Palabras clave
gonadotrofina independiente
McCune-Albright
quistes ováricos
inhibidores de aromatasa
Cómo citar
Resumen
La pubertad precoz periférica (PPP), también denominada independiente de gonadotrofinas, se caracteriza por la aparición temprana de caracteres sexuales secundarios debido a la presencia de esteroides sexuales, sin la activación del eje hipotálamohipófiso-gonadal. Aunque es menos frecuente que la pubertad precoz central, su diagnóstico oportuno es esencial para evitar alteraciones en el crecimiento, maduración ósea y desarrollo psicosocial. El objetivo de esta revisión es proporcionar una actualización de la fisiopatología, clasificación, diagnóstico y tratamiento de la PPP, integrando la evidencia reciente y los avances terapéuticos; teniendo en cuenta que es una entidad con importante heterogeneidad clínica y etiológica y que constituye un desafío diagnóstico. La identificación precisa de la causa permite un tratamiento dirigido y la prevención de complicaciones.
Citas
Laufer D, Davrieux M, García L. Desarrollo puberal en la niña y adolescente. Arch Pediatr Urug, 2023 Jun;94(s1):e403. [Disponible en: http://www.scielo.edu.uy/pdf/adp/v94nnspe1/1688-1249-adp-94-nspe1-e403.pdf][Último acceso: 5/12/2025]
Soriano Guillén L, Argente J. Pubertad precoz periférica: fundamentos clínicos y diagnóstico-terapéuticos. An Pediatr (Barc). 2012 Apr;76(4):229.e1-10. doi: 10.1016/j.anpedi.2011.09.014. Epub 2011 Nov 25. PMID: 22119235.
Kang E, Cho JH, Choi JH, Yoo HW. Etiology and therapeutic outcomes of children with gonadotropin-independent precocious puberty. Ann Pediatr Endocrinol Metab. 2016 Sep;21(3):136-142. doi: 10.6065/apem.2016.21.3.136. Epub 2016 Sep 30. PMID: 27777905; PMCID: PMC5073159.
Eugster EA. Peripheral precocious puberty: causes and current management. Horm Res. 2009 Jan;71 Suppl 1:64-7. doi: 10.1159/000178041. Epub 2009 Jan 21. PMID: 19153509.
M Pombo Arias, J Argemí. Tratado de endocrinología pediátrica. Madrid: Díaz De Santos; 1997.
Albright F, Butler AM, Hampton AO, Smith P. Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction, with precocious puberty in females. N Engl J Med. 1937;216:727-46.[Disponible en:https://www.nejm.org/doi/full/10.1056/NEJM193704292161701] [Último acceso: 5/12/2025]
Spencer T, Pan KS, Collins MT, Boyce AM. The Clinical Spectrum of McCune-Albright Syndrome and Its Management. Horm Res Paediatr. 2019;92(6):347-356. doi: 10.1159/000504802. Epub 2019 Dec 19. PMID: 31865341; PMCID: PMC7302983.
Phornphutkul C, Okubo T, Wu K, Harel Z, Tracy TF Jr, Pinar H, Chen S, Gruppuso PA, Goodwin G. Aromatase p450 expression in a feminizing adrenal adenoma presenting as isosexual precocious puberty. J Clin Endocrinol Metab. 2001 Feb;86(2):649-52. doi: 10.1210/jcem.86.2.7201. PMID: 11158024.
Papanikolaou A, Michala L. Autonomous Ovarian Cysts in Prepubertal Girls. How Aggressive Should We Be? A Review of the Literature. J Pediatr Adolesc Gynecol. 2015 Oct;28(5):292-6. doi: 10.1016/j.jpag.2015.05.004. Epub 2015 May 16. PMID: 26228588.
Mieszczak J, Eugster EA. Treatment of precocious puberty in McCune-Albright syndrome. Pediatr Endocrinol Rev. 2007 Aug;4 Suppl 4(0 4):419-22. PMID: 17982389; PMCID: PMC4118734.
Bercaw-Pratt JL, Moorjani TP, Santos XM, Karaviti L, Dietrich JE. Diagnosis and Management of Precocious Puberty in Atypical Presentations of McCune-Albright Syndrome: A Case Series Review. Journal of Pediatric and Adolescent Gynecology [Internet]. 2011 Nov 3;25(1):e9–13. [Disponible en: https://www.sciencedirect.com/science/article/abs/pii/S1083318811003779][Último acceso: 5/12/2025]
Dhivyalakshmi J, Bhattacharyya S, Reddy R, Arulselvi KI. Precocious pseudopuberty due to ovarian causes. Indian Pediatr. 2014 Oct;51(10):831-3. PMID: 25362018.
de Sousa G, Wunsch R, Andler W. Precocious pseudopuberty due to autonomous ovarian cysts: a report of ten cases and long-term follow-up. Hormones (Athens). 2008 Apr- Jun;7(2):170-4. doi: 10.1007/BF03401509. PMID: 18477555.
Koukourakis GV, Kouloulias VE, Koukourakis MJ, Zacharias GA, Papadimitriou C, Mystakidou K, et al. Granulosa cell tumor of the ovary: tumor review. Integr Cancer Ther. 2008 Sep;7(3):204-15. doi: 10.1177/1534735408322845. PMID: 18815151.
Tobia González SG, Urresti A, Varea S, Braschi JC. Gonadoblastoma in an anatomical normal man. Case report and bibliography review. RevArg de Urol.2000; 65(1):23. [Disponible en: https://www.revistasau.org/index.php/revista/article/viewFile/2981/2930][Último acceso: 5/12/2025]
Roth LM, Cheng L. Gonadoblastoma: origin and outcome. Hum Pathol. 2020 Jun;100:47-53. doi: 10.1016/j.humpath.2019.11.005. Epub 2019 Dec 2. PMID: 31805291.
Grand T, Hermann AL, Gérard M, Arama E, Ouerd L, Garrouche N, et al. Precocious puberty related to Leydig cell testicular tumor: the diagnostic imaging keys. Eur J Med Res. 2022 May 12;27(1):67. doi: 10.1186/s40001-022-00692-1. PMID: 35550623; PMCID: PMC9097322.
Haddad NG, Eugster EA. Peripheral precocious puberty including congenital adrenal hyperplasia: causes, consequences, management and outcomes. Best Pract Res Clin
Endocrinol Metab. 2019 Jun;33(3):101273. doi: 10.1016/j.beem.2019.04.007. Epub 2019 Apr 13. PMID: 31027974.
Ferrito L, Cobellis G, Giobbi D, Pannunzi CP, Iannilli A, Cherubini V. Peripheral Precocious Puberty due to Functioning Adrenocortical Tumor: Description of Two Cases. J Pediatr Adolesc Gynecol. 2017 Feb;30(1):e1-e4. doi: 10.1016/j.jpag.2016.09.006. Epub 2016 Sep 22. PMID: 27666809.
Young J, Bulun SE, Agarwal V, Couzinet B, Mendelson CR, Simpson ER, et al. Aromatase expression in a feminizing adrenocortical tumor. J Clin Endocrinol Metab. 1996 Sep;81(9):3173-6. doi: 10.1210/jcem.81.9.8784064. PMID: 8784064.
Shibata N, Nyuzuki H, Sasaki S, Ogawa Y, Okada M, Nagasaki K. Peripheral precocious puberty in a girl with an intracranial hCG-producing tumor: case report and literature review. Endocr J. 2021 Dec 28;68(12):1463-1467. doi: 10.1507/endocrj.EJ21-0117. Epub 2021 Jul 17. PMID: 34275973.
Nella AA, Kaplowitz PB, Ramnitz MS, Nandagopal R. Benign vaginal bleeding in 24 prepubertal patients: clinical, biochemical and imaging features. J Pediatr Endocrinol Metab. 2014 Sep;27(9-10):821-5. doi: 10.1515/jpem-2013-0415. PMID: 24756050.
Baranowski E, Högler W. An unusual presentation of acquired hypothyroidism: the Van Wyk-Grumbach syndrome. Eur J Endocrinol. 2012 Mar;166(3):537-42. doi: 10.1530/EJE-11-0494. Epub 2011 Dec 14. PMID: 22170796.
Cabrera SM, DiMeglio LA, Eugster EA. Incidence and characteristics of pseudoprecocious puberty because of severe primary hypothyroidism. J Pediatr. 2013 Mar;162(3):637-9. https://pubmed.ncbi.nlm.nih.gov/23196132/
Martinez-Pajares JD, Diaz-Morales O, Ramos-Diaz JC, Gomez- Fernandez E. Peripheral precocious puberty due to inadvertent exposure to testosterone: case report and review of the literature. J Pediatr Endocrinol Metab. 2012;25(9- 10):1007-12. doi: 10.1515/jpem-2012-0124. PMID: 23426834.
Pasquino AM, Balducci R, Manca Bitti ML, Spadoni GL, Boccherini B. Transient pseudo-precocious puberty by probable oestrogen intake in 3 girls. Arch Dis Child. 1982 Dec;57(12):954-6. doi: 10.1136/adc.57.12.954. PMID: 6758707; PMCID: PMC1628083.
Gurnurkar S, DiLillo E, Carakushansky M. A Case of Familial Male-limited Precocious Puberty with a Novel Mutation. J Clin Res Pediatr Endocrinol. 2021 Jun 2;13(2):239-244. doi: 10.4274/jcrpe.galenos.2020.2020.0067. Epub 2020 Aug 6. PMID: 32757547; PMCID: PMC8186329.
Qudsiya Z, Gupta V. Precocious Pseudopuberty [Internet]. PubMed. Treasure Island (FL): StatPearls Publishing; 2021. [Disponible en: https://www.ncbi.nlm.nih.gov/books/NBK560588/][Último acceso: 9/12/2025]
Pasternak Y, Friger M, Loewenthal N, Haim A, Hershkovitz E. The utility of basal serum LH in prediction of central precocious puberty in girls. Eur J Endocrinol. 2012 Feb;166(2):295- 9. doi: 10.1530/EJE-11-0720. Epub 2011 Nov 14. PMID: 22084156.
Lee DS, Ryoo NY, Lee SH, Kim S, Kim JH. Basal luteinizing hormone and follicular stimulating hormone: is it sufficient for the diagnosis of precocious puberty in girls? Ann Pediatr Endocrinol Metab. 2013 Dec;18(4):196-201. doi: 10.6065/apem.2013.18.4.196. Epub 2013 Dec 31. PMID: 24904877;
PMCID: PMC4027088.
Cantas-Orsdemir S, Eugster EA. Update on central precocious puberty: from etiologies to outcomes. Expert Rev Endocrinol Metab. 2019 Mar;14(2):123-130. doi: 10.1080/17446651.2019.1575726. Epub 2019 Feb 12. PMID: 30763521.
Talarico V, Rodio MB, Viscomi A, Galea E, Galati MC, Raiola G. The role of pelvic ultrasound for the diagnosis and management of central precocious puberty: An update. Acta Biomed. 2021 Nov 4;92(5):e2021480. doi: 10.23750/abm.v92i5.12295. PMID: 34738554; PMCID: PMC8689311.
Outwater EK, Siegelman ES, Hunt JL. Ovarian teratomas:tumor types and imaging characteristics. Radiographics. 2001;21(2):475-90. [Disponible en: https://pubs.rsna.org/doi/10.1148/radiographics.21.2.g01mr09475?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200pubmed][Último acceso: 9/12/2025]
Dunnick NR, Korobkin M, Francis I. Adrenal radiology: distinguishing benign from malignant adrenal masses. AJR Am J Roentgenol. 1996;167(4):861-7 [Disponible en: https://www.ajronline.org/doi/10.2214/ajr.167.4.8819372][Último acceso: 9/12/2025]
Estrada A, Boyce AM, Brillante BA, Guthrie LC, Gafni RI, Collins MT. Long-term outcomes of letrozole treatment for precocious puberty in girls with McCune-Albright syndrome. Eur J Endocrinol. 2016 Nov;175(5):477-483. doi: 10.1530/EJE-16-0526. Epub 2016 Aug 25. PMID: 27562402; PMCID:PMC5066167.
Neyman A, Eugster EA. Treatment of Girls and Boys with McCune-Albright Syndrome with Precocious Puberty - Update 2017. Pediatr Endocrinol Rev. 2017 Dec;15(2):136-141. doi: 10.17458/per.vol15.2017.nau.treatmentgirlsboys. PMID: 29292624; PMCID: PMC5808444.
Ng SM, Apperley LJ, Upradrasta S, Natarajan A. Vaginal Bleeding in Pre-pubertal Females. J Pediatr Adolesc Gynecol. 2020 Aug;33(4):339-342. doi: 10.1016/j.jpag.2020.01.152. Epub 2020 Feb 6. PMID: 32035994.
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