Pseudo-precocious Puberty Associated with an Adrenocortical Tumor in a Young Child

Kafi, Shahd E; Alagha, Eid; Shazly, Mohamed Abdelmaksoud; Al-Agha, Abdulmoein Eid

doi:10.7759/cureus.6440

Cited by 3 publications

(3 citation statements)

References 10 publications

(14 reference statements)

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“…[ 7 ] Central PP usually presents with gonadal enlargement of ovaries in females or testicles in males, whereas pseudo-precocious puberty presents mainly with the early development of pubic and/or axillary hair. [ 8 ] The excess secretion of androgenic hormones resulting in virilization and pseudo-precocious puberty is the most common symptom of ACTs. But, a late presentation of ACT can be seen with CPP secondary to the maturation of the hypothalamic–pituitary–gonadal axis.…”

Section: Discussionmentioning

confidence: 99%

Precocious puberty in a child: A rare cause and review of literature

Saminathan

Dhivyalakshmi

Sneha

et al. 2022

Journal of Family Medicine and Primary Care

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Precocious puberty (PP) in pediatric office practice is challenging as the cause varies from benign to malignant conditions. Adrenocortical tumors are rare in childhood and pseudo-precocious puberty is the most common clinical presentation in children. We report a case of a 5-year-old boy who presented with features of abdominal distention and virilization, and his abdominal magnetic resonance imaging (MRI) revealed an adrenal tumor which was confirmed as adrenocortical carcinoma by biopsy. This case report highlights the importance of the awareness among general practioners and pediatricians to rule out adrenocortical tumors while evaluating a child with PP.

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Section: Discussionmentioning

confidence: 99%

Precocious puberty in a child: A rare cause and review of literature

Saminathan

Dhivyalakshmi

Sneha

et al. 2022

Journal of Family Medicine and Primary Care

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“…Finally, we retrieved 16 detailed case reports using citation searching. We summarized their clinical and histological findings, and when available, the results of genetic testing in Table 2 and compared them with our patient [ 5 – 19 ].…”

Section: Case Seriesmentioning

confidence: 99%

Peripheral precocious puberty in Li–Fraumeni syndrome: a case report and literature review of pure androgen-secreting adrenocortical tumors

Ryckx,

De Schepper,

Giron

et al. 2023

J Med Case Reports

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Introduction Pure androgen-secreting adrenocortical tumors are a rare but important cause of peripheral precocious puberty. Case presentation Here, we report a pure androgen-secreting adrenocortical tumor in a 2.5-year-old boy presenting with penile enlargement, pubic hair, frequent erections, and rapid linear growth. We confirmed the diagnosis through laboratory tests, medical imaging, and histology. Furthermore, genetic testing detected a pathogenic germline variant in the TP53 gene, molecularly confirming underlying Li–Fraumeni syndrome. Discussion Only 15 well-documented cases of pure androgen-secreting adrenocortical tumors have been reported so far. No clinical or imaging signs were identified to differentiate adenomas from carcinomas, and no other cases of Li–Fraumeni syndrome were diagnosed in the four patients that underwent genetic testing. However, diagnosing Li–Fraumeni syndrome is important as it implies a need for intensive tumor surveillance and avoidance of ionizing radiation. Conclusion In this article, we emphasize the need to screen for TP53 gene variants in children with androgen-producing adrenal adenomas and report an association with arterial hypertension.

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“…• Рекомендуется у мальчиков с ППР обязательное определение исследование уровня 17-гидроксипрогестерона (17ОНР) в крови для исключения врожденной дисфункции коры надпочечников (ВДКН), альфа-фетопротеина, бета-субъединицы хорионического гонадотропина человека (β-ХГЧ) для исключения герминогенных опухолей, исследование уровня дегидроэпиандростерона (ДГЭА) крови и/или дегидроэпиандростерона сульфата крови (ДГЭА-С) для исключения андроген-продуцирующих опухолей надпочечников [82][83][84][85][86][87]. УДД -4, УУР -С.…”

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Clinical guidelines «Precocious puberty»

Петеркова

Алимова

Bashnina

et al. 2021

Probl Endokrinol (Mosk)

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The precocious puberty is an urgent problem of pediatric endocrinology characterized by clinical and pathogenetic heterogeneity. The appearance of secondary sex characteristics before the age of 8 years in girls and 9 years in boys requires timely diagnosis and the appointment of pathogenetically justified treatment in order to achieve the target indicators of final growth and prevent social deprivation. The developed clinical guidelines are the main working tool of the practitioner. They briefly and structurally present the main information about the epidemiology and modern classification of рrecocious puberty, methods of its diagnosis and treatment based on the principles of evidence-based medicine.

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Pseudo-precocious Puberty Associated with an Adrenocortical Tumor in a Young Child

Cited by 3 publications

References 10 publications

Precocious puberty in a child: A rare cause and review of literature

Precocious puberty in a child: A rare cause and review of literature

Peripheral precocious puberty in Li–Fraumeni syndrome: a case report and literature review of pure androgen-secreting adrenocortical tumors

Clinical guidelines «Precocious puberty»

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