Adrenal Cortex Carcinoma

The age range distribution is diverse and estimation of malignant potential using Lin-Weiss Bisceglia scoring criteria of OACC is of paramount importance. Beta-catenin can be applied as a useful marker for prognostication of OACCs. Insulin growth factor-2 overexpression of conventional adrenocortical carcinomas must not be extrapolated to the oncocytic variant.

P2, N=137, Active, not recruiting, Canadian Cancer Trials Group | Trial completion date: Dec 2025 --> Jun 2026

Cortisol secretion was not suppressed following a 1-mg dexamethasone suppression test, and computed tomography revealed a left adrenal tumor...A histopathological examination confirmed adrenocortical adenoma. This case demonstrates that mild autonomous cortisol secretion can impair GH secretion and cause central hypothyroidism even in the absence of any overt cushingoid features.

We observed BHDS, confirmed by a pathogenic FLCN variant in 0.7% of all ACC patients, or 1.5% of patients evaluated by a genetic counselor and 2.0% of all patients who underwent genetic testing. Testing for FLCN pathogenic variants should be considered during routine genetic evaluation of patients with ACC and special attention should be paid to adrenal tumors in BHDS patients during surveillance for renal neoplasms as more than 50% of patients in this series developed metastatic disease.

P=N/A, N=32, Not yet recruiting, University of Arizona

In conclusion, comparative Whole Exome Sequencing (WES) analysis of three adrenal tumors in a MEN1 patient suggests a possible relationship between malignant and benign lesions occurring in MEN1 patients, without, however, demonstrating any causal adenoma-to-carcinoma progression driven by MEN1 LOH. Overall, these data further suggest an increased risk of MEN1 patients to develop adrenocortical malignancy.

Although rare, adrenocortical carcinoma (ACC) should be considered in individuals with neurofibromatosis type 1 (NF1) presenting with adrenal incidentalomas. Despite pheochromocytoma being more prevalent in NF1, appropriate functional evaluation is important in this context to rule out adrenocortical carcinoma given its aggressive nature and poor prognosis.

Adult-type epithelial neoplasms in children and adolescents are distinct entities, characterized by unique biological behaviors and genetic signatures. Treatment approaches should integrate principles from adult oncology and collaborative studies are essential to define the epidemiology, for staging and prognostic markers, and also to develop pediatric-specific treatment protocols for these malignancies.

P1, N=70, Active, not recruiting, Eisai Co., Ltd. | Trial completion date: Jan 2026 --> Mar 2027 | Trial primary completion date: Jan 2026 --> Mar 2027

identifying agents capable of complementing or replacing standard therapy remains a central challenge in ACC research. Our findings suggest that celastrol is a potent bioactive compound with activity against both monolayer and 3D ACC models, offering potential translational relevance. By elucidating ER stress as a shared mechanism between celastrol and mitotane, our work supports further exploration of this pathway as a strategy to potentially improve therapeutic outcomes in ACC patients.

The patient was started on mitotane and metyrapone, followed by 8 cycles of etoposide-doxorubicin-cisplatin plus mitotane. The patient underwent complete surgical resection, achieving full remission. More than 1 year later, she remains disease free.

P2, N=48, Recruiting, National Cancer Institute (NCI) | Initiation date: Jul 2026 --> Feb 2026