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Complete Remission in Metastatic Pheochromocytoma Treated with Extensive Surgery

Pheochromocytomas are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla. Malignant pheochromocytoma is defined as the presence of metastatic spread in tissues where chromaffin cells are not usually present. This case report describes the case of a relapsed malignant...

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Autores principales: Arnas-Leon, Claudia, Sánchez, Víctor, Santana Suárez, Ana D., Quintana Arroyo, Sara, Acosta, Carmen, Martinez Martin, Francisco Javier
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4744074/
https://www.ncbi.nlm.nih.gov/pubmed/26918215
http://dx.doi.org/10.7759/cureus.447
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author Arnas-Leon, Claudia
Sánchez, Víctor
Santana Suárez, Ana D.
Quintana Arroyo, Sara
Acosta, Carmen
Martinez Martin, Francisco Javier
author_facet Arnas-Leon, Claudia
Sánchez, Víctor
Santana Suárez, Ana D.
Quintana Arroyo, Sara
Acosta, Carmen
Martinez Martin, Francisco Javier
author_sort Arnas-Leon, Claudia
collection PubMed
description Pheochromocytomas are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla. Malignant pheochromocytoma is defined as the presence of metastatic spread in tissues where chromaffin cells are not usually present. This case report describes the case of a relapsed malignant pheochromocytoma, spread to the right liver lobe, superior pole of the right kidney, posterior right hemidiaphragm, right hemidiaphragmatic pillar, inferior vena cava, and regional lymph nodes. After evaluation, an extensive surgery was performed, with resection of all the affected tissues and regional lymphadenectomy. No adjuvant treatment (radiotherapy or chemotherapy) was given. Complete clinical, biochemical, and radiological remission was achieved, with normalisation of metanephrine and normetanephrine. To date, six years after surgery, the patient remains asymptomatic and normotensive without taking any antihypertensive medication. We conclude that the therapeutic approach should be individualized in the case of metastatic pheochromocytoma. Extensive surgery can be considered as a treatment option, even in the case of multiple metastases, as it may be able to achieve complete remission of the disease, avoiding costly and potentially dangerous adjuvant therapies.
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spelling pubmed-47440742016-02-25 Complete Remission in Metastatic Pheochromocytoma Treated with Extensive Surgery Arnas-Leon, Claudia Sánchez, Víctor Santana Suárez, Ana D. Quintana Arroyo, Sara Acosta, Carmen Martinez Martin, Francisco Javier Cureus General Surgery Pheochromocytomas are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla. Malignant pheochromocytoma is defined as the presence of metastatic spread in tissues where chromaffin cells are not usually present. This case report describes the case of a relapsed malignant pheochromocytoma, spread to the right liver lobe, superior pole of the right kidney, posterior right hemidiaphragm, right hemidiaphragmatic pillar, inferior vena cava, and regional lymph nodes. After evaluation, an extensive surgery was performed, with resection of all the affected tissues and regional lymphadenectomy. No adjuvant treatment (radiotherapy or chemotherapy) was given. Complete clinical, biochemical, and radiological remission was achieved, with normalisation of metanephrine and normetanephrine. To date, six years after surgery, the patient remains asymptomatic and normotensive without taking any antihypertensive medication. We conclude that the therapeutic approach should be individualized in the case of metastatic pheochromocytoma. Extensive surgery can be considered as a treatment option, even in the case of multiple metastases, as it may be able to achieve complete remission of the disease, avoiding costly and potentially dangerous adjuvant therapies. Cureus 2016-01-05 /pmc/articles/PMC4744074/ /pubmed/26918215 http://dx.doi.org/10.7759/cureus.447 Text en Copyright © 2016, Arnas-Leon et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle General Surgery
Arnas-Leon, Claudia
Sánchez, Víctor
Santana Suárez, Ana D.
Quintana Arroyo, Sara
Acosta, Carmen
Martinez Martin, Francisco Javier
Complete Remission in Metastatic Pheochromocytoma Treated with Extensive Surgery
title Complete Remission in Metastatic Pheochromocytoma Treated with Extensive Surgery
title_full Complete Remission in Metastatic Pheochromocytoma Treated with Extensive Surgery
title_fullStr Complete Remission in Metastatic Pheochromocytoma Treated with Extensive Surgery
title_full_unstemmed Complete Remission in Metastatic Pheochromocytoma Treated with Extensive Surgery
title_short Complete Remission in Metastatic Pheochromocytoma Treated with Extensive Surgery
title_sort complete remission in metastatic pheochromocytoma treated with extensive surgery
topic General Surgery
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4744074/
https://www.ncbi.nlm.nih.gov/pubmed/26918215
http://dx.doi.org/10.7759/cureus.447
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