ESPE2024 Poster Category 2 Adrenals and HPA Axis (25 abstracts)
Severe Adrenocortical Carcinoma Presentation with Vena Cava Involvement in a 1-Year-Old Boy: A Case Presentation and Management Considerations
Dimitrije Cvetković 1 , Rade Vuković 1,2 , Tatjana Milenković 1 , Slađana Todorović 1 , Katarina Mitrović 1,2 , Sanja Panić Zarić 1 , Maja Čehić 1 , Dragomir Đokić 1 , Danka Redžić 1 , Dragana Aleksić 1 , Ratko Radeta 1 , Mehul Dattani 3 , Imran Mushtaq 3 , Mette Jorgensen 3 , Nagarajan Muthialu 3 , Tom Watson 3 & Trung Nguyen 3
1Mother and Child Healthcare Institute of Serbia “Dr Vukan Čupić”, Belgrade, Serbia. 2Faculty of Medicine, University of Belgrade, Belgrade, Serbia. 3Great Ormond Street Hospital for Children, London, United Kingdom
Introduction: Adrenocortical carcinoma (ACC) is a rare malignancy in children, presenting diagnostic and therapeutic challenges. This case report details the presentation, workup, and initial management of a highly aggressive ACC in a young infant.
Case Presentation: A 12-month-old boy presented with premature pubarche. Parents also noticed rapid weight gain, increased body hair growth, and penile enlargement. Clinical picture for Cushing syndrome and hyperandrogenism prompted a comprehensive evaluation, confirming excess of cortisol and androgen secretion. Cortisol levels remained unsuppressed with dexamethasone administration and imaging studies identified a right adrenal mass with infiltration of the inferior vena cava (IVC) up to right atrium of the heart and a suspected pulmonary metastasis.
Management: Due to the extensive local invasion, surgical intervention could only be performed in the center of excellence for pediatric ACC. The management plan involved initiating chemotherapy and expedited referral to specialized pediatric oncology center in Great Ormon Street Hospital for Children in London (GOSH) for surgical intervention and further oncological and endocrinological treatment. Pre-operative management included neoadjuvant chemotherapy with a SIOPE-ACC protocol followed by metyrapone initiation to control hypercortisolemia.
Surgical Intervention and Pathology: The patient underwent surgical resection of the adrenal mass, IVC thrombectomy, and IVC reconstruction under cardiopulmonary bypass in a London Children’s Hospital. Pathological analysis confirmed a low-grade adrenocortical carcinoma (NOS) with vascular and lymphatic invasion. Postoperatively, additional cycles of chemotherapy incorporating mitotane were continued, and the patient was tolerating the treatment well.
Post-Operative Course: He experienced a transient COVID-19 from which he recovered nicely. Parents were educated for hydrocortisone stress dosing.
Conclusion: This case highlights the aggressive nature of ACC in a young child and the complexities of managing such cases. Collaboration between pediatric endocrinologists, radiologists, oncologists and surgeons within multidisciplinary teams is crucial for timely diagnosis, optimizing treatment strategies, and potentially achieving successful outcomes. The report concludes with the patient's return to the home institution for continuation of treatment.
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