ISSN: 0443-511
e-ISSN: 2448-5667
Usuario/a
Idioma
Herramientas del artículo
Envíe este artículo por correo electrónico (Inicie sesión)
Enviar un correo electrónico al autor/a (Inicie sesión)
Tamaño de fuente

Open Journal Systems

Incidentaloma adrenal bilateral en un paciente con apendicitis aguda / Bilateral adrenal incidentaloma in a patient with acute appendicitis

José Luis Paz-Ibarra, Jacsel Suarez- Rojas, Víctor Raúl García-Ruíz, Julio César Álvarez-Gamero, José Somocurcio-Peralta

Resumen


Resumen

Introducción: los incidentalomas adrenales son tumores que suelen ser detectados en estudios de imágenes indicados sin sospecha de enfermedad adrenal. El feocromocitoma es un tumor neuroendocrino que puede presentarse esporádicamente o asociado a síndromes genéticos, habiendo sido descrito en 0.1-5% de pacientes con neurofibromatosis tipo 1, que es una enfermedad progresiva multisistémica de herencia autosómica dominante que afecta a 1/2600-3000 individuos. 
Caso clínico: se reporta el caso de un paciente de 50 años quien ingresó al departamento de Emergencia por apendicitis aguda y cuya tomografía mostró la presencia de masas adrenales bilaterales. Luego de la intervención quirúrgica fue hospitalizado para estudio. El paciente negó cefalea, diaforesis, hipertensión arterial, palpitaciones y pérdida de peso; en el examen físico se evidenció presencia de múltiples lentígines, manchas café con leche > 15 mm, lesiones nodulares fijas y pedunculadas, compatibles con neurofibromas focales, sin signos sugestivos de hipo o hipercortisolismo. En la analítica se identificaron metanefrinas totales y ácido vanilil-mandélico en orina encontrándose en valores muy elevados, siendo sometido a adrenalectomía laparoscópica en 2 tiempos, luego de una adecuada preparación que incluyó bloqueo alfa y beta adrenérgicos.
Conclusiones: en los pacientes con masas adrenales es necesario reconocer la importancia de una evaluación clínica integral para guiar un adecuado estudio diagnóstico, así como la realización de un óptimo estudio preoperatorio que incluye las pruebas hormonales para descartar funcionalidad.

 

Abstract

Background: Adrenal incidentalomas are tumors that are usually detected in imaging studies indicated without suspicion of adrenal disease. Pheochromocytoma is a neuroendocrine tumor that can occur sporadically or associated with genetic syndromes, having been described in 0.1-5% of patients with type 1 neurofibromatosis, which is a progressive multisystemic disease of autosomal dominant inheritance that affects 1 / 2600-3000 individuals .

Clinical case: We present the case of a 50-year-old patient who was admitted to the Emergency Department for acute appendicitis and whose CT scan showed the presence of bilateral adrenal masses is reported. After the surgical intervention, he was hospitalized for study. The patient denied headache, sweating, hypertension, palpitations, and weight loss; Physical examination revealed the presence of multiple lentigines, café-au-lait spots > 15 mm, fixed and pedunculated nodular lesions, compatible with focal neurofibromas, without signs suggestive of hypo or hypercortisolism. In the analysis, total metanephrines and vanillyl-mandelic acid were identified in urine, they were found in very high values, being subjected to laparoscopic adrenalectomy in 2 stages, after adequate preparation that included alpha and beta adrenergic blockade.

Conclusions: In patients with adrenal masses, it is necessary to recognize the importance of a comprehensive clinical evaluation to guide an adequate diagnostic study, as well as the performance of an optimal preoperative study that includes hormonal tests to rule out functionality.

 


Palabras clave


Feocromocitoma; Neoplasias de las Glándulas Suprarrenales; Neurofibromatosis 1 / Pheochromocytoma; Adrenal Gland Neoplasms; Neurofibromatosis 1

Texto completo:

PDF

Referencias


 

Fassnacht M, Arlt W, Bancos I, Dralle H, Newell-Price J, Sahdev A, et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2016;175(2):G1-34. DOI: 10.1530/EJE-16-0467

 

Bourdeau I, El Ghorayeb N, Gagnon N, Lacroix A. Management of endocrine disease: Differential diagnosis, investigation and therapy of bilateral adrenal incidentalomas. Eur J Endocrinol. 2018;179(2):R57-67. DOI: 10.1530/EJE-18-0296

 

Canu L, Parenti G, De Filpo G, Mannelli M. Pheochromocytomas and paragangliomas as causes of endocrine hypertension. Front Endocrinol. 2019;10(JUN):1-5. DOI: 10.3389/fendo.2019.00333

 

Koopman K, Gaal J, de Krijger RR. Pheochromocytomas and Paragangliomas: New Developments with Regard to Classification, Genetics, and Cell of Origin. Cancers. 2019;11(8):1070. DOI: 10.3390/cancers11081070. DOI: 10.1016/j.mcna.2019.07.004

 

Ly KI, Blakeley JO. The Diagnosis and Management of Neurofibromatosis Type 1. Med Clin North Am. 2019;103(6):1035-54. DOI: 10.1186/s40842-018-0065-4

 

Petr EJ, Else T. Pheochromocytoma and Paraganglioma in Neurofibromatosis type 1: frequent surgeries and cardiovascular crises indicate the need for screening. Clin Diabetes Endocrinol. 2018;4(1):1-5. DOI: 10.1186/s40842-018-0065-4

 

March MR. Type 1 neurofibromatosis and pheochromocytoma: Focus on hypertension. J Neurosci Rural Pract. 2012;3(1):107-8. DOI: 10.4103/0976-3147.91987

 

Barzon L, Sonino N, Fallo F, Palù G, Boscaro M. Prevalence and natural history of adrenal incidentalomas. Eur J Endocrinol. 2003;149(4):273-85. DOI: 10.1530/eje.0.1490273

 

Amodru V, Taieb D, Guerin C, Paladino NC, Brue T, Sebag F, et al. Large adrenal incidentalomas require a dedicated diagnostic procedure. Endocr Pract Off J Am Coll Endocrinol Am Assoc Clin Endocrinol. 2019;25(7):669-77. DOI: 10.4158/EP-2018-0616

 

Angeli A, Osella G, Alì A, Terzolo M. Adrenal incidentaloma: an overview of clinical and epidemiological data from the National Italian Study Group. Horm Res. 1997;47(4- 6):279-83. DOI: 10.1159/000185477

 

Kulis T, Knezevic N, Pekez M, Kastelan D, Grkovic M, Kastelan Z. Laparoscopic adrenalectomy: lessons learned from 306 cases. J Laparoendosc Adv Surg Tech A. 2012;22(1):22-6. DOI: 10.1089/lap.2011.0376

 

Patócs A, Karádi E, Tóth M, Varga I, Szücs N, Balogh K, et al. Clinical and biochemical features of sporadic and hereditary phaeochromocytomas: an analysis of 41 cases investigated in a single endocrine centre. Eur J Cancer Prev. 2004;13(5):403- 9. DOI: 10.1097/00008469-200410000-00008

 

Humphrey R, Gray D, Pautler S, Davies W. Laparoscopic compared with open adrenalectomy for resection of pheochromocytoma: a review of 47 cases. Can J Surg. 2008;51(4):276-80.

 

Thompson LDR. Pheochromocytoma of the Adrenal gland Scaled Score (PASS) to separate benign from malignant neoplasms: a clinicopathologic and immunophenotypic study of 100 cases. Am J Surg Pathol. 2002;26(5):551-66. DOI: 10.1097/00000478- 200205000-00002

 

Feng F, Zhu Y, Wang X, Wu Y, Zhou W, Jin X, et al. Predictive factors for malignant pheochromocytoma: analysis of 136 patients. J Urol. 2011;185(5):1583-90. DOI: 10.1016/j.juro.2010.12.050

 

Hamidi O, Young WF, Iñiguez-Ariza NM, Kittah NE, Gruber L, Bancos C, et al. Malignant pheochromocytoma and paraganglioma: 272 patients over 55 years. J Clin Endocrinol Metab. 2017;102(9):3296-305. DOI: 10.1210/jc.2017-00992

 

Mantero F, Terzolo M, Arnaldi G, Osella G, Masini AM, Alì A, et al. A survey on adrenal incidentaloma in Italy. J Clin Endocrinol Metab. 2000;85(2):637-44. DOI: 10.1210/jcem.85.2.6372

 

Lenders JWM, Duh Q-Y, Eisenhofer G, Gimenez-Roqueplo A-P, Grebe SKG, Murad MH, et al. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014;99(6):1915-42. DOI: 10.1210/jc.2014-1498

 

Evans DG, Howard E, Giblin C, Clancy T, Spencer H, Huson SM, et al. Birth incidence and prevalence of tumor-prone syndromes: estimates from a UK family genetic register service. Am J Med Genet A. 2010;152A(2):327-32. DOI: 10.1002/ajmg.a.33139

 

Guller U, Turek J, Eubanks S, Delong ER, Oertli D, Feldman JM. Detecting pheochromocytoma: defining the most sensitive test. Ann Surg. 2006;243(1):102-7. DOI: 10.1097/01.sla.0000193833.51108.24

 

Easton DF, Ponder MA, Huson SM, Ponder BA. An analysis of variation in expression of neurofibromatosis (NF) type 1 (NF1): evidence for modifying genes. Am J Hum Genet. 1993;53(2):305-13.

 

Gutmann DH, Ferner RE, Listernick RH, Korf BR, Wolters PL, Johnson KJ. Neurofibromatosis type 1. Nat Rev Dis Primer. 2017;3:17004. DOI: 10.1038/nrdp.2017.4.

 

Zografos GN, Vasiliadis GK, Zagouri F, Aggeli C, Korkolis D, Vogiaki S, et al. Pheochromocytoma associated with neurofibromatosis type 1: Concepts and current trends. World J Surg Oncol. 2010;8:14-7. DOI: 10.1186/1477-7819-8-14

 

Castinetti F, Taieb D, Henry JF, Walz M, Guerin C, Brue T, et al. Management of endocrine disease: Outcome of adrenal sparing surgery in heritable pheochromocytoma. Eur J Endocrinol. 2016;174(1):R9-18. DOI: 10.1530/EJE-15-0549

 

Kittah NE, Gruber LM, Bancos I, Hamidi O, Tamhane S, Iñiguez-Ariza N, et al. Bilateral pheochromocytoma: Clinical characteristics, treatment and longitudinal follow- up. Clin Endocrinol (Oxf). 2020;93(3):288-95. DOI: 10.1111/cen.14222

 


Enlaces refback

  • No hay ningún enlace refback.