Cargando…

FRI146 Stereotactic Ablative Radiotherapy As A Local Valuable Therapeutic Procedure To Control Aldosterone-Secreting Adrenocortical Carcinoma

Disclosure: L.M. Mermejo: None. R.H. Zanella: None. L. Cocicov: None. C.A. Molina: None. S. Tucci Jr: None. J. Elias Jr: None. V.F. Muglia: None. G. Viani: None. P.C. Elias: None. A.C. Moreira: None. M. De Castro: None. Aldosterone-secreting adrenocortical carcinomas (ACC) are rare and usually descr...

Descripción completa

Detalles Bibliográficos
Autores principales: Mara Mermejo, Livia, Zanella, Renato H, Cocicov, Larissa, AF Molina, Carlos, Tucci, Silvio, Elias, Jorge, Muglia, Valdair F, Viani, Gustavo, Elias, Paula C L, Custodio Moreira, Ayrton, De Castro, Margaret
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10555029/
http://dx.doi.org/10.1210/jendso/bvad114.658
_version_ 1785116557127450624
author Mara Mermejo, Livia
Zanella, Renato H
Cocicov, Larissa
AF Molina, Carlos
Tucci, Silvio
Elias, Jorge
Muglia, Valdair F
Viani, Gustavo
Elias, Paula C L
Custodio Moreira, Ayrton
De Castro, Margaret
author_facet Mara Mermejo, Livia
Zanella, Renato H
Cocicov, Larissa
AF Molina, Carlos
Tucci, Silvio
Elias, Jorge
Muglia, Valdair F
Viani, Gustavo
Elias, Paula C L
Custodio Moreira, Ayrton
De Castro, Margaret
author_sort Mara Mermejo, Livia
collection PubMed
description Disclosure: L.M. Mermejo: None. R.H. Zanella: None. L. Cocicov: None. C.A. Molina: None. S. Tucci Jr: None. J. Elias Jr: None. V.F. Muglia: None. G. Viani: None. P.C. Elias: None. A.C. Moreira: None. M. De Castro: None. Aldosterone-secreting adrenocortical carcinomas (ACC) are rare and usually described as large tumors. We report the case of a 44-year-old man referred to our University Hospital presenting back pain, muscular weakness and hypokalemia. A year earlier, hypertension had been diagnosed triggered by the investigation of daily headaches. Throughout this period, hypertension had been refractory to antihypertensives and patient also showed progressive weight loss of 10 kg. At the time of presentation, the patient had no signs of cortisol or androgen excess, his blood pressure was 180x120 mmHg, and there was no peripheral edema or abdominal mass detected. Due to abdominal pain and liver enzymes abnormalities, an abdominal CT was performed and revealed a heterogeneously 10 x 8 x 6 cm (volume: 256 cm³) left adrenal mass compressing the left renal vein with invasion through the inferior cava vein and extending to the liver caudate lobe. He also had a sodium of 143 mmol/L, potassium of 1.2 mmol/L and metabolic alkalosis. The serum aldosterone level of 75 ng/dL and plasma direct renin concentration < 2 mIU/L whereas urinary fractionated metanephrines were within normal limits, suggesting a probable aldosterone-secreting ACC. His family history was negative for hypertension or adrenal diseases. The patient was initially treated with spironolactone and potassium replacement with clinical improvement and an open adrenalectomy was proposed. However, patient professed to be Jehovah's Witnesses and refused the surgery due to the risk of blood transfusions. Therefore, the patient received mitotane but owing to hepatotoxity, the drug was withdrawn after two months of follow-up. A follow up abdominal MRI after 10 months of diagnosis revealed an increase in the mass (12 x 8 x 9 cm; volume: 460 cm³) and, now, extending to the left kidney. A CT-guided percutaneous biopsy showed an ACC with a Weiss score of three and the immunohistochemistry was positive for vimentin, inhibin and MART-1/ Melan A. After a multidisciplinary discussion, it was proposed to use stereotactic ablative radiotherapy (SABR) as an alternative option. The patient accepted SABR, being treated with 35Gy/5 fractions. During the follow-up, the patients had a progressive improvement of pain complaints, reduction of tumor burden after eighteen months on control abdomen CT (9.1 x 4.9 x 8.7 cm; volume of 202 cm³), improvement of blood pressure and hypokalemia levels, reduction of antihypertensive drugs defined daily dose and potassium replacement as well as reduction of a serum aldosterone to 13 ng/dL and increased of paired renin to 25 mU/L. This is the first published report of a pure aldosterone-producing ACC patient receiving SABR. The response was significant, indicating that the use of SABR should be considered as an alternative for local treatment of aldosterone-secreting ACC when the surgery cannot be indicated. Presentation: Friday, June 16, 2023
format Online
Article
Text
id pubmed-10555029
institution National Center for Biotechnology Information
language English
publishDate 2023
publisher Oxford University Press
record_format MEDLINE/PubMed
spelling pubmed-105550292023-10-06 FRI146 Stereotactic Ablative Radiotherapy As A Local Valuable Therapeutic Procedure To Control Aldosterone-Secreting Adrenocortical Carcinoma Mara Mermejo, Livia Zanella, Renato H Cocicov, Larissa AF Molina, Carlos Tucci, Silvio Elias, Jorge Muglia, Valdair F Viani, Gustavo Elias, Paula C L Custodio Moreira, Ayrton De Castro, Margaret J Endocr Soc Cardiovascular Endocrinology Disclosure: L.M. Mermejo: None. R.H. Zanella: None. L. Cocicov: None. C.A. Molina: None. S. Tucci Jr: None. J. Elias Jr: None. V.F. Muglia: None. G. Viani: None. P.C. Elias: None. A.C. Moreira: None. M. De Castro: None. Aldosterone-secreting adrenocortical carcinomas (ACC) are rare and usually described as large tumors. We report the case of a 44-year-old man referred to our University Hospital presenting back pain, muscular weakness and hypokalemia. A year earlier, hypertension had been diagnosed triggered by the investigation of daily headaches. Throughout this period, hypertension had been refractory to antihypertensives and patient also showed progressive weight loss of 10 kg. At the time of presentation, the patient had no signs of cortisol or androgen excess, his blood pressure was 180x120 mmHg, and there was no peripheral edema or abdominal mass detected. Due to abdominal pain and liver enzymes abnormalities, an abdominal CT was performed and revealed a heterogeneously 10 x 8 x 6 cm (volume: 256 cm³) left adrenal mass compressing the left renal vein with invasion through the inferior cava vein and extending to the liver caudate lobe. He also had a sodium of 143 mmol/L, potassium of 1.2 mmol/L and metabolic alkalosis. The serum aldosterone level of 75 ng/dL and plasma direct renin concentration < 2 mIU/L whereas urinary fractionated metanephrines were within normal limits, suggesting a probable aldosterone-secreting ACC. His family history was negative for hypertension or adrenal diseases. The patient was initially treated with spironolactone and potassium replacement with clinical improvement and an open adrenalectomy was proposed. However, patient professed to be Jehovah's Witnesses and refused the surgery due to the risk of blood transfusions. Therefore, the patient received mitotane but owing to hepatotoxity, the drug was withdrawn after two months of follow-up. A follow up abdominal MRI after 10 months of diagnosis revealed an increase in the mass (12 x 8 x 9 cm; volume: 460 cm³) and, now, extending to the left kidney. A CT-guided percutaneous biopsy showed an ACC with a Weiss score of three and the immunohistochemistry was positive for vimentin, inhibin and MART-1/ Melan A. After a multidisciplinary discussion, it was proposed to use stereotactic ablative radiotherapy (SABR) as an alternative option. The patient accepted SABR, being treated with 35Gy/5 fractions. During the follow-up, the patients had a progressive improvement of pain complaints, reduction of tumor burden after eighteen months on control abdomen CT (9.1 x 4.9 x 8.7 cm; volume of 202 cm³), improvement of blood pressure and hypokalemia levels, reduction of antihypertensive drugs defined daily dose and potassium replacement as well as reduction of a serum aldosterone to 13 ng/dL and increased of paired renin to 25 mU/L. This is the first published report of a pure aldosterone-producing ACC patient receiving SABR. The response was significant, indicating that the use of SABR should be considered as an alternative for local treatment of aldosterone-secreting ACC when the surgery cannot be indicated. Presentation: Friday, June 16, 2023 Oxford University Press 2023-10-05 /pmc/articles/PMC10555029/ http://dx.doi.org/10.1210/jendso/bvad114.658 Text en © The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs licence (https://creativecommons.org/licenses/by-nc-nd/4.0/), which permits non-commercial reproduction and distribution of the work, in any medium, provided the original work is not altered or transformed in any way, and that the work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Cardiovascular Endocrinology
Mara Mermejo, Livia
Zanella, Renato H
Cocicov, Larissa
AF Molina, Carlos
Tucci, Silvio
Elias, Jorge
Muglia, Valdair F
Viani, Gustavo
Elias, Paula C L
Custodio Moreira, Ayrton
De Castro, Margaret
FRI146 Stereotactic Ablative Radiotherapy As A Local Valuable Therapeutic Procedure To Control Aldosterone-Secreting Adrenocortical Carcinoma
title FRI146 Stereotactic Ablative Radiotherapy As A Local Valuable Therapeutic Procedure To Control Aldosterone-Secreting Adrenocortical Carcinoma
title_full FRI146 Stereotactic Ablative Radiotherapy As A Local Valuable Therapeutic Procedure To Control Aldosterone-Secreting Adrenocortical Carcinoma
title_fullStr FRI146 Stereotactic Ablative Radiotherapy As A Local Valuable Therapeutic Procedure To Control Aldosterone-Secreting Adrenocortical Carcinoma
title_full_unstemmed FRI146 Stereotactic Ablative Radiotherapy As A Local Valuable Therapeutic Procedure To Control Aldosterone-Secreting Adrenocortical Carcinoma
title_short FRI146 Stereotactic Ablative Radiotherapy As A Local Valuable Therapeutic Procedure To Control Aldosterone-Secreting Adrenocortical Carcinoma
title_sort fri146 stereotactic ablative radiotherapy as a local valuable therapeutic procedure to control aldosterone-secreting adrenocortical carcinoma
topic Cardiovascular Endocrinology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10555029/
http://dx.doi.org/10.1210/jendso/bvad114.658
work_keys_str_mv AT maramermejolivia fri146stereotacticablativeradiotherapyasalocalvaluabletherapeuticproceduretocontrolaldosteronesecretingadrenocorticalcarcinoma
AT zanellarenatoh fri146stereotacticablativeradiotherapyasalocalvaluabletherapeuticproceduretocontrolaldosteronesecretingadrenocorticalcarcinoma
AT cocicovlarissa fri146stereotacticablativeradiotherapyasalocalvaluabletherapeuticproceduretocontrolaldosteronesecretingadrenocorticalcarcinoma
AT afmolinacarlos fri146stereotacticablativeradiotherapyasalocalvaluabletherapeuticproceduretocontrolaldosteronesecretingadrenocorticalcarcinoma
AT tuccisilvio fri146stereotacticablativeradiotherapyasalocalvaluabletherapeuticproceduretocontrolaldosteronesecretingadrenocorticalcarcinoma
AT eliasjorge fri146stereotacticablativeradiotherapyasalocalvaluabletherapeuticproceduretocontrolaldosteronesecretingadrenocorticalcarcinoma
AT mugliavaldairf fri146stereotacticablativeradiotherapyasalocalvaluabletherapeuticproceduretocontrolaldosteronesecretingadrenocorticalcarcinoma
AT vianigustavo fri146stereotacticablativeradiotherapyasalocalvaluabletherapeuticproceduretocontrolaldosteronesecretingadrenocorticalcarcinoma
AT eliaspaulacl fri146stereotacticablativeradiotherapyasalocalvaluabletherapeuticproceduretocontrolaldosteronesecretingadrenocorticalcarcinoma
AT custodiomoreiraayrton fri146stereotacticablativeradiotherapyasalocalvaluabletherapeuticproceduretocontrolaldosteronesecretingadrenocorticalcarcinoma
AT decastromargaret fri146stereotacticablativeradiotherapyasalocalvaluabletherapeuticproceduretocontrolaldosteronesecretingadrenocorticalcarcinoma