ECE2024 Poster Presentations Endocrine-Related Cancer (40 abstracts)
Advanced ACC stage: Therapeutic approach and biological findings
Alessandra Cossa 1 , Sara Pagotto 2 , Silvia Perconti 2 , Elisa Taschin 3 , Elena Sofia Scialis 2 , Simone De Fabritiis 2 , Maria Elena Aloini 4 , Diana Liberata Esposito 2 , Iolanda Matarazzo 5 , Fabio Verginelli 2 , Lavinia Vittoria Lotti 6 , Francesca Schiavi 3 , Renato Mariani Costantini 2 & Antonio Stigliano 4
1Surgery, Rome; 2Center for Advanced Studies and Technology, Chieti; 3Familial Cancer Clinical and Oncoendocrinology, Veneto Institute of Oncology, Padua; 4Endocrinology, Clinical and Molecular Medicine, Rome; 5Radiology, Surgical and Medical Sciences and Translational Medicine, Rome; 6Sapienza University of Rome, Experimental Medicine, Rome
Adrenocortical carcinoma (ACC) is a rare and aggressive cancer most frequent in middle-aged females. Management is challenging and debulking surgery has been advocated to improve survival and quality of life in advanced disease. We report a remarkable case in a 60-year-old female without individual or familial risk factors who, despite good performance status, presented with a 10.3×5.5×9.0 cm right adrenal mass, liver metastases, inferior vena cava thrombosis, hypercortisolism, and hyperandrogenism. The patient underwent debulking surgery with adrenalectomy, intracaval thrombectomy and liver metastasectomies. Pathology and immunohistochemistry confirmed high-grade ACC with hepatic metastases and neoplastic inferior vena cava thrombosis, mENS@T stage pIVa (pT4, pN2, pM1). Transmission electron microscopy revealed that both the primary and the metastatic tumor were a mosaic of two cell morphotypes with differential affinity for osmium tetroxide. The most abundant corresponded to large electron-lucent cells, the other (<30%) to thinner electron-dense cells with cytoplasmic filopodia, the latter characteristics reportedly associated to oxidative stress. Both morphotypes showed mitochondrial hypertrophy and hyperplasia, accompanied, particularly in dark cells, by mitochondrial swelling and cristae degradation, known to be linked to respiratory dysfunction. Genetically, the primary and the metastatic samples demonstrated microsatellite-stable phenotype. No alterations in 81 cancer-related genes, including TP53, MEN1 and PRKAR1A, three major ACC-related genes, were detected at germline level, while a pathogenic TP53 c.843C>A p.(Asp281Glu) transversion was detected with strikingly conserved 27% variant allele frequency in both primary and metastatic DNA. Despite adjuvant mitotane, CT scans at one month showed multiple hepatic and bilateral pulmonary metastases with pleural and mediastinal involvement, consistent with rapid deterioration of the hormonal and clinical picture. Death ensued 40 days after surgery. This case challenges the utility of debulking surgery in advanced ACC. Furthermore, its ultrastructural and genetic features suggest that mitochondrial dysfunction and adaptive cooperation between tumor cells differing in TP53 status and morphotype might contribute to biological aggressiveness.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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