Adrenocortical tumor with precocious puberty in a 2‐month‐old girl

Marret, Jean-Baptiste; Raffoul, Lara; Ribault, Virginie; Ravasse, P; Rod, Julien

doi:10.1111/ped.12702

Cited by 6 publications

(13 citation statements)

References 15 publications

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“…Immunohistochemical research using p53 and Ki-67 antibodies 8 . Also, Tp53 mutation analysis makes accurate diagnosis possible 29 . Regarding radiographs, CT, which is the most helpful, is used to make the diagnosis in the first place 21 .…”

Section: Discussionmentioning

confidence: 99%

Adrenocortical carcinoma in a 10-month-old infant: A literature review and a rare case report

Al-Ghotani

Alabdallah

Shaaban

et al. 2023

Annals of Medicine &Amp; Surgery

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Introduction and importance: Adrenocortical carcinoma (ACC) in children is a rare condition. The annual incidence of ACC is extremely low, with only 0.2–0.3 cases per million children. The clinical presentations of ACC are numerous, such as terminal hair appearance, pubertal progress, hypercortisolism, enlarged clitoris, acne, systemic arterial hypertension, weight gain, and voice change. Case presentation: A 10-month-old female infant presented by her parents to the Department of Endocrinology with a mass on the right adrenal gland and Cushing’s syndrome symptoms. Surgery was performed. The death occurred after two times resuscitation due to sudden cardiac arrest. Clinical discussion: The adrenal gland consists of two distinct parts. Different types of tumors arise from each part of the adrenal gland. The most common tumor in adrenomedullary tumors was neuroblastoma which accounted for 60.4% of adrenal tumors. ACC in children is a rare condition. The etiology of ACTs is unclear. Conclusion: This case emphasizes that early diagnosis has a considerable role in preventing major complications. Also to advise considering ACC as a differential diagnosis when similar symptoms are found in an infant.

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Section: Discussionmentioning

confidence: 99%

Adrenocortical carcinoma in a 10-month-old infant: A literature review and a rare case report

Al-Ghotani

Alabdallah

Shaaban

et al. 2023

Annals of Medicine &Amp; Surgery

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“…Girls present a two-fold increase in risk of presenting an adrenal tumour, compared to boys. The most common presenting signs and symptoms result from virilization (17). Feminizing tumours are even more rare.…”

Section: Adrenal Tumoursmentioning

confidence: 99%

Pathophysiology of Peripheral Precocious Puberty in girls: an overview

Toutoudaki

Paltoglou

Paschalidou

et al. 2021

DAH

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Precocious puberty can be distinguished in central, or peripheral, based on the presence or absence of the Hypothalamic- Pituitary- Gonadal axis activation. The pathogenesis of peripheral precocious puberty (PPP) is based mainly on excessive estrogenic exposure, either endogenous or exogenous. The congenital causes of PPP include McCune- Albright syndrome (MAS) and Congenital Adrenal Hyperplasia (CAH). The main causes of acquired PPP are oestrogen producing tumours, which are mainly of ovarian or adrenal origin, hypothyroidism and environmental oestrogens or substances with estrogenic function.

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“…ACT are more common in women, with a proportion of 1.4:1. This rate varies according to age, but remains higher in women (2,4,5), especially in malignant adreno-cortical carcinomas; however, there are certain subtypes of ACC, such as oncocytic, with no preference for any sex (6).…”

Section: Introductionmentioning

confidence: 99%

“…Adrenocortical tumours (ACT) including adrenocortical carcinomas (ACC) and adrenocortical adenomas (ACA) are rare in the paediatric population, with an incidence of 0.3-0.38:1,000,000, accounting for approximately 0.2% of all paediatric neoplasms (1)(2)(3)(4).…”

Section: Introductionmentioning

confidence: 99%

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Case Report: Adrenocortical carcinoma in children—symptoms, diagnosis, and treatment

Zagojska,

Malka,

Gorecka

et al. 2023

Front. Endocrinol.

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Adrenocortical carcinomas are extremely rare in the paediatric population. Most of them are hormone-secretive lesions; therefore, they should be taken into consideration in a child with signs of precocious puberty and/or Cushing’s syndrome symptoms. Nonetheless, differentiation from benign adrenal tumours is necessary. We report a rare case of adrenocortical carcinoma in a girl and a literature review using the PubMed database. A four-year-old girl presented with rapidly progressing precocious puberty and signs of Cushing’s syndrome. Imaging of the abdomen revealed a large heterogeneous solid mass. Histopathologic evaluation confirmed adrenocortical carcinoma with high mitotic activity, atypical mitoses, pleomorphism, necrosis, and vascular invasion. After tumourectomy, a decrease of previously elevated hormonal blood parameters was observed. Genetic tests confirmed Li Fraumeni syndrome. Adrenocortical carcinoma should be suspected in children with premature pubarche and signs of Cushing’s syndrome. Diagnosis must be based on clinical presentation, hormonal tests, imaging, and histopathological evaluation. Complete surgical resection of the tumour is the gold standard. Oncological treatment in children is not yet well-studied and should be individually considered, especially in advanced, inoperable carcinomas with metastases. Genetic investigations are useful for determining the prognosis in patients and their siblings.

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Adrenocortical tumor with precocious puberty in a 2‐month‐old girl

Cited by 6 publications

References 15 publications

Adrenocortical carcinoma in a 10-month-old infant: A literature review and a rare case report

Adrenocortical carcinoma in a 10-month-old infant: A literature review and a rare case report

Pathophysiology of Peripheral Precocious Puberty in girls: an overview

Case Report: Adrenocortical carcinoma in children—symptoms, diagnosis, and treatment

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