scholarly journals Medical Approaches in Adrenocortical Carcinoma

Biomedicines ◽  
2020 ◽  
Vol 8 (12) ◽  
pp. 551
Author(s):  
Rosa Maria Paragliola ◽  
Andrea Corsello ◽  
Pietro Locantore ◽  
Giampaolo Papi ◽  
Alfredo Pontecorvi ◽  
...  

Adrenocortical carcinoma (ACC) represents one of the most aggressive endocrine tumors. In spite of a correct therapeutic strategy based on a multidisciplinary approach between endocrinologist, surgeon and oncologist, the prognosis is often poor. Surgery is the mainstay treatment in ACC. Mitotane, a dichloro-diphenyl-trichloro-ethane derivate, represents the main medical treatment of ACC in consideration of its adrenocytolitic activity and it is mainly employed as adjuvant treatment after complete surgical resection and for the treatment of advanced ACC. However, the use of mitotane as adjuvant therapy is still controversial, also in consideration of the retrospective nature of several studies. The recurrence of disease is frequent, especially in advanced disease at the diagnosis. Therefore, in these contexts, conventional chemotherapy must be considered in association with mitotane, being the combination etoposide, doxorubicin and cisplatin (EDP) the standard of care in this setting. A more modern therapeutic approach, based on the need of a salvage therapy for advanced ACC that progresses through first-line EDP, is focused on molecular-targeted therapies. However, robust clinical trials are necessary to assess the real efficacy of these treatments.

Author(s):  
Steve Ball ◽  
Sajid Kalathil

Adrenocortical cancer (ACC) is rare and associated with poor prognosis. The incidence is estimated at 0.7–2 cases per one million. Overall survival rate at five years for ACC is 37–47%. While the pathogenesis of ACC is incompletely understood, inherited predisposition syndromes are common in childhood ACC. Clinical presentation can be with symptoms and signs of hormone excess (functional tumours), mass effects, or as an incidental radiological finding. A multidisciplinary approach combining radiology, biochemistry, and tissue-based pathology is needed to establish a diagnosis to guide a surgical approach aimed at complete resection of the tumour where possible. At present, recommended first-line therapies for advanced disease are mitotane monotherapy or etopiside, doxorubicin, and cisplatin plus mitotane. Metronomic capecitabine and gemcitabine have been used as alternatives. Adjuvant radiotherapy to the tumour bed should be considered for patients considered to be at high risk of recurrence.


2012 ◽  
Vol 30 (15_suppl) ◽  
pp. e18102-e18102 ◽  
Author(s):  
Thibault Vieira ◽  
Aurelie Cazes ◽  
Bonnette Pierre ◽  
Leila Zemoura ◽  
Isabelle Monnet ◽  
...  

e18102 Background: Sarcomatoid lung cancers (SC) are associated with poor prognosis. A few studies suggest that these tumors are resistant to platinum-based chemotherapy. Methods: Between January 1994 and October 2011, all consecutive patients with SC were screened from six French centers. Patients with advanced disease and treated with first-line chemotherapy were included in this study. Clinical data, staging (TNM 2009 classification), drugs and response to chemotherapy (RECIST 1.1) at first tumor assessment were reported. Factors associated with progressive disease (PD) in univariate (p<0.2) were included in multivariate analysis. Results: 111 patients with SC were screened. 51 had advanced stage and were treated by a first-line chemotherapy. Among them, 27 (53%) had initial advanced disease and 24 (47%) had relapse after radical surgery or radiotherapy. Patients were 60.7±3 years old, 69% were male, 86% smokers and 86% were symptomatic. PS was 0 (57%), 1 (39%) 2 (4%). Stages at the diagnosis were I (15.5%), II (13.5%), IIIA (20%), IIIB (6%), IV (45%). Histological subtype were pleomorphic (70%), pure sarcoma-like (20%) unspecified (6%), carcino-sarcoma (4%). 38 (75%) received a platinum based-combination and 13 (25%) a monotherapy. Drug given alone or combined with platin were gemcitabine (23%), navelbine (16%), placlitaxel (16%), docetaxel (12%), pemetrexed (9.8%), ifosfamide (9.8%), vepeside (4%) and others (11%). At first tumor assessment, PD and disease control rates were 71% and 29%, respectively. Median progression free survival was 2 months IC95% [1.3, 2.7]. Median overall survival was 6 months [3.2, 8.8]. In univariate analysis, age, gender, smoking status, numbers of metastatic locations, histological subtypes, monotherapy vs platin-based combinations, relapse after radical treatment vs initial advanced disease were not associated with PD. In multivariate analysis, initial advanced disease was the only independent factor of PD (OR 4.59 [1.17, 18] p=0.03). Conclusions: Our series confirm that advanced SC are refractory to conventional chemotherapy. Molecular characterizations are needed to improve therapeutic strategy.


2021 ◽  
Vol 12 ◽  
pp. 204062072199648
Author(s):  
Matteo Franchi ◽  
Claudia Vener ◽  
Donatella Garau ◽  
Ursula Kirchmayer ◽  
Mirko Di Martino ◽  
...  

Introduction: Randomized clinical trials showed that bortezomib, in addition to conventional chemotherapy, improves survival and disease progression in multiple myeloma (MM) patients not eligible for stem cell transplantation. The aim of this retrospective population-based cohort study is the evaluation of both clinical and economic profile of bortezomib-based versus conventional chemotherapy in daily clinical practice. Methods: Healthcare utilization databases of six Italian regions were used to identify adult patients with non-transplant MM, who started a first-line therapy with bortezomib-based or conventional chemotherapy. Patients were matched by propensity score and were followed from treatment start until death, lost to follow-up or study end-point. Overall survival (OS) and restricted mean survival time (RMST) were estimated using the Kaplan–Meier method. Association between first-line treatment and risk of death was estimated by a conditional Cox proportional regression model. Average mean cumulative costs were estimated and compared between groups. Results: In the period 2010–2016, 3509 non-transplant MM patients met the inclusion criteria, of which 1157 treated with bortezomib-based therapy were matched to 1826 treated with conventional chemotherapy. Median OS and RMST were 33.9 and 27.9 months, and 42.9 and 38.4 months, respectively, in the two treatment arms. Overall, these values corresponded to a HR of death of 0.79 (95% CI 0.71–0.89) over a time horizon of 84 months. Average cumulative cost were 83,839 € and 54,499 €, respectively, corresponding to an incremental cost-effectiveness ratio of 54,333 € per year of life gained, a cost coherent with the willingness-to-pay thresholds frequently adopted from Western countries. Conclusions: These data suggested that, in a large cohort of non-transplant MM patients treated outside the experimental setting, first-line treatment with bortezomib-based therapy was associated with a favourable effectiveness and cost-effectiveness profile.


2021 ◽  
Vol 13 ◽  
pp. 175883592110311
Author(s):  
Chiun Hsu ◽  
Lorenza Rimassa ◽  
Hui-Chuan Sun ◽  
Arndt Vogel ◽  
Ahmed O. Kaseb

In light of positive efficacy and safety findings from the IMbrave150 trial of atezolizumab plus bevacizumab, this novel combination has become the preferred first-line standard of care for patients with unresectable hepatocellular carcinoma (HCC). Several additional trials are ongoing that combine an immune checkpoint inhibitor with another agent such as a multiple kinase inhibitor or antiangiogenic agent. Therefore, the range of first-line treatment options for unresectable HCC is likely to increase, and healthcare providers need succinct information about the use of such combinations, including their efficacy and key aspects of their safety profiles. Here, we review efficacy and safety data on combination immunotherapies and offer guidance on monitoring and managing adverse events, especially those associated with atezolizumab plus bevacizumab. Because of their underlying liver disease and high likelihood of portal hypertension, patients with unresectable HCC are at particular risk of gastrointestinal bleeding, and this risk may be exacerbated by treatments that include antiangiogenic agents. Healthcare providers also need to be alert to the risks of proteinuria and hypertension, colitis, hepatitis, and reactivation of hepatitis B or C virus infection. They should also be aware of the possibility of rarer but potentially life-threatening adverse events such as pneumonitis and cardiovascular events. Awareness of the risks associated with these therapies and knowledge of adverse event monitoring and management will become increasingly important as the therapeutic range broadens in unresectable HCC.


Author(s):  
Kevin C. Miller ◽  
John P. Marinelli ◽  
Jeffrey R. Janus ◽  
Ashish V. Chintakuntlawar ◽  
Robert L. Foote ◽  
...  

AbstractEsthesioneuroblastoma (ENB) is a rare olfactory malignancy that can present with locally advanced disease. At our institution, patients with ENB in whom the treating surgeon believes that a margin-negative resection is initially not achievable are selected to undergo induction with chemotherapy with or without radiotherapy prior to surgery. In a retrospective review of 61 patient records, we identified six patients (10%) treated with this approach. Five of six patients (83%) went on to definitive surgery. Prior to surgery, three of five patients (60%) had a partial response after induction therapy, whereas two of five (40%) had stable disease. Microscopically margin-negative resection was achieved in four of five (80%) of the patients who went on to surgery, while one patient had negative margins on frozen section but microscopically positive margins on permanent section. Three of five patients (60%) recurred after surgery; two of these patients died with recurrent/metastatic ENB. In summary, induction therapy may facilitate margin-negative resection in locally advanced ENB. Given the apparent sensitivity of ENB to chemotherapy and radiotherapy, future prospective studies should investigate the optimal multidisciplinary approach to improve long-term survival in this rare disease.


Cancers ◽  
2021 ◽  
Vol 13 (5) ◽  
pp. 952
Author(s):  
Lucy Dumas ◽  
Rebecca Bowen ◽  
John Butler ◽  
Susana Banerjee

Older women with ovarian cancer have disproportionately poorer survival outcomes than their younger counterparts and receive less treatment. In order to understand where the gaps lie in the treatment of older patients, studies incorporating more detailed assessment of baseline characteristics and treatment delivery beyond the scope of most cancer registries are required. We aimed to assess the proportion of women over the age of 65 who are offered and receive standard of care for first-line ovarian cancer at two UK NHS Cancer Centres over a 5-year period (December 2009 to August 2015). Standard of care treatment was defined as a combination of cytoreductive surgery and if indicated platinum-based chemotherapy (combination or single-agent). Sixty-five percent of patients aged 65 and above received standard of care treatment. Increasing age was associated with lower rates of receiving standard of care (35% > 80 years old versus 78% of 65–69-year-olds, p = 0.000). Older women were less likely to complete the planned chemotherapy course (p = 0.034). The oldest women continue to receive lower rates of standard care compared to younger women. Once adjusted for Federation of Gynaecology and Obstetrics (FIGO) stage, Eastern Cooperative Oncology Group (ECOG) performance status and first-line treatment received, age was no longer an independent risk factor for poorer overall survival. Optimisation of vulnerable patients utilising a comprehensive geriatric assessment and directed interventions to facilitate the delivery of standard of care treatment could help narrow the survival discrepancy between the oldest patients and their younger counterparts.


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