density exceeding 24 HU was the strongest risk factor of adrenal malignancy (RR 3.23), followed by history of lung or renal cancer (RR 2.82) and maximum tumor diameter over 37 mm (RR 2.14).
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Sarcoidosis is a systemic inflammatory disease of unknown aetiology. Given its complex clinical presentation, the disorder frequently causes diagnostic challenges. In most cases, the primary manifestation is in the lungs and mediastinum. Breast involvement as the primary manifestation of sarcoidosis is rare, accounting for less than 1% of cases. The authors present the case of a 44-year-old woman whose disease first manifested as multiple non-specific BIRADS 4 lesions in both breasts, accompanied by axillary lymphadenopathy, detected by ultrasound examination. The lesions were not visible on mammography. The course of the disease was clinically silent, with intermittent remissions, until the complete resolution of focal breast lesions on ultrasound after two years of follow-up. The paper presents an algorithm for the management of multifocal breast pathology with associated lymphadenopathy, which led to the prompt verification of sarcoidosis.
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Molecular profile of breast cancer provides information about its biological activity, prognosis and treatment strategies. The purpose of our study was to investigate the correlation between ultrasound features and molecular subtypes of breast cancer. From June 2019 to December 2019, 86 patients (median age 57 years; range 32-88) with 102 breast cancer tumors were included in the study. The molecular subtypes were classified into five types: luminal A (LA), luminal B without HER2 overexpression (LB HER2-), luminal B with HER2 overexpression (LB HER2+), human epidermal growth factor receptor 2 positive (HER2+) and triple negative breast cancer (TNBC). Histopathological verification was obtained in core biopsy or/and post-surgery specimens in all cases. Univariate logistic regression analysis was performed to assess the association between the subtypes and ultrasound imaging features. Experienced radiologists assessed lesions according to the BIRADS-US lexicon. The ultrasound scans were performed with a Supersonic Aixplorer and Supersonix. Based on histopathological verification, the rates of LA, LB HER2-, LB HER2+, HER2+, and TNBC were 33, 17, 17, 16, 19, respectively. Both LB HER2+ and HER2+ subtypes presented higher incidence of calcification (OR = 3.125, p = 0.02, CI 0.0917-5.87) and HER2+ subtype presented a higher incidence of posterior enhancement (OR = 5.75, p = 0.03, CI 1.2257-32.8005), compared to other subtypes. The calcifications were less common in TNBC (OR = 0.176, p = 0.0041, CI 0.0469-0.5335) compared to other subtypes. There were no differences with regard to margin, shape, orientation, elasticity values and vascularity among five molecular subtypes. Our results suggest that there is a correlation between ultrasonographic features assessed according to BIRADS-US lexicon and BC subtypes with HER2 overexpression (both LB HER2+ and HER2+). It may be useful for identification of these aggressive subtypes of breast cancer.
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INTRODUCTION: There is an increasing number of adrenal tumours discovered incidentally during imaging examinations performed for many different indications. Based on imaging results, it is possible to differentiate benign from malignant adrenal masses, although there is still a number of equivocal imaging findings. Our study presents 77 cases of adrenal tumours in which imaging was inconclusive and the final diagnosis was stated only after surgery and histopathological examination. MATERIAL AND METHODS: Retrospective data analysis: 77 cases of indeterminate adrenal tumours with a presumptive diagnosis of "nonadenoma" in patients operated within a 16-year period (2004-2019). None of the patients had a history of malignancy, and all tumours were hormonally inactive. On contrast-enhanced computed tomography (CT), the native density of all tumours was higher than 10 Hounsfield Units (HU), and the absolute percentage washout (APW) and relative percentage washout (RPW) were lower than 60% and 40%, respectively. RESULTS: The most common findings were adrenal adenoma (25.9%), macronodular adrenal hyperplasia (16.9%), ganglioneuroma (15.6%), and haemorrhage with posthaemorrhagic changes (13%). In total, there were 12 various histopathological diagnoses in this group. There were only 2 (2.6%) malignant (adrenal cancer and leiomyosarcoma) and 3 (3.9%) potentially malignant (pheochromocytoma) lesions in this group. CONCLUSIONS: It is often impossible to make a correct diagnosis in a clinical setting until it is histologically verified. "Nonadenoma" adrenal tumours constitute a heterogeneous group including very rare pathologies. The risk of malignancy in indeterminate adrenal tumours is relatively low.
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Doenças das Glândulas Suprarrenais/diagnóstico por imagem , Glândulas Suprarrenais/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
PURPOSE: To evaluate the ultrasound (US) response in patients with breast cancer (BC) during neoadjuvant chemotherapy (NAC). METHODS: Prospective US analysis was performed on 19 malignant tumors prior to NAC treatment and 7â¯days after each first four courses of NAC in 13 patients (median ageâ¯=â¯57â¯years). Echogenicity, size, vascularity, and sonoelastography were measured and compared with posttreatment scores of residual cancers burden. RESULTS: Changes in the echogenicity of tumors after 3 courses of NAC had the most statistically strong correlation with the percentage of residual malignant cells used in histopathology to assess the response to treatment (odds ratioâ¯=â¯60, pâ¯<â¯0.05). Changes in lesion size and elasticity were also significant (pâ¯<â¯0.05). CONCLUSIONS: There is a statistically significant relationship between breast tumors' echogenicity in US, neoplasm size, and stiffness and the response to NAC. In particular, our results show that the change in tumor echogenicity could predict a pathological response with satisfactory accuracy and may be considered in NAC monitoring.
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Neoplasias da Mama/patologia , Terapia Neoadjuvante , Ultrassonografia/métodos , Adulto , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias da Mama/tratamento farmacológico , Técnicas de Imagem por Elasticidade/métodos , Feminino , Humanos , Pessoa de Meia-Idade , Neoplasia Residual , Razão de Chances , Estudos ProspectivosRESUMO
BACKGROUND: Neoadjuvant chemotherapy (NAC) is used in patients with breast cancer to reduce tumor focus, metastatic risk, and patient mortality. Monitoring NAC effects is necessary to capture resistant patients and stop or change treatment. The existing methods for evaluating NAC results have some limitations. The aim of this study was to assess the tumor response at an early stage, after the first doses of the NAC, based on the variability of the backscattered ultrasound energy, and backscatter statistics. The backscatter statistics has not previously been used to monitor NAC effects. METHODS: The B-mode ultrasound images and raw radio frequency data from breast tumors were obtained using an ultrasound scanner before chemotherapy and 1 week after each NAC cycle. The study included twenty-four malignant breast cancers diagnosed in sixteen patients and qualified for neoadjuvant treatment before surgery. The shape parameter of the homodyned K distribution and integrated backscatter, along with the tumor size in the longest dimension, were determined based on ultrasound data and used as markers for NAC response. Cancer tumors were assigned to responding and non-responding groups, according to histopathological evaluation, which was a reference in assessing the utility of markers. Statistical analysis was performed to rate the ability of markers to predict the final NAC response based on data obtained after subsequent therapeutic doses. RESULTS: Statistically significant differences (p<0.05) between groups were obtained after 2, 3, 4, and 5 doses of NAC for quantitative ultrasound markers and after 5 doses for the assessment based on maximum tumor dimension. Statistical analysis showed that, after the second and third NAC courses the classification based on integrated backscatter marker was characterized by an AUC of 0.69 and 0.82, respectively. The introduction of the second quantitative marker describing the statistical properties of scattering increased the corresponding AUC values to 0.82 and 0.91. CONCLUSIONS: Quantitative ultrasound information can characterize the tumor's pathological response better and at an earlier stage of therapy than the assessment of the reduction of its dimensions. The introduction of statistical parameters of ultrasonic backscatter to monitor the effects of chemotherapy can increase the effectiveness of monitoring and contribute to a better personalization of NAC therapy.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias da Mama/diagnóstico por imagem , Carcinoma Intraductal não Infiltrante/diagnóstico por imagem , Quimioterapia Adjuvante/métodos , Terapia Neoadjuvante/métodos , Ultrassonografia/métodos , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias da Mama/tratamento farmacológico , Neoplasias da Mama/patologia , Carcinoma Intraductal não Infiltrante/tratamento farmacológico , Carcinoma Intraductal não Infiltrante/patologia , Feminino , Seguimentos , Humanos , Pessoa de Meia-Idade , Invasividade Neoplásica , PrognósticoRESUMO
BACKGROUND: Neuroendocrine neoplasms of the pancreas (p-NEN) are common gastro-entero-pancreatic neuroendocrine neoplasms (GEP-NENs). The aim of this retrospective study was to review the of value of Somatostatin Receptor Scintigraphy (SRS) in initial detection of p-NEN, evaluation of tumour extent and as imaging follow-up after radical surgery in patients with confirmed well (NETG1) or moderate (NETG2) differentiated p-NEN based on pathological WHO 2017 classification. MATERIAL AND METHODS: Overall 281 patients with confirmed p-NEN were enrolled. The SRS was performed to evaluation of primary p-NEN, also to assess clinical stage of disease, based on current World Health Organization (WHO) classification and during clinical follow-up. A total of 829 examinations were performed over time in these 281 patients using 99mTc HYNICTOC. Images were acquired between 1 - 3 h after i.v. injection of radiotracer. Initially whole body WB-SPECT and then WB-SPECT/CT, with standard iterative reconstruction were used. RESULTS: There were 159 patients with NETG1 (57%) and 122 subjects with NETG2 (43%). The female to male ratio was 1.1:1. In 68 patients (22%) with NETG1/G2 eight-seven SRS (10%) were performed to confirm initial diagnosis. SRS results were as follow: true positive (TP) = 84 (97%), false negative (FN) = 3 (3%), no true negative (TN) or false positive (FP) results of SRS examination (sensitivity of SRS per patient was 96%). In 198 subjects (66%) SRS was used in evaluation and re-evaluation of the clinical stage, A total of 661 (80%) examinations were carried out in these patients. There were TP=514 (77%), TN=136 (21%), FN=7 (1%) and FP=4 (1%) results. The sensitivity and specificity per patient were: 96% and 95%. The sensitivity and specificity per study: 98% and 97%. In 35 patients (12%) SRS was used as imaging follow-up after radical surgery, there were overall 81 examination (10%) which were performed. There were 76 (91%) TN results of examinations of SRS and in 4 patients we identified recurrence (TP). In total, which consists of initial diagnosis/staging and follow-up patients, the sensitivity of SRS was 96% and specificity 97% per patient and per study sensitivity and specificity was 98%. CONCLUSIONS: SRS using 99mTc HYNICTOC acquired in WB-SPECT or WB-SPECT/CT techniques is an excellent imaging modality in detection of primary NETG1/G2 p-NEN. Our study confirms that SRS has high sensitivity and specificity, as a result has tremendous value as an examination method to assess clinical stage of disease and as an imaging follow-up after radical treatment.
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Tumores Neuroendócrinos/diagnóstico por imagem , Tumores Neuroendócrinos/metabolismo , Neoplasias Pancreáticas/diagnóstico por imagem , Neoplasias Pancreáticas/metabolismo , Receptores de Somatostatina/metabolismo , Tomografia Computadorizada com Tomografia Computadorizada de Emissão de Fóton Único , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/patologia , Neoplasias Pancreáticas/patologia , Estudos Retrospectivos , Adulto JovemRESUMO
Objective: Neoadjuvant chemotherapy was initially used in locally advanced breast cancer, and currently it is recommended for patients with Stage 3 and with early-stage disease with human epidermal growth factor receptors positive or triple-negative breast cancer. Ultrasound imaging in combination with a quantitative ultrasound method is a novel diagnostic approach. Aim of study: The aim of this study was to analyze the variability of the integrated backscatter coefficient, and to evaluate their use to predict the effectiveness of treatment and compare to ultrasound examination results. Material and method: Ten patients (mean age 52.9) with 13 breast tumors (mean dimension 41 mm) were selected for neoadjuvant chemotherapy. Ultrasound was performed before the treatment and one week after each course of neoadjuvant chemotherapy. The dimensions were assessed adopting the RECIST criteria. Tissue responses were classified as pathological response into the following categories: not responded to the treatment (G1, cell reduction by ≤9%) and responded to the treatment partially: G2, G3, G4, cell reduction by 10-29% (G2), 30-90% (G3), >90% (G4), respectively, and completely. Results: In B-mode examination partial response was observed in 9/13 cases (completely, G1, G3, G4), and stable disease was demonstrated in 3/13 cases (completely, G1, G4). Complete response was found in 1/13 cases. As for backscatter coefficient, 10/13 tumors (completely, and G2, G3, and G4) were characterized by an increased mean value of 153%. Three tumors 3/13 (G1) displayed a decreased mean value of 31%. Conclusion: The variability of backscatter coefficient, could be associated with alterations in the structure of the tumor tissue during neoadjuvant chemotherapy. There were unequivocal differences between responded and non-responded patients. The backscatter coefficient analysis correlated better with the results of histopathological verification than with the B-mode RECIST criteria.Objective: Neoadjuvant chemotherapy was initially used in locally advanced breast cancer, and currently it is recommended for patients with Stage 3 and with early-stage disease with human epidermal growth factor receptors positive or triple-negative breast cancer. Ultrasound imaging in combination with a quantitative ultrasound method is a novel diagnostic approach. Aim of study: The aim of this study was to analyze the variability of the integrated backscatter coefficient, and to evaluate their use to predict the effectiveness of treatment and compare to ultrasound examination results. Material and method: Ten patients (mean age 52.9) with 13 breast tumors (mean dimension 41 mm) were selected for neoadjuvant chemotherapy. Ultrasound was performed before the treatment and one week after each course of neoadjuvant chemotherapy. The dimensions were assessed adopting the RECIST criteria. Tissue responses were classified as pathological response into the following categories: not responded to the treatment (G1, cell reduction by ≤9%) and responded to the treatment partially: G2, G3, G4, cell reduction by 1029% (G2), 3090% (G3), >90% (G4), respectively, and completely. Results: In B-mode examination partial response was observed in 9/13 cases (completely, G1, G3, G4), and stable disease was demonstrated in 3/13 cases (completely, G1, G4). Complete response was found in 1/13 cases. As for backscatter coefficient, 10/13 tumors (completely, and G2, G3, and G4) were characterized by an increased mean value of 153%. Three tumors 3/13 (G1) displayed a decreased mean value of 31%. Conclusion: The variability of backscatter coefficient, could be associated with alterations in the structure of the tumor tissue during neoadjuvant chemotherapy. There were unequivocal differences between responded and non-responded patients. The backscatter coefficient analysis correlated better with the results of histopathological verification than with the B-mode RECIST criteria.
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The aim of this study was to evaluate the clinical usefulness of the chromogranin A (CgA) determination in patients with neuroendocrine neoplasms (NENs) of the digestive system and to analyse the association between concentration of the marker and progression-free survival (PFS) and overall survival (OS). Serum concentrations of CgA were determined before the treatment in 131 patients with NENs, including patients with tumours located in the pancreas, the small intestine, caecum, appendix and in the colon. No significant associations were identified in CgA concentrations between the control group and patients with NENs in appendix and colon. In patients with NENs of the pancreas and NENs of the small intestine and caecum, increased CgA levels were associated with lymph node involvement, distant metastases and a baseline liver involvement. Analyses revealed significantly higher CgA concentrations in patients with active disease compared to those without symptoms of NEN. In patients with NENs of the pancreas, CgA concentration was correlated with tumour grade and Ki67. Significantly higher CgA levels were also found in patients who died compared to those who lived. Analyses of PFS and OS revealed that CgA concentration was not a prognostic factor in patients with NENs of the pancreas. In patients with NENs of the small intestine and caecum, increased CgA concentrations are independent, poor prognostic factors for both PFS and OS. In conclusion, in patients with NENs in pancreas, CgA levels are associated with disease progression, while in patients with NENs in small intestine and caecum, its concentration is a predictive indicator for PFS and OS.
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Introduction Adrenocortical carcinoma (ACC) is a rare malignancy, associated with poor outcome and few therapeutic options. Despite increasing attention, the knowledge about the clinical course and treatment of these tumors is limited. Objectives Survival rates in ACC are still low and the percentage of relapse is high. Thus, it is crucial to identify the prognostic factors of overall survival (OS) and recurrencefree survival (RFS). Patients and methods This was a retrospective analysis of 66 patients diagnosed with ACC between 2002 and 2015. Results The median OS was 43.5 months, 78.19 months for stage I + II, 22.95 months for stage III, and 19.54 months for stage IV ACC. Older age, stage IV ACC, margin status R2, and no mitotane treatment were associated with poor OS. Low Ki67 and mitotic indices were related to improved OS in a univariate analysis. The median RFS was 101.1 months. Disease recurrence after potentially curative surgery was reported in 1 patient (25%) with stage I, 12 patients (46%) with stage II, and 9 patients (45%) with stage III ACC. Male sex and no mitotane treatment were associated with a reduced RFS in a multivariate analysis and higher Ki67 and mitotic indices in the univariate analysis. Conclusions Ki67 and mitotic indices should be considered as prognostic factors when planning the adjuvant treatment of ACC. Mitotane treatment may be independently associated with better outcomes regardless of the tumor stage.
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Carcinoma Adrenocortical/cirurgia , Índice Mitótico , Carcinoma Adrenocortical/tratamento farmacológico , Carcinoma Adrenocortical/terapia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Mitotano/uso terapêutico , Polônia , Prognóstico , Estudos Retrospectivos , Taxa de SobrevidaRESUMO
INTRODUCTION: There is an increasing number of adrenal being tumours discovered incidentally during imaging examinations performed for many different indications. Radiological findings suggesting adrenal pathology may be caused by true adrenal tumours or by other retroperitoneal masses. Generally, the larger the tumour, the higher the possibility of adrenal cancer. MATERIAL AND METHODS: Analysis of our data - 139 operations performed over 11 years (2004-2014) in patients with tumours in the adrenal area larger than 5 cm. RESULTS: The most common finding was adrenal cancer (25.2%), benign adenoma (24.5%), pheochromocytoma (12.9%), and metastatic cancer (10.1%). In total, there were 19 various histopathological diagnoses in this group. CONCLUSION: Although adrenal cancer is the most likely diagnosis in large adrenal tumours, a broad spectrum of various adrenal and retroperitoneal tumours with size more than 5 cm can be found in such patients.
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Adenoma/patologia , Neoplasias das Glândulas Suprarrenais/patologia , Feocromocitoma/patologia , Carga Tumoral , Adenoma/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/secundário , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Feocromocitoma/diagnóstico por imagem , Adulto JovemRESUMO
The precise diagnosis of thyroid neoplasias will guide surgical management. Primary thyroid paraganglioma has been rarely reported. Data on prevalence, immunohistochemistry (IHC), and molecular genetics in a systematic series of such patients are pending. We performed a multinational population-based study on thyroid paraganglioma and analyzed prevalence, IHC, and molecular genetics. Patients with thyroid paraganglioma were recruited from the European-American-Head-and-Neck-Paraganglioma-Registry. Demographic and clinical data were registered. Histopathology and IHC were re-investigated. All patients with thyroid paraganglioma underwent molecular genetic analyses of the SDHA, SDHB, SDHC, SDHD, SDHAF2, VHL, RET, TMEM127, and MAX genes. Analyses included Sanger sequencing and multiplex ligation-dependent probe amplification (MLPA) for detection of large rearrangements. Of 947 registrants, eight candidates were initially identified. After immunohistochemical analyses of these eight subjects, 5 (0.5%) were confirmed to have thyroid paraganglioma. IHC was positive for chromogranin, synaptophysin, and S-100 and negative for calcitonin in all five thyroid paragangliomas, whereas the three excluded candidate tumors stained positive for pan-cytokeratin, a marker excluding endocrine tumors. Germline variants, probably representing mutations, were found in four of the five confirmed thyroid paraganglioma cases, two each in SDHA and SDHB, whereas the excluded cases had no mutations in the tested genes. Thyroid paraganglioma is a finite entity, which must be differentiated from medullary thyroid carcinoma, because medical, surgical, and genetic management for each is different. Notably, approximately 80% of thyroid paragangliomas are associated with germline variants, with implications for additional tumors and a potential risk for the family. As opposed to sporadic tumors, surgical management and extent of resection are different for heritable tumors, each guided by the precise gene involved.
Assuntos
Paraganglioma , Neoplasias da Glândula Tireoide , Adulto , Idoso , Calcitonina/metabolismo , Cromogranina A/metabolismo , Proteínas de Ligação a DNA/metabolismo , Complexo II de Transporte de Elétrons/genética , Feminino , Alemanha/epidemiologia , Humanos , Queratinas/metabolismo , Masculino , Pessoa de Meia-Idade , Mutação , Paraganglioma/epidemiologia , Paraganglioma/genética , Paraganglioma/patologia , Prevalência , Sistema de Registros , Proteínas S100/metabolismo , Succinato Desidrogenase/genética , Sinaptofisina/metabolismo , Neoplasias da Glândula Tireoide/epidemiologia , Neoplasias da Glândula Tireoide/genética , Neoplasias da Glândula Tireoide/patologia , Fatores de TranscriçãoRESUMO
The study involved 51 patients with histopathologically verified diagnoses of primary mediastinal large B-cell lymphoma (PMBL). Clinically they presented as the anterior mediastinal tumours, frequently with the superior vena cava syndrome (21 patients) and usually (in 42 out of 51) without involvement of other locations. Morphologically the tumours were very pleomorphic. Histopathologically they were composed of germinal center cells, clear cells or polymorphic cells of various size and shape. However, there were also PMBLs composed of cells resembling Reed-Sternberg and Hodgkin cells and small cells. Various types of stromal sclerosis were evident in most biopsies. Due to lymphoma patterns mimicking other tumours with primary or secondary mediastinal location it was necessary to use a panel of immunocytochemical stains. CD20 staining proved to be indispensable for distinguishing PMBL. The patients were treated with CHOP or MEVA (V-VIII courses) followed by radiotherapy of the mediastinum (3600-4200cGy/t). Complete remission was obtained in 21 patients with overall survival ranging from 36 to 99 months. A total of 24 patients are alive, 3 of them live with persisting disease. One patients was lost to follow up, 26 died despite the treatment due to persistence of mediastinal tumour or dissemination to distant organs.