Romero-Uribe JL, Chaidez-Mendivil M, Camacho-Castro AJ

Language: Spanish
References: 11
Page: 1-12
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ABSTRACT

Clinical case: a 51-year-old female patient, with a history of controlled type 2 Diabetes mellitus, reported diaphoresis, headache, fine tremors of 6 months’ duration, grade I obesity, stretch marks on the abdominal wall and cortisol of 1189 µg/dL. A CT scan showed an increase in the size of both adrenal glands. Patient with clinical data of Cushing’s syndrome, it was decided to perform laparoscopic left adrenalectomy, without complications. During follow-up, there was a significant decrease in cortisol and she was asymptomatic with significant improvement.
Relevance: primary bilateral macronodular adrenal hyperplasia (PHAMBP) is a rare cause of Cushing’s syndrome less than 2 % and is often diagnosed by bilateral adrenal incidentalomas with hypercortisolism. Characterized by bilateral benign adrenal macronodules larger than 1 cm responsible for elevated cortisol levels. In this report we describe a case of this condition undergoing unilateral laparoscopic adrenalectomy.
Clinical implications: the importance lies in a multidisciplinary approach to make a correct diagnosis, to avoid the progression of symptoms with the possibility of clinical and biochemical remission with adrenalectomy.
Conclusions: unilateral resection of the largest adrenal gland can result in clinical and biochemical remission in ›90 % of cases, with laparoscopic adrenalectomy being the treatment of choice.

REFERENCES

1. Tang P, Zhang J, Peng S, Yan X, Wang Y,Wang S, et al. Primary bilateral macronodularadrenocortical hyperplasia (PBMAH) patientwith ARMC5 mutations. BMC EndocrineDisorders. 2023;23: 77. https://doi.org/10.1186/s12902-023-01324-3.

2. Vassiliadi DA, Tsagarakis S. Diagnosis andmanagement of primary bilateral macronodularadrenal hyperplasia. Endocrine-Related Cancer.2019;26(10): R567–R581. https://doi.org/10.1530/ERC-19-0240.

3. Araujo-Castro M, Iturregui Guevara M,Calatayud Gutiérrez M, Parra Ramírez P, GraciaGimeno P, Hanzu FA, et al. Practical guide onthe initial evaluation, follow-up, and treatmentof adrenal incidentalomas Adrenal DiseasesGroup of the Spanish Society of Endocrinologyand Nutrition. Endocrinologia, Diabetes YNutricion. 2020;67(6): 408–419. https://doi.org/10.1016/j.endinu.2020.03.002.

4. Di Dalmazi G, Reincke M. Adrenal Surgeryfor Cushing’s Syndrome. Endocrinology andMetabolism Clinics of North America. 2018;47(2):385–394. https://doi.org/10.1016/j.ecl.2018.01.004.

5. Charchar HLS, Fragoso MCBV. An Overview ofthe Heterogeneous Causes of Cushing SyndromeResulting From Primary Macronodular AdrenalHyperplasia (PMAH). Journal of the EndocrineSociety. 2022;6(5): bvac041. https://doi.org/10.1210/jendso/bvac041.

6. Bourdeau I, El Ghorayeb N, Gagnon N, LacroixA. MANAGEMENT OF ENDOCRINE DISEASE:Differential diagnosis, investigation and therapyof bilateral adrenal incidentalomas. EuropeanJournal of Endocrinology. 2018;179(2): R57–R67. https://doi.org/10.1530/EJE-18-0296.

7. Michelle M A, Jensen CT, Habra MA, MeniasCO, Shaaban AM, Wagner-Bartak NA, et al.Adrenal cortical hyperplasia: diagnostic workup,subtypes, imaging features and mimics. BritishJournal of Radiology. 2017;90(1079): 20170330.https://doi.org/10.1259/bjr.20170330.

8. Yu Z, Gao J, Sun F. The effect of unilateraladrenalectomy on patients with primary bilateralmacronodular adrenal hyperplasia. Hormones(Athens, Greece). 2023;22(2): 235–242. https://doi.org/10.1007/s42000-023-00428-8.

9. Bouys L, Chiodini I, Arlt W, Reincke M,Bertherat J. Update on primary bilateralmacronodular adrenal hyperplasia (PBMAH).Endocrine. 2021;71(3): 595–603. https://doi.org/10.1007/s12020-021-02645-w.

10. Chevalier B, Vantyghem MC, EspiardS. Bilateral Adrenal Hyperplasia:Pathogenesis and Treatment. Biomedicines.2021;9(10): 1397. https://doi.org/10.3390/biomedicines9101397.

11. Yoshiaki Tanno F, Srougi V, Almeida MQ,Ide Yamauchi F, Morbeck Almeida Coelho F,Nishi MY, et al. A New Insight into the SurgicalTreatment of Primary Macronodular AdrenalHyperplasia. Journal of the Endocrine Society.2020;4(8): bvaa083. https://doi.org/10.1210/jendso/bvaa083.