Reply to “Pathological prognostication of pediatric adrenocortical tumors: Is a gold standard emerging?”

Picard, Cécile; Orbach, Daniel; Dijoud, Frédérique

doi:10.1002/pbc.27710

Cited by 5 publications

(4 citation statements)

References 9 publications

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“…Higher mitotic rate, higher percent of necrosis, and larger tumor size are usually associated with aggressive behavior ( 3 ). The Wieneke criteria, which include tumor size, local invasion, and histological features, have been reported useful in pediatric ACT malignancy definition and prognosis prediction ( 11 , 18 , 30 ). Recently, Picard and colleagues ( 31 ) proposed a pathological scoring system incorporating the Ki67 index ≥15% in a prognostication algorithm to guide adjuvant treatment in pediatric ACTs, mostly for those with incomplete resection.…”

Section: Discussionmentioning

confidence: 99%

Clinical, Genetic, and Prognostic Features of Adrenocortical Tumors in Children: A 10-Year Single-Center Experience

et al. 2020

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Background and Aims: Pediatric adrenocortical tumors (ACTs) are very rare endocrine neoplasms in childhood. In this study, we performed a retrospective analysis of children with ACT treated at our institution by examining clinical and genetic disease features, treatment strategies, and outcomes. Methods: We retrospectively analyzed a cohort of 13 children treated at the Bambino Gesù Children's Hospital from November 2010 to March 2020. Results: The median age at diagnosis was 17 months (range = 0-82 months). The female: male ratio was 3.3/1. Mixed symptomatology (>1 hormone abnormality) was the most common presentation (46.1%). In three cases, the tumor was detected during prenatal or perinatal echographic screening. All patients presented with localized disease at diagnosis and underwent total adrenalectomy. Six patients were identified as having malignancies according to the Wieneke scoring system, five benign, and two undetermined. Seven patients underwent mitotane adjuvant therapy for 12 months. There was metastatic disease in three patients, with no correlation with age or Wieneke score. The most common sites of metastases were the liver and lungs. Metastatic patients were treated with surgery (n = 2), mitotane (n = 1), chemotherapy (n = 2) associated with anti-EGFR (n = 1), or immunotherapy with anti-PD1 (pembrolizumab) (n = 1); two patients achieved complete disease remission. Overall 2-and 5-year survival rates were 100%, with a median follow-up of 5 years (range = 2-9.5 years). Two-and 5-year disease free survival was 76.9 and 84.6%, respectively (95% confidence interval = −66.78-114.76 months). All patients are alive, 12 without disease, and one with stable disease. Genetic analyses showed TP53 germline mutations in six of eight patients analyzed (five inherited, one de novo). One patient had Beckwith-Wiedemann syndrome, with mosaic paternal uniparental disomy of chromosome 11, in both neoplastic and healthy adrenal tissue. Miele et al. Adrenocortical Tumors in Children Conclusion: We report the cases of 13 patients treated for ACT, including 12 aged <4 years at diagnosis, with a relative short time from symptoms onset. Our cohort experienced an excellent prognosis. TP53 mutation was found in 75% of tested patients (6/8) confirming the need to perform genetic tests and familial counseling in this disease.

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Section: Discussionmentioning

confidence: 99%

Clinical, Genetic, and Prognostic Features of Adrenocortical Tumors in Children: A 10-Year Single-Center Experience

et al. 2020

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“…The factors that determine aggressive behavior include older age, a higher mitotic rate, a higher percentage of necrosis, and a large tumor size [ 12 ]. Histologically, the Wieneke criteria has been utilized for differentiating between the two, and has been reported to be useful [ 8 , 13 , 14 ]. In our study, those with a Wieneke score of 1–3 indicated benign or indeterminate disease, and clinically behaved in a benign manner, favoring the possibility of an adrenal adenoma.…”

Section: Discussionmentioning

confidence: 99%

Adrenocortical tumors in children: Sri Lankan experience from a single center, and a mini review

et al. 2023

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Background Pediatric adrenocortical tumors include both benign adenomas and highly virulent malignant tumors. However, they are very rare among children. The aim of this study is to evaluate the clinicopathological data of children presenting with adrenocortical tumors and assess their survival in a South Asian population. Case presentation This is a retrospective cohort study that includes patients diagnosed with adrenocortical tumors from August 2020 to August 2022 followed-up at Lady Ridgeway Hospital. Seven children were diagnosed with adrenal cortical tumors. Their ages ranged from 10 months to 6.5 years. Five of them were boys. All displayed signs of peripheral precocious puberty. One boy phenotypically had features of Beckwith–Wiedemann syndrome. The median time for diagnosis after the onset of symptoms was 4.4 months. The preoperative diagnosis was based on clinical manifestations, elevated dehydroepiandrosterone sulfate levels, and suprarenal masses on computed tomography. All five boys had right-sided suprarenal masses, while the two girls had them on the left side. All underwent surgery for tumor resection. The diagnosis was confirmed based on the histopathology of the adrenal specimens. Four children had a Wieneke score of 4 or more, suggesting the possibility of adrenocortical carcinoma; however, only two of them behaved as malignant tumors. To date, two children have developed local recurrences within a very short period. Conclusion Adrenocortical tumors are uncommon in children, and treatment options are limited. To identify early recurrences, routine clinical, radiological, and biochemical examinations at least once every 6–8 weeks is important.

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“…However, the histology of ACT in children is challenging, as the classical Weiss criteria used in adults often misclassify childhood ACT as carcinomas [ 24 ]. Therefore, a distinct pediatric grading system, the Wieneke criteria, should be combined with the Ki67 index and p53 status for childhood ACT [ 25 – 27 ]. Here, in the present case, both the Wieneke criteria (score: 0) and the Ki67 index (< 15%) indicated an adrenal adenoma.…”

Section: Discussionmentioning

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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Reply to “Pathological prognostication of pediatric adrenocortical tumors: Is a gold standard emerging?”

Cited by 5 publications

References 9 publications

Clinical, Genetic, and Prognostic Features of Adrenocortical Tumors in Children: A 10-Year Single-Center Experience

Clinical, Genetic, and Prognostic Features of Adrenocortical Tumors in Children: A 10-Year Single-Center Experience

Adrenocortical tumors in children: Sri Lankan experience from a single center, and a mini review

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

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