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  1. Article ; Online: High-Specific-Activity 131I-MIBG for the Treatment of Advanced Pheochromocytoma and Paraganglioma.

    Al-Ward, Ruaa / Brondani, Vania Balderrama / Sawani, Sahar / Potter, Cheryl L / Xu, Guofan / Waguespack, Steven G / Varghese, Jeena / Habra, Mouhammed Amir / Lu, Yang / Jimenez, Camilo

    Clinical nuclear medicine

    2024  

    Abstract: Patients and methods: The primary endpoints were objective response rate (ORR) and disease control rate (DCR). Secondary endpoints were duration of response, blood pressure control, safety, overall and progression-free survival rates, MIBG uptake, and ... ...

    Abstract Patients and methods: The primary endpoints were objective response rate (ORR) and disease control rate (DCR). Secondary endpoints were duration of response, blood pressure control, safety, overall and progression-free survival rates, MIBG uptake, and correlations with genetic background.
    Results: The study included 25 patients. Twenty-four patients had distant metastases, 17 (68%) had hormonally active tumors, and 13 (52%) had previously received antineoplastic treatment. In 24 evaluable patients, the ORR was 38%, including 2 patients with complete response, and the DCR was 83%; median time to response was 12.5 months (95% confidence interval, 4.6-25.1). Twelve patients had sporadic disease, among whom the ORR was 25% and DCR was 83%. Twelve patients had hereditary disease (SDHB, VHL, RET); among these, the ORR was 50%, and DCR was 83%. Plasma metanephrines normalized in 30% of patients and improved by greater than 50% in 46%. Sixteen patients had hormonally active tumors and hypertension; in 9 (56%) of these, blood pressure normalized, leading to discontinuation of antihypertensive therapy.The most common adverse events were grades 1-2 nausea/vomiting and transient bone marrow suppression. One patient developed premature ovarian failure. Reversible grades 3-4 myelosuppression were seen in 7 patients (28%). One patient had fatal pneumonitis.
    Conclusions: HSA-131I-MIBG is associated with a high DCR in patients with MPPGL, regardless of underlying genetic mutation.
    Language English
    Publishing date 2024-04-17
    Publishing country United States
    Document type Journal Article
    ZDB-ID 197628-x
    ISSN 1536-0229 ; 0363-9762
    ISSN (online) 1536-0229
    ISSN 0363-9762
    DOI 10.1097/RLU.0000000000005184
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 1276: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
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  2. Article ; Online: Adrenal cysts of lymphatic origin: A clinical and pathological study of six cases and systematic literature review.

    Marques-Piubelli, Mario L / Gonzales, Eloiza Wilma Poma / Gonçalves, Victor Lucas / Balancin, Marcelo Luiz / Botelho, Maria Luiza A A / Yamauchi, Fernando Ide / de Mello Ando, Sabrina / Brondani, Vania Balderrama / Chambo, Jose Luis / Fragoso, Maria Candida Barisson Villares / Zerbini, Maria Claudia Nogueira

    Annals of diagnostic pathology

    2022  Volume 57, Page(s) 151888

    Abstract: Adrenal cysts are rare, benign, and usually asymptomatic, being detected as an incidental finding on imaging methods. Adrenal Cysts of Lymphatic Origin (ACLO) and Adrenal Lymphangiomas (AL) are types of endothelial cyst and are the most prevalent subtype ...

    Abstract Adrenal cysts are rare, benign, and usually asymptomatic, being detected as an incidental finding on imaging methods. Adrenal Cysts of Lymphatic Origin (ACLO) and Adrenal Lymphangiomas (AL) are types of endothelial cyst and are the most prevalent subtype in this series. This study aims to present a single institutional experience of these rare cysts and compare their features with those found in the review of existing literature on ACLO and AL. Overall, thirteen cases of adrenal cysts were diagnosed and surgically excised during the study period, onto which we performed immunohistochemistry using a panel of antibodies (CD31, CD34, Pan Cytokeratin AE-1/AE-3, Factor VII, D2-40, and ERG). Four cases of ACLO and two AL were found. The lesions predominantly affected right adrenal, and the majority of patients were middle-age females, of Caucasian ethnicity, and asymptomatic. In our literature review, we found 108 cases of ACLO/AL from 57 articles with similar sex and age distribution. The diagnosis and subclassification of adrenal cysts are challenging, and there is a significant overlapping between the definition of ACLO and AL.
    MeSH term(s) Adrenal Gland Neoplasms/diagnosis ; Adrenal Gland Neoplasms/pathology ; Cysts/pathology ; Female ; Humans ; Immunohistochemistry ; Middle Aged
    Language English
    Publishing date 2022-01-04
    Publishing country United States
    Document type Journal Article ; Systematic Review
    ZDB-ID 1440011-x
    ISSN 1532-8198 ; 1092-9134
    ISSN (online) 1532-8198
    ISSN 1092-9134
    DOI 10.1016/j.anndiagpath.2021.151888
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 5463: Show issues Location:
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  3. Article ; Online: Low Protein Expression of both

    Brondani, Vania Balderrama / Lacombe, Amanda Meneses Ferreira / Mariani, Beatriz Marinho de Paula / Montenegro, Luciana / Soares, Iberê Cauduro / Bezerra-Neto, João Evangelista / Tanno, Fabio Yoshiaki / Srougi, Victor / Chambo, José Luis / Mendonca, Berenice Bilharinho / Almeida, Madson Q / Zerbini, Maria Claudia Nogueira / Fragoso, Maria Candida Barisson Villares

    International journal of molecular sciences

    2021  Volume 22, Issue 3

    Abstract: Adrenocortical carcinoma (ACC) is a rare malignancy that is associated with a dismal prognosis. Pan-genomic studies have demonstrated the involvement ... ...

    Abstract Adrenocortical carcinoma (ACC) is a rare malignancy that is associated with a dismal prognosis. Pan-genomic studies have demonstrated the involvement of
    MeSH term(s) Adolescent ; Adrenal Cortex Neoplasms/metabolism ; Adrenal Cortex Neoplasms/mortality ; Adrenal Cortex Neoplasms/pathology ; Adrenocortical Carcinoma/metabolism ; Adrenocortical Carcinoma/mortality ; Adrenocortical Carcinoma/pathology ; Adult ; Aged ; Aged, 80 and over ; Biomarkers, Tumor/metabolism ; Cohort Studies ; Disease-Free Survival ; Female ; Humans ; Immunohistochemistry ; Kaplan-Meier Estimate ; Ki-67 Antigen/metabolism ; Male ; Middle Aged ; Neoplasm Recurrence, Local/metabolism ; Neoplasm Recurrence, Local/pathology ; Prognosis ; Regression Analysis ; Tissue Array Analysis ; Ubiquitin-Protein Ligases/metabolism ; X-linked Nuclear Protein/metabolism
    Chemical Substances Biomarkers, Tumor ; Ki-67 Antigen ; Ubiquitin-Protein Ligases (EC 2.3.2.27) ; ZNRF3 protein, human (EC 2.3.2.27) ; ATRX protein, human (EC 3.6.4.12) ; X-linked Nuclear Protein (EC 3.6.4.12)
    Language English
    Publishing date 2021-01-27
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2019364-6
    ISSN 1422-0067 ; 1422-0067 ; 1661-6596
    ISSN (online) 1422-0067
    ISSN 1422-0067 ; 1661-6596
    DOI 10.3390/ijms22031238
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Clinical spectrum of Li-Fraumeni syndrome/Li-Fraumeni-like syndrome in Brazilian individuals with the TP53 p.R337H mutation.

    Ferreira, Amanda Meneses / Brondani, Vania Balderrama / Helena, Vanessa Petry / Charchar, Helaine Laiz Silva / Zerbini, Maria Claudia Nogueira / Leite, Luiz Antonio Senna / Hoff, Ana Oliveira / Latronico, Ana Claudia / Mendonca, Berenice Bilharinho / Diz, Maria Del Pilar Estevez / de Almeida, Madson Queiroz / Fragoso, Maria Candida Barisson Villares

    The Journal of steroid biochemistry and molecular biology

    2019  Volume 190, Page(s) 250–255

    Abstract: Background: The TP53 p.R337H germline mutation is highly prevalent among children with adrenocortical tumors (ACTs) from South and Southeast Brazil. However, the prevalence of other tumors of the Li-Fraumeni syndrome (LFS) and Li-Fraumeni-like syndrome ( ...

    Abstract Background: The TP53 p.R337H germline mutation is highly prevalent among children with adrenocortical tumors (ACTs) from South and Southeast Brazil. However, the prevalence of other tumors of the Li-Fraumeni syndrome (LFS) and Li-Fraumeni-like syndrome (LFL) spectrum, the clinical outcomes and the potential tumor occurrence in relatives carrying this distinct TP53 mutation were not fully investigated.
    Patients and methods: We investigated tumor profile data and outcomes of individuals and their close relatives with the TP53 p.R337H germline mutation. A questionnaire and the Toronto protocol were used for evaluation of asymptomatic carriers of this TP53 mutation.
    Results: The cohort of this study comprised 51 patients from 46 different families; 67% were female. All but one harbored the TP53 p.R337H mutation in heterozygous state; only one child was homozygous for this variant. Maternal allele inheritance occurred in 72% of the cases (p= 0,002). In pediatric group, ACT was the most common primary tumor at the diagnosis (55%; median age= 2 years). No patient of the pediatric group who initially presented with ACT developed a second primary tumor and 11% (n= 3) died due to complications related to the primary tumor (median follow-up time of 81.5 months, range= 3-378 months). In adult group, the main tumors at diagnosis were: adrenocortical carcinoma (ACC) (23%; median age= 29.5 years), breast cancer (12%; median age= 38.5 years), soft tissue sarcoma (8%; median age= 50.3 years) and choroid plexus carcinoma (CPC) (2%; median age= 18 years). Among adult patients who were diagnosed with ACC as the first primary tumor, all presented with aggressive disease as per histologic and clinical criteria at diagnosis, and 75% of patients died (median follow-up time of 19 months, range= 1-69 months). Five adult patients (22%) had a second primary tumor, including bronchoalveolar lung cancer (2 cases), ACC, uterine cervical carcinoma and fibrosarcoma. The diagnosis of these tumors was established from 8 to 36 months after the first primary tumor. Three families presented more than one case of ACT. Nine malignant neoplasms were diagnosed in asymptomatic carriers using Toronto protocol.
    Conclusions: This study confirms a high frequency of TP53 p.R337H mutation in pediatric group with ACT. In addition, we observed the occurrence of other tumors of LFS/LFL spectrum and a difference in the aggressiveness of ACTs depending on the age group in which they were diagnosed. The predominance of maternal mutated allele inheritance was first demonstrated in the affected Brazilian's families.
    MeSH term(s) Adolescent ; Adrenal Cortex Neoplasms/epidemiology ; Adrenal Cortex Neoplasms/genetics ; Adult ; Brazil/epidemiology ; Child ; Child, Preschool ; Cohort Studies ; Female ; Germ-Line Mutation ; Humans ; Infant ; Li-Fraumeni Syndrome/epidemiology ; Li-Fraumeni Syndrome/genetics ; Male ; Middle Aged ; Point Mutation ; Tumor Suppressor Protein p53/genetics ; Young Adult
    Chemical Substances TP53 protein, human ; Tumor Suppressor Protein p53
    Language English
    Publishing date 2019-04-08
    Publishing country England
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 1049188-0
    ISSN 1879-1220 ; 0960-0760
    ISSN (online) 1879-1220
    ISSN 0960-0760
    DOI 10.1016/j.jsbmb.2019.04.011
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 708: Show issues Location:
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  5. Article: High Prevalence of Alterations in DNA Mismatch Repair Genes of Lynch Syndrome in Pediatric Patients with Adrenocortical Tumors Carrying a Germline Mutation on

    Brondani, Vania Balderrama / Montenegro, Luciana / Lacombe, Amanda Meneses Ferreira / Magalhães, Breno Marchiori / Nishi, Mirian Yumie / Funari, Mariana Ferreira de Assis / Narcizo, Amanda de Moraes / Cardoso, Lais Cavalca / Siqueira, Sheila Aparecida Coelho / Zerbini, Maria Claudia Nogueira / Denes, Francisco Tibor / Latronico, Ana Claudia / Mendonca, Berenice Bilharinho / Almeida, Madson Queiroz / Lerario, Antonio Marcondes / Soares, Ibere Cauduro / Fragoso, Maria Candida Barisson Villares

    Cancers

    2020  Volume 12, Issue 3

    Abstract: Adrenocortical cancer is a rare malignant neoplasm associated with a dismal prognosis. Identification of the molecular pathways involved in adrenal tumorigenesis is essential for a better understanding of the disease mechanism and improvement of its ... ...

    Abstract Adrenocortical cancer is a rare malignant neoplasm associated with a dismal prognosis. Identification of the molecular pathways involved in adrenal tumorigenesis is essential for a better understanding of the disease mechanism and improvement of its treatment. The aim of this study is to define the prevalence of alterations in DNA mismatch repair (MMR) genes in Lynch syndrome among pediatric patients with adrenocortical neoplasia from southern Brazil, where the prevalence of a specific
    Language English
    Publishing date 2020-03-07
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2527080-1
    ISSN 2072-6694
    ISSN 2072-6694
    DOI 10.3390/cancers12030621
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article: Allelic Variants of

    Mariani, Beatriz Marinho de Paula / Nishi, Mirian Yumie / Wanichi, Ingrid Quevedo / Brondani, Vania Balderrama / Lacombe, Amanda Meneses Ferreira / Charchar, Helaine / Pereira, Maria Adelaide Albergaria / Srougi, Victor / Tanno, Fabio Yoshiaki / Ceccato, Filippo / Regazzo, Daniela / Barbot, Mattia / Occhi, Gianluca / Albiger, Nora Maria Elvira / Vieira-Corrêa, Marcelo / Kater, Claudio Elias / Scaroni, Carla / Chambô, José Luis / Zerbini, Maria Claudia Nogueira /
    Mendonca, Berenice B / Almeida, Madson Q / Fragoso, Maria Candida Barisson Villares

    Frontiers in endocrinology

    2020  Volume 11, Page(s) 36

    Abstract: Objective: ...

    Abstract Objective:
    MeSH term(s) Adrenal Gland Diseases/epidemiology ; Adrenal Gland Diseases/etiology ; Adrenal Gland Diseases/genetics ; Adrenal Gland Neoplasms/epidemiology ; Adrenal Gland Neoplasms/genetics ; Adult ; Alleles ; Armadillo Domain Proteins/genetics ; Case-Control Studies ; Cushing Syndrome/complications ; Cushing Syndrome/epidemiology ; Cushing Syndrome/genetics ; Female ; Genetic Association Studies ; Genetic Predisposition to Disease ; Germ-Line Mutation ; Humans ; Male ; Middle Aged ; Polymorphism, Single Nucleotide ; Retrospective Studies
    Chemical Substances ARMC5 protein, human ; Armadillo Domain Proteins
    Language English
    Publishing date 2020-02-07
    Publishing country Switzerland
    Document type Journal Article ; Multicenter Study ; Research Support, Non-U.S. Gov't
    ZDB-ID 2592084-4
    ISSN 1664-2392
    ISSN 1664-2392
    DOI 10.3389/fendo.2020.00036
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article: Sterol O-Acyl Transferase 1 as a Prognostic Marker of Adrenocortical Carcinoma.

    Lacombe, Amanda Meneses Ferreira / Soares, Iberê Cauduro / Mariani, Beatriz Marinho de Paula / Nishi, Mirian Yumie / Bezerra-Neto, João Evangelista / Charchar, Helaine da Silva / Brondani, Vania Balderrama / Tanno, Fabio / Srougi, Victor / Chambo, José Luiz / Costa de Freitas, Ricardo Miguel / Mendonca, Berenice Bilharinho / Hoff, Ana O / Almeida, Madson Q / Weigand, Isabel / Kroiss, Matthias / Zerbini, Maria Claudia Nogueira / Fragoso, Maria Candida Barisson Villares

    Cancers

    2020  Volume 12, Issue 1

    Abstract: Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with an unfavorable prognosis. Despite the poor prognosis in the majority of patients, no improvements in treatment strategies have been achieved. Therefore, the discovery of new prognostic ... ...

    Abstract Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with an unfavorable prognosis. Despite the poor prognosis in the majority of patients, no improvements in treatment strategies have been achieved. Therefore, the discovery of new prognostic biomarkers is of enormous interest. Sterol-O-acyl transferase 1 (SOAT1) is involved in cholesterol esterification and lipid droplet formation. Recently, it was demonstrated that SOAT1 inhibition leads to impaired steroidogenesis and cell viability in ACC. To date, no studies have addressed the impact of SOAT1 expression on ACC prognosis and clinical outcomes. We evaluated SOAT1 expression by quantitative real-time polymerase chain reaction and immunohistochemistry in a tissue microarray of 112 ACCs (Weiss score ≥ 3) from adults treated in a single tertiary center in Brazil. Two independent pathologists evaluated the immunohistochemistry results through a semiquantitative approach (0-4). We aimed to evaluate the correlation between SOAT1 expression and clinical, biochemical and anatomopathological parameters, recurrence-free survival (RFS), progression-free survival (PFS), and overall survival (OS). SOAT1 protein expression was heterogeneous in this cohort, 37.5% of the ACCs demonstrated a strong SOAT1 protein expression (score > 2), while 62.5% demonstrated a weak or absent protein expression (score ≤ 2). Strong SOAT1 protein expression correlated with features of high aggressiveness in ACC, such as excessive tumor cortisol secretion (
    Language English
    Publishing date 2020-01-19
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2527080-1
    ISSN 2072-6694
    ISSN 2072-6694
    DOI 10.3390/cancers12010247
    Database MEDical Literature Analysis and Retrieval System OnLINE

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