Publications by authors named "Rossella Libe"
Article Synopsis
- This study aimed to improve the identification of malignancy risk and genetic status in patients with primary paraganglioma or pheochromocytoma (PPGL) by assessing various biomarkers.
- Conducted over four years with 231 patients, the study found that tumor analysis was better than germline testing for determining genetic status, with specific biomarkers like plasma succinate levels and miR-483-5p showing promise in predicting metastasis.
- The combination of biomarkers, such as SDHB immunostaining and TERT promoter methylation, significantly enhanced the predictive accuracy for both SDHx genetic status and metastatic potential.
Article Synopsis
- The study aims to identify easily measurable biomarkers that reflect the biological effects of glucocorticoids in patients with Cushing's syndrome through whole blood transcriptome analysis.
- It analyzed transcriptomic profiles from blood samples of different patient groups, creating a prediction model that effectively distinguishes between those with overt Cushing's syndrome and other conditions.
- Findings indicate that the transcriptome can indicate glucocorticoid levels, with FKBP5 expression showing potential as a nonhormonal marker for diagnosing Cushing's syndrome.
Adrenocortical carcinoma: Diagnosis, prognostic classification and treatment of localized and advanced disease.
Cancer Treat Res Commun
December 2023
Adrenocortical carcinoma (ACC) is a rare cancer with an estimated incidence of 0.7 to 2.0 cases per 1 million population per year in the United States.
View Article and Find Full Text PDFBackground: Adjuvant treatment with mitotane is commonly used after resection of adrenocortical carcinoma; however, treatment remains controversial, particularly if risk of recurrence is not high. We aimed to assess the efficacy and safety of adjuvant mitotane compared with surveillance alone following complete tumour resection in patients with adrenocortical carcinoma considered to be at low to intermediate risk of recurrence.
Methods: ADIUVO was a multicentre, open-label, parallel, randomised, phase 3 trial done in 23 centres across seven countries.
Article Synopsis
- Pheochromocytomas and paragangliomas are rare neuroendocrine tumors that arise in the adrenal glands and other locations, with malignancy marked by distant metastases.
- About 40% of these tumors are inherited and may be linked to various hereditary syndromes, with management provided by specialized centers in France.
- Diagnosis and treatment require a comprehensive approach considering clinical symptoms, hormonal levels, genetics, and imaging, with multidisciplinary interventions including surgery, radiotherapy, and targeted medical therapies like sunitinib and temozolomide.
Surgical resection of adrenocortical carcinoma (ACC) is the only curative treatment. Even in localized (I-II) stages, open adrenalectomy (OA) is the gold standard, though laparoscopic adrenalectomy (LA) can be proposed in selected patients. Despite the postoperative benefits of LA, its role in the surgical management of patients with ACC remains controversial regarding oncologic outcomes.
View Article and Find Full Text PDFBackground: At metastatic stage, treatment of adrenocortical carcinoma (ACC) relies in first line on mitotane therapy, combination of mitotane with locoregional therapies or cisplatin-based chemotherapy according to initial presentation. In second line, ESMO-EURACAN recommendations favour enrolment of patients in clinical trials investigating experimental therapies. However, the benefit of this approach remains unknown.
View Article and Find Full Text PDFThe adrenocortical carcinoma (ACC) is a primary malignant tumor developed from the adrenal cortex, defined by a Weiss score≥3. Its prognosis is poor and depends mainly on the stage of the disease at diagnosis. Care is organized in France by the multidisciplinary expert centers of the national ENDOCAN-COMETE "Adrenal Cancers" network, certified by the National Cancer Institute.
View Article and Find Full Text PDFPurpose Of The Report: Adrenocortical carcinoma (ACC) is an extremely rare endocrine malignancy, which cannot always be diagnosed during conventional radiology and hormonal investigations. 18 F-FDG PET could help predict malignancy, but more data are necessary to support future guidelines.
Methods: A cohort of 63 patients with histologically proven ACC (n = 55) or metastatic ACC with steroid oversecretion (n = 8) was assembled.
Objective: After temozolomide failure, no evidence-based treatment is available for pituitary carcinomas (PCs) and aggressive pituitary tumors (APTs). To date, only 12 cases treated with immune-checkpoint inhibitors (ICIs) have been published, showing encouraging efficacy. Predictive factors of response are lacking.
View Article and Find Full Text PDFArticle Synopsis
- The study focuses on primary bilateral macronodular adrenal hyperplasia (PBMAH), a condition linked to adrenal macronodules and excess cortisol levels, highlighting the role of ARMC5 gene mutations as a key factor.
- Out of 352 patients analyzed, 52 (14.8%) were found to have ARMC5 mutations, exhibiting significantly higher cortisol levels and larger adrenal glands compared to non-mutated patients.
- The research identifies specific criteria—bilateral adrenal involvement and autonomous cortisol secretion—that are highly predictive of ARMC5 mutations, suggesting that these criteria should guide genetic testing and help refine diagnostic standards for PBMAH.
Article Synopsis
- - The detection and characterization of adrenal lesions have improved significantly over the past 20 years, with imaging techniques like CT being central for diagnosis, especially for hormone-secreting lesions.
- - Non-functioning adrenal lesions pose a challenge for accurate diagnosis, often requiring a combination of imaging methods to determine which lesions necessitate surgery or biopsy.
- - New advancements such as dual-energy CT, MRI, and F-FDG PET/CT are enhancing the ability to differentiate between benign and malignant lesions, while emerging technologies like radiomics and artificial intelligence offer promising results in improving diagnostic accuracy.
Article Synopsis
- The study looked into the genetic causes of a condition called food-dependent Cushing syndrome (FDCS) in patients with a specific type of adrenal gland problem.
- Researchers analyzed samples from 36 patients to find out more about their genes.
- They discovered that a gene called KDM1A is important for understanding FDCS, and testing for both KDM1A and another gene called ARMC5 can help doctors diagnose and treat patients better.
Context: Patients with adrenocortical carcinoma (ACC) are frequently on mitotane therapy for a long time period. The drug exerts adrenolytic activity requiring glucocorticoid supplementation, which can be potentially detrimental for bone.
Objective: To explore whether mitotane with/without chemotherapy is associated with an increased proportion of morphometric vertebral fractures (VFs) in ACC patients.
We report the case of a 75-year-old woman with liver metastasis as a recurrence of a pheochromocytoma resected 10 years ago, with a rare germline mutation in transmembrane protein 127, falsely negative on 18F-FDOPA and 18F-FDG PET/CT scans but strongly positive on 123I-MIBG scintigraphy and on 68Ga-DOTATOC PET/CT. Functional imaging has a key role in diagnosis of pheochromocytoma and paraganglioma, especially 18F-FDOPA shows very high sensitivity and specificity. However, 18F-FDOPA might be falsely negative in some of these tumors, depending on specific mutations, and thus MIBG or 68Ga-DOTATOC imaging could be an alternative.
View Article and Find Full Text PDFA relevant issue on the treatment of adrenocortical carcinoma (ACC) concerns the optimal duration of adjuvant mitotane treatment. We tried to address this question, assessing whether a correlation exists between the duration of adjuvant mitotane treatment and recurrence-free survival (RFS) of patients with ACC. We conducted a multicenter retrospective analysis on 154 ACC patients treated for ≥12 months with adjuvant mitotane after radical surgery and who were free of disease at the mitotane stop.
View Article and Find Full Text PDFThe major prognosis factor of adrenocortical carcinoma (ACC) is the completeness of surgery. The aim of our study was to identify preoperative imaging features associated with direct liver involvement (DLI) by right-sided ACC. Two radiologists, blinded to the outcome, independently reviewed preoperative CT and MRI examinations for eight signs of DLI, in patients operated for right-sided ACC and retrospectively included from November 2007 to January 2020.
View Article and Find Full Text PDFAdrenocortical carcinoma is a rare malignant tumor of poor prognosis, frequently requiring additional treatments after initial surgery. Due to its adrenolytic action, mitotane has become the first-line medical treatment in patients with aggressive adrenocortical carcinoma. Over the last 2years, apart from the classical chemotherapy based on etoposide and platinum salts, several studies reported the use of drugs such as temozolomide, tyrosine kinase inhibitors or immunotherapy, with more or less convincing results.
View Article and Find Full Text PDFBackground: The prognosis of adrenocortical carcinoma (ACC) is heterogeneous. Genomic studies have identified ACC subgroups characterized by specific molecular alterations, including features measured at DNA level (somatic mutations, chromosome alterations, DNA methylation), which are closely associated with outcome. The aim of this study was to evaluate intratumor heterogeneity of prognostic molecular markers at the DNA level.
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