SFEBES2022 Poster Presentations Adrenal and Cardiovascular (66 abstracts)
Coincidence of Primary Adrenocortical Carcinoma and Melanoma: Three Case Reports
Ye Lynn Ko 1 , Vaishnavi Kumar 1 , Juliane Lippert 2 , Salvador Diaz-Cano 3 , Kassiani Skordilis 3 , Otilia Kimpel 2 , Stefan Kircher 4 , Miriam Asia 1 , Yasir S. Elhassan 1 , Barbara Altieri 2 & Cristina L. Ronchi 1,2,5,6
1Department of Endocrinology, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom; 2Division of Endocrinology and Diabetes, Department of Internal Medicine I, University Hospital; University of Würzburg, Würzburg, Germany; 3Department of Histopathology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom; 4Institute for Pathology, University of Wuerzburg, Würzburg, Germany; 5Institute of Metabolism and System Research, University of Birmingham, Birmingham, United Kingdom; 6Centre for Endocrinology, Diabetes, and Metabolism (CEDAM), Birmingham Health Partners, Birmingham, United Kingdom
Adrenocortical carcinoma (ACC) is a rare endocrine malignancy. ACC may rarely occur as part of familial cancer syndromes, but the majority of the cases occur sporadically. A significant proportion of sporadic ACC cases may be preceded by other malignancies and adrenal metastasis from these primary tumours may frequently occur. Herein we present three cases where sporadic ACC was identified in patients with coexistent or previous history of melanoma.
Case description: Patient 1 - A progressively enlarging left adrenal mass was found in a 37 year old man with a superficial spreading BRAF-positive melanoma. Initially, adrenal metastasis was suspected, but histology after adrenalectomy diagnosed ACC. Patient 2 – A rapidly enlarging left adrenal mass was found in a 68-year-old man with a history of recurrent BRAF-positive disseminated melanoma. Consequently, he underwent left adrenalectomy, and histology showed a diagnosis of ACC. Patient 3 – A 50-year-old man with a background of pT1 melanoma was referred with histological diagnosis of metastatic ACC. Germline TP53 variants (Li-Fraumeni syndrome) were excluded in all cases. Targeted DNA sequencing of ACC tissue samples was performed in all cases. Somatic variants were observed in the known driver genes CTNNB1 (Patient 1), APC and KMT2D (Patient 2), and APC and TP53 (Patient 3). The retrospective review of the patient cohort referred for adrenal masses during the last 21 years revealed that 1.6% of patients with histologically confirmed ACC had a previous history of melanoma. Only 0.5% of our patients had histologically proven adrenal metastasis.
Conclusion: sporadic ACC can occur in the background of melanoma, even if adrenal metastasis might appear to be the most likely diagnosis. Coexistent primary adrenal malignancy should be considered in all patients with a history of melanoma with suspicious adrenal lesions.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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