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High accuracy of bilateral and simultaneous petrosal sinus sampling with desmopressin for the differential diagnosis of pediatric ACTH-dependent Cushing’s syndrome

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Abstract

Purpose

To analyze the bilateral and simultaneous petrosal sinus sampling (BIPSS) in a subgroup of children and adolescents with ACTH-dependent Cushing’s syndrome (ADCS)

Methods

Retrospective study in a tertiary reference center. From 1993 and 2017, 19 children and adolescents (PED) were submitted to the BIPSS, median age of 14 years (range 9–19 years), 53% were males, 18 had Cushing’s disease (CD) and one had ectopic ACTH syndrome (EAS). All procedures were performed with 10 µg of intravenous desmopressin.

Results

The catheter positioning was successful in all cases. The central ACTH gradient was met in 17/19 cases. At baseline, central gradient occurred in 16/19 (84%) with gradient values of 7.2 ± 6.0. After stimulation, there was an increase in the center-periphery gradient values (33.6 ± 44.3). In one case, central gradient was defined only after stimulation. Two cases presented without a central gradient; one case of CD with a false-negative and one EAS case. Lateralization occurred in all cases with a central gradient. Confirmation of the tumor location presumed by the procedure with the surgical description occurred in 60% of the cases. The BIPSS in this PED subgroup of ADCS presented a sensitivity of 94.4% and specificity of 100%. There were no complications of the procedure.

Conclusion

In a series of children and adolescents with ADCS, BIPSS was safe and highly accurate in defining the central to peripheral ACTH gradient using desmopressin as secretagogue. Nevertheless, there was a limited value of the ACTH-gradient between the petrosal sinuses for the tumor location.

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References

  1. Lodish MB, Keil MF, Stratakis CA (2018) Cushing's syndrome in pediatrics: an update. Endocrinol Metab Clin N Am 47(2):451–462

    Article  Google Scholar 

  2. Lacroix A, Feelders RA, Stratakis CA, Nieman LK (2015) Cushing's syndrome. Lancet 386(9996):913–927

    Article  CAS  Google Scholar 

  3. More J, Young J, Reznik Y, Raverot G, Borson-Chazot F, Rohmer V, Baudin E, Coutant R, Tabarin A, Groupe Français des Tumeurs Endocrines (GTE) (2011) Ectopic ACTH syndrome in children and adolescents. J Clin Endocrinol Metab 96(5):1213–1222

    Article  PubMed  CAS  Google Scholar 

  4. Chen S, Chen K, Lu L, Zhang X, Tong A, Pan H, Zhu H, Lu Z (2019) The effects of sampling lateralization on bilateral inferior petrosal sinus sampling and desmopressin stimulation test for pediatric Cushing's disease. Endocrine 63(3):582–591

    Article  PubMed  CAS  Google Scholar 

  5. Puglia P Jr, Caldas JG, Barbosa LA, Sá AT Jr, Machado MC, Salgado LR (2008) Inferior petrosal sinus catheterization: technical aspects. Arq Bras Endocrinol Metabol 52(4):692–696

    Article  PubMed  Google Scholar 

  6. Magiakou MA, Mastorakos G, Oldfield EH, Gomez MT, Doppman JL, Cutler GB Jr, Nieman LK, Chrousos GP (1994) Cushing's syndrome in children and adolescents. Presentation, diagnosis, and therapy. N Engl J Med. 331(10):629–636

    Article  PubMed  CAS  Google Scholar 

  7. He L, Ladner TR, Pruthi S, Day MA, Desai AA, Jordan LC, Froehler MT (2016) Rule of 5: angiographic diameters of cervicocerebral arteries in children and compatibility with adult neurointerventional devices. J Neurointerv Surg 8(10):1067–1071

    Article  PubMed  Google Scholar 

  8. Batista D, Gennari M, Riar J, Chang R, Keil MF, Oldfield EH, Stratakis CA (2006) An assessment of petrosal sinus sampling for localization of pituitary microadenomas in children with Cushing disease. J Clin Endocrinol Metab 91(1):221–224

    Article  PubMed  CAS  Google Scholar 

  9. Lonser RR, Wind JJ, Nieman LK, Weil RJ, DeVroom HL, Oldfield EH (2013) Outcome of surgical treatment of 200 children with Cushing's disease. J Clin Endocrinol Metab 98(3):892–901

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  10. Storr HL, Savage MO (2015) Management of endocrine disease: Paediatric Cushing's disease. Eur J Endocrinol 173(1):R35–45

    Article  PubMed  CAS  Google Scholar 

  11. Storr HL, Afshar F, Matson M, Sabin I, Davies KM, Evanson J, Plowman PN, Besser GM, Monson JP, Grossman AB, Savage MO (2005) Factors influencing cure by transsphenoidal selective adenomectomy in paediatric Cushing's disease. Eur J Endocrinol 152(6):825–833

    Article  PubMed  CAS  Google Scholar 

  12. Machado MC, Sa SV, Domenice S, Fragoso MCBV, Puglia P Jr, Pereira MAA, Mendonça BB, Salgado LR (2007) The role of desmopressin in bilateral and simultaneous inferior petrosal sinus sampling for differential diagnosis of ACTH-dependent Cushing's syndrome. Clin Endocrinol (Oxf) 66(1):136–142

    CAS  Google Scholar 

  13. Suda T, Tozawa F, Dobashi I, Horiba N, Ohmori N, Yamakado M, Yamada M, Demura H (1993) Corticotropin-releasing hormone, proopiomelanocortin, and glucocorticoid receptor gene expression in adrenocorticotropin-producing tumors in vitro. J Clin Invest 92(6):2790–2795

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  14. Luque RM, Ibáñez-Costa A, López-Sánchez LM, Jiménez-Reina L, Venegas-Moreno E, Gálvez MA, Villa-Osaba A, Madrazo-Atutxa AM, Japón MA, de la Riva A, Cano DA, Benito-López P, Soto-Moreno A, Gahete MD, Leal-Cerro A, Castaño JP (2013) A cellular and molecular basis for the selective desmopressin-induced ACTH release in Cushing disease patients: key role of AVPR1b receptor and potential therapeutic implications. J Clin Endocrinol Metab 98(10):4160–4169

    Article  PubMed  CAS  Google Scholar 

  15. Tsagarakis S, Vassiliadi D, Kaskarelis IS, Komninos J, Souvatzoglou E, Thalassinos N (2007) The application of the combined corticotropin-releasing hormone plus desmopressin stimulation during petrosal sinus sampling is both sensitive and specific in differentiating patients with Cushing's disease from patients with the occult ectopic adrenocorticotropin syndrome. J Clin Endocrinol Metab 92(6):2080–2086

    Article  PubMed  CAS  Google Scholar 

  16. Lienhardt A, Grossman AB, Dacie JE, Evanson J, Huebner A, Afshar F, Plowman PN, Besser GM, Savage MO (2001) Relative contributions of inferior petrosal sinus sampling and pituitary imaging in the investigation of children and adolescents with ACTH-dependent Cushing's syndrome. J Clin Endocrinol Metab 86(12):5711–5714

    PubMed  CAS  Google Scholar 

  17. Joshi SM, Hewitt RJ, Storr HL, Rezajooi K, Ellamushi H, Grossman AB, Savage MO, Afshar F (2005) Cushing's disease in children and adolescents: 20 years of experience in a single neurosurgical center. Neurosurgery 57(2):281–285

    Article  PubMed  Google Scholar 

  18. Dias RP, Kumaran A, Chan LF, Martin L, Afshar F, Matson M, Plowman PN, Monson JP, Besser GM, Grossman AB, Savage MO, Storr HL (2010) Diagnosis, management and therapeutic outcome in prepubertal Cushing's disease. Eur J Endocrinol 162(3):603–609

    Article  PubMed  CAS  Google Scholar 

  19. Storr HL, Alexandraki KI, Martin L, Isidori AM, Kaltsas GA, Monson JP, Besser GM, Matson M, Evanson J, Afshar F, Sabin I, Savage MO, Grossman AB (2011) Comparisons in the epidemiology, diagnostic features and cure rate by transsphenoidal surgery between paediatric and adult-onset Cushing's disease. Eur J Endocrinol 164(5):667–674

    Article  PubMed  CAS  Google Scholar 

  20. Storr HL, Drake WM, Evanson J, Matson M, Berney DM, Grossman AB, Akker SA, Monson JP, Alusi G, Savage MO, Sabin I (2014) Endonasal endoscopic transsphenoidal pituitary surgery: early experience and outcome in paediatric Cushing's disease. Clin Endocrinol (Oxf) 80(2):270–276

    Article  Google Scholar 

  21. Güemes M, Murray PG, Brain CE, Spoudeas HA, Peters CJ, Hindmarsh PC, Dattani MT (2016) Management of Cushing syndrome in children and adolescents: experience of a single tertiary centre. Eur J Pediatr 175(7):967–976

    Article  PubMed  Google Scholar 

  22. Yordanova G, Martin L, Afshar F, Sabin I, Alusi G, Plowman NP, Riddoch F, Evanson J, Matson M, Grossman AB, Akker SA, Monson JP, Drake WM, Savage MO, Storr HL (2016) Long-term outcomes of children treated for Cushing's disease: a single center experience. Pituitary 19(6):612–624

    Article  PubMed  PubMed Central  Google Scholar 

  23. Crock PA, Lüdecke DK, Knappe UJ, Saeger W (2018) A personal series of 100 children operated for Cushing's disease (CD): optimizing minimally invasive diagnosis and transnasal surgery to achieve nearly 100% remission including reoperations. J Pediatr Endocrinol Metab 31(9):1023–1031

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Neuroendocrine Unit, Division of Endocrinology and Metabolism, University of Sao Paulo Medical School, Sao Paulo, SP, Brazil

    Lara Bessa Campelo Pinheiro Cavalcante, Thais Castanheira Freitas, Marcello Delano Bronstein & Marcio Carlos Machado

  2. Neurosurgery Service, University of Sao Paulo Medical School, Sao Paulo, SP, Brazil

    Nina Rosa Castro Musolino, Valter Angelo Sperling Cescato & Gilberto Ochman Silva

  3. Adrenal Unit, Division of Endocrinology and Metabolism, University of Sao Paulo Medical School, Sao Paulo, SP, Brazil

    Maria Candida Barisson Villares Fragoso

  4. Neuroradiology Service, University of Sao Paulo Medical School, Sao Paulo, SP, Brazil

    Paulo Puglia Jr.

  5. Laboratory of Cellular and Molecular Endocrinology/LIM25, University of Sao Paulo Medical School, Sao Paulo, SP, Brazil

    Marcello Delano Bronstein & Marcio Carlos Machado

  6. Endocrinology Service, AC Camargo Cancer Center, Sao Paulo, SP, Brazil

    Marcio Carlos Machado

Authors
  1. Lara Bessa Campelo Pinheiro Cavalcante
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  2. Thais Castanheira Freitas
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  6. Maria Candida Barisson Villares Fragoso
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Correspondence to Marcio Carlos Machado.

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Cavalcante, L.B.C.P., Freitas, T.C., Musolino, N.R.C. et al. High accuracy of bilateral and simultaneous petrosal sinus sampling with desmopressin for the differential diagnosis of pediatric ACTH-dependent Cushing’s syndrome. Pituitary 23, 507–514 (2020). https://doi.org/10.1007/s11102-020-01051-1

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  • DOI: https://doi.org/10.1007/s11102-020-01051-1

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