scholarly journals Resection of Adrenocortical Carcinoma with Bilobar Liver Metastasis Following Neoadjuvant Therapy Using a Modified Mini-ALPPS Approach: A Case Report

Author(s):  
Daniel Anaya ◽  
Bela Kis ◽  
Daniel Anaya ◽  
Jasmina Ehab ◽  
Julie E. Hallanger Johnson ◽  
...  

Introduction: Adrenocortical carcinoma (ACC) is an aggressive tumor with high proportion of patients presenting with metastatic disease, most commonly in the liver. Prognosis in this population is extremely poor. Resection of the primary tumor and liver metastasis offers a survival benefit in well-selected patients. However, the extent of surgery is often significant and can limit the ability to accomplish a safe marginnegative resection. Presentation of case: A 35-year-old male presented with a large left ACC (15.2cm) and multiple bilobar liver metastases (1.5-12.5cm). He was treated with mitotane and chemotherapy / immunotherapy, with excellent response. Multidisciplinary discussion led to recommendations for a curative-intent approach with surgery. A staged approach was performed for the resection, using a modified Mini-ALPPS technique. A complete margin-negative resection of all disease was accomplished. The patient recovered well and remains free of disease 24-months following diagnosis. Discussion: This case highlights novel components of treatment for metastatic ACC and for hepatectomy for bilobar liver metastasis. The decisions to proceed to surgery for complete resection and to use a staged approach with a modified Mini-ALPPS technique were both critical components to render the patient disease-free. Appropriate expertise and multidisciplinary teamwork are essential for implementation of these approaches. Conclusion: Neoadjuvant chemotherapy for stage IV ACC can result in disease control and improved selection of candidates for curative-intent surgery. In the setting of bilobar liver disease and a large primary in place, a modified Mini-ALPPS approach provides a safe and feasible way to accomplish complete resection and improved survival.

2020 ◽  
Author(s):  
Zhilong Wang ◽  
Xin Sun ◽  
Fengli Zhang ◽  
Ting Wang ◽  
Ping Li

Abstract Background: Adrenocortical carcinoma(ACC) is a rare and highly invasive endocrine malignant tumor with poor prognosis and insensitivity tochemotherapy, which don't have effective treatment. Although Surgical resection is considered to be the main treatment for ACC, postoperative recurrence and metastasis have become the most important factors of death. Therefore, local treatments such as trans-catheter arterial chemo-embolization, radiofrequency ablation become be new treatment for ACC. Trans-catheter arterial chemo-embolization therapy for ACC patients with liver metastasis has good efficacy and can effectively reduce the tumor burden of patients, which is considered to be safe and easy for patients. Case presentation: We report a 47-year-old female patient diagnosed with stage Ⅳ ACC with liver metastases, who developed symptoms of acute adrenocortical dysfunctions after hepatic arterial catheter chemo-embolization.Conclusion: Trans-catheter arterial chemo-embolization therapy for ACC patients with liver metastasis is not completely safe, and there is a certain probability that it will lead to secondary adrenal dysfunction. Hydrocortisone supplementation can effectively alleviate the symptoms.


2017 ◽  
Vol 83 (7) ◽  
pp. 761-768 ◽  
Author(s):  
Caroline E. Poorman ◽  
Lauren M. Postlewait ◽  
Cecilia G. Ethun ◽  
Thuy B. Tran ◽  
Jason D. Prescott ◽  
...  

Perioperative blood transfusion is associated with decreased survival in pancreatic, gastric, and liver cancer. The effect of transfusion in adrenocortical carcinoma (ACC) has not been studied. Patients with available transfusion data undergoing curative-intent resection of ACC from 1993 to 2014 at 13 institutions comprising the United States Adrenocortical Carcinoma Group were included. Factors associated with blood transfusion were determined. Primary and secondary end points were recurrence-free survival (RFS) and overall survival (OS), respectively. Out of 265 patients, 149 were included for analysis. Out of these, 57 patients (38.3%) received perioperative transfusions. Compared to nontransfused patients, transfused patients more commonly had stage 4 disease (46% vs 24%, P = 0.01), larger tumors (15.8 vs 10.2 cm, P < 0.001), inferior vena cava involvement (24.6% vs 5.4%, P = 0.002), additional organ resection (78.9% vs 36.3%, P < 0.001), and major complications (29% vs 2%, P < 0.001). Transfusion was associated with decreased RFS (8.9 vs 24.7 months, P = 0.006) and OS (22.8 vs 91.0 months, P < 0.001). On univariate Cox regression, transfusion, stage IV, hormonal hypersecretion, and adjuvant therapy were associated with decreased RFS. On multivariable analysis, only transfusion [hazard ratio (HR) = 1.7, 95% confidence interval (CI) 51.0–2.9, P = 0.04], stage IV (HR = 3.2, 95% CI = 1.7–5.9, P < 0.001), and hormonal hypersecretion (HR = 2.6, 95% CI = 1.5–4.2, P < 0.001) were associated with worse RFS. When applying this model to OS, similar associations were seen (transfusion HR = 2.0, 95% CI = 1.1–3.8, P = 0.02; stage 4 HR = 6.2, 95% CI = 3.1–12.4, P < 0.001; hormonal hypersecretion HR = 3.5, 95% CI = 1.9–6.4, P < 0.001). There was no difference in outcomes between patients who received 1 to 2 units versus >2 units of packed red blood cells in median RFS (8.9 vs 8.4 months, P = 0.95) or OS (26.5 vs 18.6 months, P = 0.63). Peri-operative transfusion is associated with earlier recurrence and decreased survival after curative-intent resection of ACC. Strategies and protocols to minimize blood transfusion should be developed and followed.


Author(s):  
Alice Boileve ◽  
Elise Mathy ◽  
Charles Roux ◽  
Matthieu Faron ◽  
Julien Hadoux ◽  
...  

Abstract Purpose European and French guidelines for ENSAT stage IV low tumor burden or indolent adrenocortical carcinoma (ACC) recommend combination of mitotane and locoregional treatments (LRT) in first-line. Nevertheless, the benefit of LRT combination with mitotane has never been evaluated in this selected group of patients. Methods A retrospective chart review was performed from 2003-2018 of patients with stage IV ACC with ≤2 tumoral organs who received mitotane in our center. Primary endpoint was the delay between mitotane initiation and first systemic chemotherapy. Secondary endpoints were progression-free survival (PFS) and overall survival (OS) from mitotane initiation. Adjusted analyses were performed on the main prognostic factors. Results Out of 79 included patients, 48 (61%) patients were female and median age at stage IVA diagnosis was 49.8 years (interquartile-range:38.8-60.0). Metastatic sites were mainly lungs (76%) and liver (48%). Fifty-eight (73%) patients received LRT including adrenal bed radiotherapy (14 patients, 18%), surgery (37 patients, 47%) and/or interventional radiology n(35,44%). Median time between mitotane initiation and first chemotherapy administration was 9 months (Interquartile-range:4-18). Median PFS1 (first tumor-progression) was 6.0 months (CI95%:4.5-8.6). Median OS was 46 months (CI95%:41-68). PFS1, PFS2 and OS were statistically longer in the mitotane plus LRT group compared to the mitotane-only group (Hazard ratio (HR)=0.39 (CI95%:0.22-0.68), HR=0.35 (CI95%:0.20-0.63) and HR=0.27 (CI95%:0.14-0.50) respectively). Ten (13%) patients achieved complete response, all from mitotane plus LRT group. Conclusion Our results endorse European and French guidelines for stage IV ACC with ≤2 tumor-organs and favor the combination of mitotane and LRT as first-line treatment. For the first time, a significant number of complete responses were observed. Prospective studies are expected to confirm these findings.


2018 ◽  
Vol 103 (9) ◽  
pp. 3566-3573 ◽  
Author(s):  
Sri Harsha Tella ◽  
Anuhya Kommalapati ◽  
Subhashini Yaturu ◽  
Electron Kebebew

Abstract Context Adrenocortical carcinoma (ACC) is rare; knowledge about prognostic factors and survival outcomes is limited. Objective To describe predictors of survival and overall survival (OS) outcomes. Design and Patients Retrospective analysis of data from the National Cancer Database (NCDB) from 2004 to 2015 on 3185 patients with pathologically confirmed ACC. Main Outcome Measures Baseline description, survival outcomes, and predictors of survival were evaluated in patients with ACC. Results Median age at ACC diagnosis was 55 (range: 18 to 90) years; did not differ significantly by sex or stage of the disease at diagnosis. On multivariate analysis, increasing age, higher Charlson-Deyo comorbidity index score, high tumor grade, and no surgical therapy (all P &lt; 0.0001); and stage IV disease (P = 0.002) and lymphadenectomy during surgery (P = 0.02) were associated with poor prognosis. Patients with stage I-III disease treated with surgical resection had significantly better median OS (63 vs 8 months; P &lt; 0.001). In stage IV disease, better median OS occurred in patients treated with surgery (19 vs 6 months; P &lt; 0.001), and postsurgical radiation (29 vs 10 months; P &lt; 0.001) or chemotherapy (22 vs 13 months; P = 0.004). Conclusion OS varied with increasing age, higher comorbidity index, grade, and stage of ACC at presentation. There was improved survival with surgical resection of primary tumor, irrespective of disease stage; postsurgical chemotherapy or radiation was of benefit only in stage IV disease.


2014 ◽  
Vol 146 (5) ◽  
pp. S-1043
Author(s):  
Afif N. Kulaylat ◽  
Jane R. Schubart ◽  
Audrey L. Stokes ◽  
Eric Kimchi ◽  
Kevin F. Staveley-O ◽  
...  

2021 ◽  
Author(s):  
Ningning Yang ◽  
Qingqing Wang ◽  
Fengxia Chen ◽  
Haibin Ou ◽  
Yangyang Zeng ◽  
...  

Abstract Background: Metastatic adrenocortical carcinomas (MAC) have a poor prognosis. Advanced adrenocortical carcinoma often metastasizes to lung and liver. Prognostic factors of MAC have been rarely reported. This study aims to identify the association between specific metastasis and overall survival (OS) in MAC and determine the survival predictors for MAC patients.Methods: MAC patients' data was obtained from Surveillance, Epidemiology, and End Results (SEER) database between 2010 and 2016. Survival differences were analyzed by Kaplan–Meier analysis and log-rank tests. Cox proportional hazard model was used to identify the prognostic factors associated with overall survival.Results: A total of 152 MAC patients were selected, among whom 77 patients (50.7%) were diagnosed with one metastatic site, 75 (49.3%) diagnosed with more than one distant metastasis. For the whole MAC cohort, multivariable analysis showed that year of diagnosis between 2013 and 2016, without liver metastasis, surgery and chemotherapy were significantly favorable predictors of OS. For patients with one metastatic site, lung metastases had a better survival outcome than liver metastases(p=0.037). Besides, compared with patients who didn’t received surgery, patients underwent surgery were correlated with longer OS (p=0.004). For patients with more than one site of distant metastases, married status, surgery, and chemotherapy predicted a better OS. Radiotherapy did not improve overall survival outcomes in the three cohort.Conclusion: Liver metastasis has a poor prognosis. Year of diagnosis, metastatic sites, surgery and chemotherapy were significant prognostic factors for OS in MAC patients. For patients with single metastasis, surgery was a favorable prognostic factor, while married patients, surgery and chemotherapy predicted a better survival outcome in patients with more than one metastasis. Based on the collective findings, surgery can be regarded as the preferred treatment option for all MAC patients. Besides, chemotherapy is also a good choice for patients with multiple metastases.


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 9500-9500
Author(s):  
Alexander M. Eggermont ◽  
Andrey Meshcheryakov ◽  
Victoria Atkinson ◽  
Christian U. Blank ◽  
Mario Mandalà ◽  
...  

9500 Background: The phase 3 double-blind EORTC 1325/KEYNOTE-054 trial evaluated pembrolizumab (pembro) vs placebo in stage III cutaneous melanoma patients (pts) with complete resection of lymph nodes. Pembro improved RFS (hazard ratio [HR] 0.57) and DMFS (HR 0.60) (Eggermont, NEJM 2018, TLO 2021). In the pembro group, the incidence of immune related AE (irAE) grade 1-5 was 37%, and of grade 3-5 was 7%. We present the safety profile, response rate and PFS for the subset of pts who had a recurrence and crossed over or were rechallenged with pembrolizumab, within protocol. Methods: Pts were randomized to receive iv. pembro 200 mg (N=514) or placebo (N=505) every 3 weeks for a total of 18 doses (~1 year). Upon recurrence with no brain metastases, pts with an ECOG PS 0-2 were eligible to enter part 2 of the study, i.e. to receive pembro 200 mg iv. every 3 weeks for a maximum of 2 years, for crossover (those who received placebo) or rechallenge (those who recurred ≥6 months after completing one year of pembro therapy). Treatment was stopped in case of disease progression (RECIST 1.1) or unacceptable toxicity. Results: At the clinical cut-off (16-Oct-2020), 298 (59%) pts had a disease recurrence in the placebo group; 155 pts participated in the crossover part 2 of the trial. A total of 297 (58%) pts completed the 1-yr pembro adjuvant treatment, of whom 47 had a recurrence ≥6 mths from the stop of treatment and 20 entered in the rechallenge part of the trial. Among 175 pts who started pembro in Part 2, 160 discontinued due to completion of therapy (N=24), disease progression (N=88), toxicity (N=20), investigator's decision (N=21), or other reason (N=7); 15 pts were still on-treatment. Results for the 2 groups are provided in the table. The median number of doses was 12 and 5.5, respectively (resp), and the median follow-up was 41 and 19 mts, resp. Among the 175 pts, 51 (29%) had a grade 1-4 irAE (by group: 47 [30%] and 4 [20%] resp) and 11 (6%) a grade 3-4 irAE. Conclusions: Pembrolizumab treatment after crossover yielded a 39% ORR in evaluable pts and an overall 3-yr PFS of ̃32%, but after rechallenge the efficacy was lower. Merck Sharp & Dohme Corp., a subsidiary of Merck & Co., Inc., Kenilworth, NJ, USA The median PFS (95% CI) from start of Part 2 was 14 (5-27) and 8 (5-15) mts for stage III-resected and III/IV various, resp. Among the 80 stage IV crossover pts with evaluable disease, 31 (39%) had an objective response: 14 (18%) CR, 17 (21%) PR. The 2-yr PFS rate from response was 69% (95% CI 48-83%). For these 80 pts, the median PFS was 6.1 mts and the 3-yr PFS rate was 31% (95% CI 21-41%). Among 9 stage IV rechallenged pts with an evaluable disease, 1 (11%) reached CR, 3 had SD and 5 PD. Clinical trial information: NCT02362594. [Table: see text]


2018 ◽  
pp. 415-420
Author(s):  
Abdullah Jibawi ◽  
Mohamed Baguneid ◽  
Arnab Bhowmick

The liver is the most common site for metastases. Perioperative investigations are explored and are critical to the decision as to whether liver metastases should be treated with curative intent. Careful preoperative staging and discussion at a multidisciplinary team meeting is standard practice in established units. This chapter examines surgical management options as well as the neoadjuvant therapies. It details a treatment algorithm for synchronous metastatic colon cancer. It explores newer techniques for improving resectability.


Blood ◽  
1991 ◽  
Vol 77 (8) ◽  
pp. 1717-1722 ◽  
Author(s):  
RJ Berenson ◽  
WI Bensinger ◽  
RS Hill ◽  
RG Andrews ◽  
J Garcia-Lopez ◽  
...  

Abstract The CD34 antigen is expressed by 1% to 4% of human and baboon marrow cells, including virtually all hematopoietic progenitors detectable by in vitro assays. Previous work from our laboratory has shown that CD34+ marrow cells can engraft lethally irradiated baboons. Because the CD34 antigen has not been detected on most solid tumors, positive selection of CD34+ cells may be used to provide marrow cells capable of engraftment, but depleted of tumor cells. In seven patients with stage IV breast cancer and two patients with stage IV neuroblastoma, 2.5 to 17.5 x 10(9) marrow cells were separated by immunoadsorption with the anti-CD34 antibody 12–8 and 50 to 260 x 10(6) positively selected cells were recovered that were 64 +/- 16% (range 35% to 92%) CD34+. The patients received 1.0 to 5.2 x 10(6) CD34-enriched cells/kg after marrow ablative therapy. Six patients engrafted, achieving granulocyte counts of greater than 500/mm3 at 34 +/- 10 (range 21 to 47) days and platelets counts of greater than 20,000/mm3 at 46 +/- 14 (range 28 to 66) days posttransplant. Five of these patients showed durable engraftment until the time of death 82 to 386 days posttransplant. One patient failed to sustain engraftment associated with metastatic marrow disease. Three patients died at days 14, 14, and 17 posttransplant, two of whom had evidence of early engraftment. These studies suggest that CD34+ marrow cells are capable of reconstituting hematopoiesis in humans.


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