medigraphic.com
ISSN 1995-9494 (Electronic)
Revista del Hospital Clínico Quirúrgico "Arnaldo Milián Castro"

2022, Number 1

<< Back Next >>

Acta Med Cent 2022; 16 (1)

Paraganglioma retroperitoneal inter aorto cava. Informe de caso

León AP, Rosales TP, Pila PR, Pila PR

Language: Spanish
References: 14
Page: 165-171
PDF size: 782.42 Kb.


ABSTRACT

Introduction: paragangliomas are very rare extra-adrenal tumors originating from the autonomic nervous system. They are classified in functioning or non-functioning tumors, according to the production of catecholamines.
Patient information: 40-year-old woman, with a history of severe arterial hypertension, who had been treated with three hypotensive drugs without obtaining control; she began to suffer diffuse abdominal pain that increased, associated with hyperpolymenorrhea and a periumbilical tumor of approximately 8 cm, painful and mobile. Laboratory tests were normal. Computed tomography and magnetic resonance imaging confirmed the diagnosis of an 8×5 cm retroperitoneal tumor attached to the aorta and displacing the inferior cava. It was necessary to carry out a highly complex surgical intervention. A tumor was found and the histological and immunohistochemical results confirmed the existence of a non-functioning extra-adrenal paraganglioma.
Conclusions: retroperitoneal paraganglioma is a rare tumor, as shown by statistics. The diagnosis was made, fundamentally, by imaging, histological and immunohistochemical studies. Treatment is fundamentally surgical, as was performed in this patient.


REFERENCES

  1. Mondragón Sánchez A, Mondragón Sánchez R, Alvear Negrete M, Bernal Maldonado R, Mondragón Ballesteros R. Paraganglioma retroperitoneal inter-aorto-cavo. Cir Genl [Internet]. 2004 [citado 20/03/2020];26(4):325-329. https://www.medigraphic.com/pdfs/cirgen/cg-2004/cg044s.pdf

  2. Faria J, Valente V, Lima P, Silva JA, Polónia J. Paraganglioma: a case of secondary hypertension. Rev Port Cardiol [Internet]. 2010 [citado 20/3/2020];29(10):1583-9. Disponible en: https://pubmed.ncbi.nlm.nih.gov/21265497/

  3. Castro-Vega LJ, Letouzé E, Burnichon N, Buffet A, Disderot PH, Khalifa E, et al. Multi-omics analysis defines core genomic alterations in pheochromocytomas and paragangliomas. Nat Commun [Internet]. 2015 [citado 20/3/2020];6:6044. Disponible en: https://www.nature.com/articles/ncomms7044. https://doi.org/10.1038/ncomms7044

  4. Madrigal Rubiales B, Vara Castrodeza A, Fresno Forcelledo M, Ablanedo Ablanedo P. Paragangliomas retroperitoneales extradrenales no secretantes. Arch Esp Urol [Internet]. 2002 [citado 20/3/2020];55(5):543-547. Disponible en: https://aeurologia.com/file.php?d=articlesf&f=d5c19f3bb60b76f09d5ff79c27813344ee3c4c68-es.pdf

  5. Ayllón Terán MD, Torres Lorite M, Benítez Cantero JM, Sánchez Hidalgo JM, Díaz Iglesias C, Rufián Peña S. Causa rara de hipertensión arterial en la juventud: paraganglioma retroperitoneal con invasión vascular. Nefrología (Madr.) [Internet]. 2013 [citado 20/3/2020];33(3):435-437.Disponible en: https://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S0211-69952013000400027. https://dx.doi.org/10.3265/Nefrologia.pre2012.Oct.11744

  6. Remacha L, Comino-Méndez I, Richter S, Contreras L, Currás-Freixes M, Pita G, et al. Targeted exome sequencing of krebs cycle genes reveals candidate cancer-predisposing mutations in pheochromocytomas and paragangliomas. Clin Cancer Res [Internet]. 2017 [citado 20/3/2020];23(20):6315–6324. Disponible en: https://pubmed.ncbi.nlm.nih.gov/28720665/. https://doi.org/10.1158/1078-0432.ccr-16-2250

  7. Yang C, Zhuang Z, Fliedner SMJ, Shankavaram U, Sun MG, Bullova P, et al. Germ-line PHD1 and PHD2 mutations detected in patients with pheochromocytoma/paraganglioma-polycythemia. J Mol Med (Berl) [Internet]. 2015 [citado 20/3/2020];93(1):93–104. Disponible en: https://pubmed.ncbi.nlm.nih.gov/25263965/. https://doi.org/10.1007/s00109-014-1205-7

  8. Eisenhofer G, Klink B, Richter S, Lenders JWM, Robledo M. Metabologenomics of phaeochromocytoma and paraganglioma: An integrated approach for personalised biochemical and genetic testing. Clin Biochem Rev [Internet]. 2017 [citado 20/3/2020];38(2):69–100. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5759086/

  9. Kantorovich V, Pacak K. New insights on the pathogenesis of paraganglioma and pheochromocytoma. F1000Res [Internet]. 2018 [citado 20/3/2020];7:F1000 Faculty Rev-1500. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6173107/. https://dx.doi.org/10.12688/f1000research.14568.1

  10. Jimenez C. Treatment for patients with malignant pheochromocytomas and paragangliomas: A perspective from the hallmarks of cancer. Front Endocrinol (Lausanne) [Internet]. 2018 [citado 20/3/2020];9:277. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5985332/. https://dx.doi.org/10.3389/fendo.2018.00277

  11. Toledo RA, Qin Y, Cheng ZM, Gao Q, Iwata S, Silva GM, et al. Recurrent mutations of chromatin-remodeling genes and kinase receptors in pheochromocytomas and paragangliomas. Clin Cancer Res [Internet]. 2016 [citado 20/3/2020];22(9):2301–2310. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4854762/. https://dx.doi.org/10.1158/1078-0432.CCR-15-1841

  12. Ben Aim L, Pigny P, Castro-Vega LJ, Buffet A, Amar L, Bertherat J, et al. Targeted next-generation sequencing detects rare genetic events in pheochromocytoma and paraganglioma. J Med Genet [Internet]. 2019 [citado 20/3/2020];56(8):513-520. Disponible en: https://pubmed.ncbi.nlm.nih.gov/30877234/. https://doi.org/10.1136/jmedgenet-2018-105714

  13. Job S, Draskovic I, Burnichon N, Buffet A, Cros J, Lépine C, et al. Telomerase activation and ATRX mutations are independent risk factors for metastatic pheochromocytoma and paraganglioma. Clin Cancer Res [Internet]. 2019 [citado 20/3/2020];25(2):760–770. Disponible en: https://clincancerres.aacrjournals.org/content/25/2/760.full-text.pdf. https://doi.org/10.1158/1078-0432.CCR-18-0139

  14. Lenders JWM, Eisenhofer G. Update on modern management of pheochromocytoma and paraganglioma. Endocrinol Metab (Seoul) [Internet]. 2017 [citado 20/3/2020];32(2):152-161. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5503859/. https://dx.doi.org/10.3803/EnM.2017.32.2.152




2020     |     www.medigraphic.com