scholarly journals Management and outcome of metastatic pheochromocytomas/paragangliomas: a monocentric experience

2021 ◽  
Author(s):  
G. De Filpo ◽  
G. Cantini ◽  
G. Rastrelli ◽  
G. Vannini ◽  
T. Ercolino ◽  
...  
Keyword(s):  
Multidisciplinary Approach ◽  
Progression Free Survival ◽  
Initial Diagnosis ◽  
University Hospital ◽  
Inherited Disease ◽  
Biochemical Activity ◽  
Free Survival ◽  
Sporadic Disease ◽  
Worse Prognosis

Abstract Background Pheochromocytoma (PHEO) and paraganglioma (PGL) are rare neuroendocrine tumors releasing catecholamines. Metastatic pheochromocytomas/paragangliomas (PPGLs) occur in about 5–26% of cases. To date, the management of patients affected by metastatic disease is a challenge in the absence of guidelines. Aim The aim of this study was to evaluate the overall survival (OS) and the progression-free survival (PFS) in metastatic PPGLs. Methods Clinical data of 20 patients referred to the Careggi University Hospital (Florence, Italy) were retrospectively collected. Follow-up ranged from 1989 to 2019. Site and size of primary tumor, biochemical activity, genetic analysis and employed therapies were considered. Data were analyzed with SPSS version 27. Results Nine PHEOs (45%) and 11 PGLs (55%) were enrolled. Median age at diagnosis was 43.5 years [30–55]. Mean follow-up was 104.6 ± 89.3 months. Catecholamines were released in 70% of cases. An inherited disease was reported in 50% of patients. OS from the initial diagnosis (OSpt) and from the metastatic appearance (OSmtx) were lower in older patients (OSpt p = 0.028; OSmtx p < 0.001), abdominal PGLs (OSpt p = 0.007; OSmtx p = 0.041), larger tumors (OSpt p = 0.008; OSmtx p = 0.025) and sporadic disease (OSpt p = 0.013; OSmtx p = 0.008). Conclusion Our data showed that older age at the initial diagnosis, sympathetic extra-adrenal localization, larger tumors and wild-type neoplasms are related to worse prognosis. Notably, the employed therapies do not seem to influence the survival of our patients. At present, effective treatments for metastatic PPGLs are missing and a multidisciplinary approach is indispensably required.

scholarly journals One-Year Progression-Free Survival of Therapy-Naive Patients With Malignant Pheochromocytoma and Paraganglioma

2013 ◽  
Vol 98 (10) ◽  
pp. 4006-4012 ◽  
Author(s):  
Ségolène Hescot ◽  
Sophie Leboulleux ◽  
Laurence Amar ◽  
Delphine Vezzosi ◽  
Isabelle Borget ◽  
...  
Keyword(s):  
Evaluation Criteria ◽  
Progression Free Survival ◽  
Malignant Pheochromocytoma ◽  
Inherited Disease ◽  
High Power Field ◽  
Free Survival ◽  
History Of ◽  
Wait And See ◽  
One Year

Abstract Context: The natural history of malignant pheochromocytoma or paragangliomas (MPP) remain unknown. Objective: The primary aim of this study was to define progression-free survival at 1 year in therapy-naive patients with MPP. Secondary objectives were to characterize MPP and to look for prognostic parameters for progression at 1 year. Design and Setting: The files of MPP followed up between January 2001 and January 2011 in two French Endocrine Networks were retrospectively reviewed. Therapy-naive patients were enrolled. Main Outcome Measures: The main outcome was progression-free survival at 1 year in therapy-naive MPP patients according to Response Evaluation Criteria In Solid Tumors 1.1 criteria. Results: Ninety files (46 men, 44 women, mean age of 47.5 ± 15 years) were reviewed on site by one investigator. MPP characteristics were as follows: presence of an adrenal primary, a mitotic count exceeding 5 per high power field, hypertension, inherited disease, and presence of bone metastases in 50%, 22%, 60%, 49%, and 56% patients, respectively. Fifty-seven of the 90 patients with MPP (63%) were classified as therapy-naive. The median follow-up of these 57 patients was 2.4 years (range, 0.4–5.7). At 1 year, progression-free survival was 46% (CI 95: 33–59). Twenty-six of 30 (87%) patients with progression at 1 year had exhibited progressive disease at the first imaging workup performed after a median of 5.7 months. No prognostic parameter was identified. Conclusions: Half of the therapy-naive patients with MPP achieved stable disease at 1 year. In symptom-free patients with MPP, a wait-and-see antitumor policy seems appropriate as first line. Modality for a prospective follow-up is proposed.

Radiation-associated versus sporadic osteosarcoma: A single-institution experience.

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. 11018-11018
Author(s):  
Brittany Siontis ◽  
Emily Roberts ◽  
Lili Zhao ◽  
Jonathan B. McHugh ◽  
Dawn Owen ◽  
...  
Keyword(s):  
Rare Complication ◽  
Progression Free Survival ◽  
Primary Malignancy ◽  
Free Survival ◽  
Significant Difference ◽  
Detectable Difference ◽  
History Of ◽  
Median Cumulative Dose ◽  
Worse Prognosis

11018 Background: Osteosarcoma (osarc) can be a rare complication from radiation (rt) therapy. Radiation-associated osarc (RAO) is reported to have a worse prognosis than non rt-associated osarc with limited objective data comparing the two. We conducted a retrospective study comparing demographics, therapy and outcomes of sporadic osarc (SO) to RAO. This study was confined to adults. Methods: We identified patients (pts) > age 18 years (yr) with osarc treated at our institution between 1990 and 2016 using an institutional database. We categorized tumors as SO or RAO based on history of prior rt within field of osarc. We extracted data on demographics, treatment, and primary malignancy characteristics. Results: We identified 159 pts with osarc, 28 were RAO tumors. Results are in Table 1. Median follow-up was 2.8 yr (0.1-19.6 yr). For RAO, median time from rt to diagnosis was 11.5 yr (1.5-28 yr) with a median cumulative dose of 60 Gy (44-75.8 Gy). Median progression free survival (PFS) and overall survival (OS) were not significantly different in pts presenting with metastatic osarc; PFS 10.3 mo vs 4.8 mo (p=0.45) and OS 15.6 mo vs 6.1 mo (p=0.96) in SO vs RAO pts, respectively. For pts with localized osarc, median relapse-free survival (RFS) and OS were significantly different, not reached vs 12.2 mo (p<0.001) and not reached vs 27.6 mo (p=0.001) in SO vs RAO, respectively. Conclusions: In our series, there was a significant difference in age, size and location of RAO vs non rt-associated osarc. Overall, all osarc pts with metastatic disease at diagnosis fared poorly. Pts presenting with localized RAO had worse outcomes than patients with localized SO. This was not associated with a detectable difference in therapy rendered or treatment effect in resection specimens. [Table: see text]

CCTG CO.26: Updated analysis and impact of plasma-detected microsatellite stability (MSS) and tumor mutation burden (TMB) in a phase II trial of durvalumab (D) plus tremelimumab (T) and best supportive care (BSC) versus BSC alone in patients (pts) with refractory metastatic colorectal carcinoma (rmCRC).

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. 3512-3512 ◽  
Author(s):  
Eric Xueyu Chen ◽  
Derek J. Jonker ◽  
Jonathan M. Loree ◽  
Hagen F. Kennecke ◽  
Scott R. Berry ◽  
...  
Keyword(s):  
Progression Free Survival ◽  
Best Supportive Care ◽  
P Value ◽  
Free Survival ◽  
Tumor Mutation Burden ◽  
Mutation Burden ◽  
Microsatellite Stability ◽  
Worse Prognosis ◽  
Clinical Trial Information

3512 Background: Targeting both PD-L1 and CTLA-4 may be synergistic immunotherapy approaches. CO.26 evaluated if dual inhibition leads to improved pt survival vs BSC alone in rmCRC. Methods: rmCRC pts were randomized 2:1 to D+T vs BSC. Treatment consisted of D (1500 mg) D1 q 28 days and T (75 mg) D1 for first 4 cycles, and supportive measures. Primary endpoint was overall survival (OS). Two-sided p < 0.10 was considered statistically significant. Cell-free (cf)DNA sequencing for MSI and TMB used GuardantOMNI panel and baseline plasma. Results: From 08/2016-06/2017, 180 pts were enrolled. Pt characteristics were balanced between arms. At median follow-up of 15.2 months (mos), median OS was 6.6 mos for D+T and 4.1 mos for BSC (p = 0.07; Hazard ratio (HR): 0.72, 90% confidence interval (CI): 0.54 – 0.97). Progression free survival (PFS) was 1.8 mos vs 1.9 mos, respectively (HR 1.01, 90% CI 0.76 – 1.34). Disease control rate (DCR) was 22.6% for D+T and 6.6% for BSC (p = 0.006). cfDNA analysis was successful in 168/169 pts (99.4%). Two pts were MSI-high. In 166 MSS pts, OS HR was 0.66 (p=0.024; 90% CI 0.49-0.89). Excluding the MSI-H cases (TMB of 74.7 and 247.1 mts/Mb), mean TMB was 20.4 ± 16.3 mts/Mb (range: 0.96 – 114.0). In MSS pts, a pre-specified cutpoint of 20 mts/Mb stratified pts into high and low TMB groups but was not predictive for OS , PFS, or DCR (interaction p-values > 0.7). Using a minimum p-value approach, pts with TMB >28 mts/Mb (21% of MSS pts) had the greatest OS benefit (HR 0.34, 90% CI 0.18-0.63) for D+T (interaction p = 0.07). High TMB was associated with a trend in worse prognosis for OS in the BSC arm using both 20 mts/Mb (HR 1.26, 90% CI 0.76-2.12) and 28 mts/Mb (HR 2.59 90% CI 1.46-4.62) cutpoints. Conclusions: D+T significantly prolonged OS in pts with rmCRC. High TMB may select a group of MSS pts who benefit from D+T. Plasma TMB appeared prognostic in the BSC arm. This is the first study showing combined PD-L1 and CTLA-4 inhibition prolongs survival in pts with MSS rmCRC. Updated results based on deaths in more than 90% of pts will be presented. Clinical trial information: NCT02870920.

Multidisciplinary approach to patients with recurrent colorectal liver metastases.

2016 ◽  
Vol 34 (4_suppl) ◽  
pp. 426-426
Author(s):  
Luca Vigano ◽  
Guido Costa ◽  
Tiziana Comito ◽  
Vittorio Pedicini ◽  
Dario Poretti ◽  
...  
Keyword(s):  
Survival Benefit ◽  
Multidisciplinary Approach ◽  
Standard Treatment ◽  
Stereotactic Body Radiation Therapy ◽  
Progression Free Survival ◽  
Colorectal Metastases ◽  
Free Survival ◽  
Aggressive Management ◽  
Hepatic Lesions

426 Background: Recurrence after liver resection (LR) for colorectal metastases (CLM) is common. Re-resection is the standard treatment. In unresectable patients, radiofrequency ablation (RFA) and stereotactic body radiation therapy (SBRT) have been proposed, but they did not show a clear survival benefit in comparison with chemotherapy alone. The present study aims to elucidate the outcome of patients with recurrent CLM according to the received treatment. Methods: A series of 323 consecutive patients undergoing first LR between 2004 and 2013 was considered. Of these, 206 (63.8%) had recurrence and were analyzed. Redo surgery was scheduled whenever possible. If surgery was excluded, RFA was considered for hepatic lesions, while SBRT for both hepatic and extra-hepatic lesions. Results: Among the 206 analyzed patients with recurrence, 72 underwent re-resection, 19 RFA and 14 SBRT. Liver-only recurrences were treated in 68.3% of cases (69/101, 47 surgery), pulmonary ones in 57.9% (22/38, 15 surgery) and other extra-hepatic ones in 20.9% (14/67, 10 surgery). After a median follow-up of 32 months, three-year survival after the diagnosis of recurrence (OS) was 42.2% (median 37.4 months). OS was associated with the administered treatment: at three years 70.5% if surgery, 53.8% if RFA/SBRT, and 15.0% if chemotherapy alone, p < 0.0001. The same association was observed focusing the analysis on patients with a total of three lesions in the liver and/or the lung (1-3 liver/lung group, n = 86): three-year OS was 82.6% if surgery, 55.8% if RFA/SBRT, and 29.2% if chemotherapy, p < 0.0001. Progression-free survival after the treatment of recurrence (PFS) was associated with the received treatment: at two years 29.0% if surgery, 14.4% if RFA/SBRT, and 2.6% if chemotherapy, p < 0.0001. The same result was observed in the “1-3 liver/lung” group: two-year PFS was 32.1% if surgery, 18.3% if RFA/SBRT, and 0% if chemotherapy, p = 0.001. RFA and SBRT groups had similar OS and PFS. Conclusions: Aggressive management of recurrent CLM prolongs OS and PFS. Surgery is the best treatment option. Despite potential selection biases, RFA and SBRT should be considered in unresectable patients because they are associated with better outcome than chemotherapy alone.

Surgery and peptide receptor radionuclide therapy: An effective multimodal approach for metastatic neuroendocrine tumors.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. 4113-4113
Author(s):  
Andreja Frilling ◽  
Ashley Clift ◽  
Adil Al-Nahhas ◽  
Richard P. Baum ◽  
Daniel Kaemmerer
Keyword(s):  
Overall Survival ◽  
Radionuclide Therapy ◽  
Peptide Receptor Radionuclide Therapy ◽  
Progression Free Survival ◽  
Initial Diagnosis ◽  
Surgical Morbidity ◽  
Naive Patient ◽  
Free Survival ◽  
Peptide Receptor

4113 Background: Neuroendocrine neoplasia (NEN) of the pancreas (PanNEN) or small bowel (SBNEN) frequently present with metastases at initial diagnosis, undermining the efficacy of surgical treatment. Peptide receptor radionuclide therapy (PRRT) with radiolabelled somatostatin analogues, 90Y-DOTATOC and 177Lu-DOTATATE, has been shown to achieve prolonged progression-free survival (PFS) and overall survival (OS) in a substantial number of non-surgical patients with advanced NEN. Our aim was to prospectively determine the efficacy of a combination of radical loco-regional surgery and 177Lu PRRT in patients with metastasised NEN. Methods: A set of inclusion criteria was defined (e.g. PanNEN or SBNEN, G1/G2 NEN, initial tumour diagnosis, treatment naïve patient, stage IV NEN, positivity on 68Ga DOTATATE or DOTATOC PET/CT, eligibility for surgery and PRRT). Patients underwent PRRT within 3 months following surgery. Follow-up included biochemistry and imaging. Outcome measures included 1-, 3-, and 5-year OS and PFS from initial diagnosis. Results: Forty-one patients met eligibility criteria and were included. There were 26 males (63.4%) and median age at surgery was 58.8 years (range 32.1-78.3). All patients with SBNEN underwent right hemicolectomy, terminal ileal resection and mesenteric lympadenectomy. In PanNEN patients either Whipple procedure or distal pancreatectomy and peripancreatic lymphadenectomy were performed. The median number of PRRT cycles was 4 (range 2-6). Post-treatment mortality was 0%. Surgical morbidity was 12% (all grade 1 according Clavien-Dindo) and transient grade 1 toxicity occurred post PRRT in 40%. There was no grade 3 toxicity. Median follow-up was 5.48 years (range 0.53 – 11.98). Median PFS and OS were 3.33 years and 9.07 years, respectively. Progression-free survival (with 95% CI) was at 1-, 3-, and 5-years 80% (68.7-92.6), 60.9% (45.9-75.9) and 43.3% (27.4-59.3), respectively. Overall survival (with 95% CI) at 1-, 3-, and 5-years was 97.6% (93-100), 97.6% (93-100), and 95% (87-100), respectively. Conclusions: Radical loco-regional surgery for primary tumours combined with PRRT provides a novel, highly efficacious approach in metastasised NEN.

scholarly journals Treatment of Angiosarcoma with Pazopanib and Paclitaxel: Results of the EVA (Evaluation of Votrient® in Angiosarcoma) Phase II Trial of the German Interdisciplinary Sarcoma Group (GISG-06)

Cancers ◽  
2021 ◽  
Vol 13 (6) ◽  
pp. 1223
Author(s):  
Daniel Pink ◽  
Dimosthenis Andreou ◽  
Sebastian Bauer ◽  
Thomas Brodowicz ◽  
Bernd Kasper ◽  
...  
Keyword(s):  
Immune Checkpoint Inhibitors ◽  
Median Overall Survival ◽  
Phase Ii Trial ◽  
Checkpoint Inhibitors ◽  
Progression Free Survival ◽  
Free Survival ◽  
Safety And Efficacy ◽  
Future Studies ◽  
Entire Cohort

We aimed to evaluate the efficacy and toxicity of paclitaxel combined with pazopanib in advanced angiosarcoma (AS). The primary end point was progression-free survival (PFS) rate at six months (PFSR6). Planned accrual was 44 patients in order to detect a PFSR6 of >55%, with an interim futility analysis of the first 14 patients. The study did not meet its predetermined interim target of 6/14 patients progression-free at 6 months. At the time of this finding, 26 patients had been enrolled between July 2014 and April 2016, resulting in an overrunning of 12 patients. After a median follow-up of 9.5 (IQR 7.7–15.4) months, PFSR6 amounted to 46%. Two patients had a complete and seven patients a partial response. Patients with superficial AS had a significantly higher PFSR6 (61% vs. 13%, p = 0.0247) and PFS (11.3 vs. 2.7 months, p < 0.0001) compared to patients with visceral AS. The median overall survival in the entire cohort was 21.6 months. A total of 10 drug-related serious adverse effects were reported in 5 patients, including a fatal hepatic failure. Although our study did not meet its primary endpoint, the median PFS of 11.6 months in patients with superficial AS appears to be promising. Taking recent reports into consideration, future studies should evaluate the safety and efficacy of VEGFR and immune checkpoint inhibitors with or without paclitaxel in a randomized, multiarm setting.

Evolution of Surgical Approaches in the Treatment of Petroclival Meningiomas: A Retrospective Review

2007 ◽  
Vol 61 (suppl_5) ◽  
pp. ONS202-ONS211 ◽  
Author(s):  
Nicholas C. Bambakidis ◽  
U. Kumar Kakarla ◽  
Louis J. Kim ◽  
Peter Nakaji ◽  
Randall W. Porter ◽  
...  
Keyword(s):  
Survival Rates ◽  
Progression Free Survival ◽  
Surgical Approaches ◽  
Single Institution ◽  
Short Term ◽  
Total Resection ◽  
Free Survival ◽  
Petroclival Meningioma ◽  
Petroclival Meningiomas

Abstract Objective: We examined the surgical approaches used at a single institution to treat petroclival meningioma and evaluated changes in method utilization over time. Methods: Craniotomies performed to treat petroclival meningioma between September of 1994 and July of 2005 were examined retrospectively. We reviewed 46 patients (mean follow-up, 3.6 yr). Techniques included combined petrosal or transcochlear approaches (15% of patients), retrosigmoid craniotomies with or without some degree of petrosectomy (59% of patients), orbitozygomatic craniotomies (7% of patients), and combined orbitozygomatic-retrosigmoid approaches (19% of patients). In 18 patients, the tumor extended supratentorially. Overall, the rate of gross total resection was 43%. Seven patients demonstrated progression over a mean of 5.9 years. No patients died. At 36 months, the progression-free survival rate for patients treated without petrosal approaches was 96%. Of 14 patients treated with stereotactic radiosurgery, none developed progression. Conclusion: Over the study period, a diminishing proportion of patients with petroclival meningioma were treated using petrosal approaches. Utilization of the orbitozygomatic and retrosigmoid approaches alone or in combination provided a viable alternative to petrosal approaches for treatment of petroclival meningioma. Regardless of approach, progression-free survival rates were excellent over short-term follow-up period.

scholarly journals FAS and Subsequent Therapies in Pancreatic Neuroendocrine Tumors

2021 ◽  
Author(s):  
Jane E. Rogers ◽  
Michael Lam ◽  
Daniel M. Halperin ◽  
Cecile G. Dagohoy ◽  
James C. Yao ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Cox Regression ◽  
Progression Free Survival ◽  
Pancreatic Neuroendocrine Tumors ◽  
Free Survival ◽  
Prior Therapy ◽  
Well Differentiated ◽  
Grade 3 ◽  
Line Of Therapy

We evaluated outcomes of treatment with 5-fluorouracil (5-FU), doxorubicin, and streptozocin (FAS) in well-differentiated pancreatic neuroendocrine tumors (PanNETs) and its impact on subsequent therapy (everolimus or temozolomide). Advanced PanNET patients treated at our center from 1992 to 2013 were retrospectively reviewed. Patients received bolus 5-FU (400 mg/m2), streptozocin (400 mg/m2) (both IV, days 1-5) and doxorubicin (40 mg/m2 IV, day 1) every 28 days. Overall response rate (ORR) was assessed using RECIST version 1.1. Of 243 eligible patients, 220 were evaluable for ORR, progression-free survival (PFS), and toxicity. Most (90%) had metastatic, nonfunctional PanNETs; 14% had prior therapy. ORR to FAS was 41% (95% confidence interval [CI]: 36-48%). Median follow-up was 61 months. Median PFS was 20 (95% CI: 15-23) months; median overall survival (OS) was 63 (95% CI: 60-71) months. Cox regression analyses suggested improvement with first-line vs subsequent lines of FAS therapy. Main adverse events ≥ grade 3 were neutropenia (10%) and nausea/vomiting (5.5%). Dose reductions were required in 32% of patients. Post-FAS everolimus (n=108; 68% second line) had a median PFS of 10 (95% CI: 8-14) months. Post-FAS temozolomide (n=60; 53% > fourth line) had an ORR of 13% and median PFS of 5.2 (95% CI: 4-12) months. In this largest reported cohort of PanNETs treated with chemotherapy, FAS demonstrated activity without significant safety concerns. FAS did not appear to affect subsequent PFS with everolimus; this sequence is being evaluated prospectively. Responses were noted with subsequent temozolomide-based regimens although PFS was possibly limited by line of therapy.

scholarly journals Cetuximab versus bevacizumab following prior FOLFOXIRI and bevacizumab in postmenopausal women with advanced KRAS and BRAF wild-type colorectal cancer: a retrospective study

BMC Cancer ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Chunlong Huang ◽  
Xiaoyuan Gu ◽  
Xianshang Zeng ◽  
Baomin Chen ◽  
Weiguang Yu ◽  
...  
Keyword(s):  
Postmenopausal Women ◽  
Survival Benefit ◽  
Progression Free Survival ◽  
Wild Type ◽  
Free Survival ◽  
Primary Endpoints ◽  
Significant Difference ◽  
Inductive Treatment ◽  
Advanced Colorectal Carcinoma

Abstract Background An upgraded understanding of factors (sex/estrogen) associated with survival benefit in advanced colorectal carcinoma (CRC) could improve personalised management and provide innovative insights into anti-tumour mechanisms. The aim of this study was to assess the efficacy and safety of cetuximab (CET) versus bevacizumab (BEV) following prior 12 cycles of fluorouracil, leucovorin, oxaliplatin, and irinotecan (FOLFOXIRI) plus BEV in postmenopausal women with advanced KRAS and BRAF wild-type (wt) CRC. Methods Prospectively maintained databases were reviewed from 2013 to 2017 to assess postmenopausal women with advanced KRAS and BRAF wt CRC who received up to 12 cycles of FOLFOXIRI plus BEV inductive treatment, followed by CET or BEV maintenance treatment. The primary endpoints were overall survival (OS), progression-free survival (PFS), response rate. The secondary endpoint was the rate of adverse events (AEs). Results At a median follow-up of 27.0 months (IQR 25.1–29.2), significant difference was detected in median OS (17.7 months [95% confidence interval [CI], 16.2–18.6] for CET vs. 11.7 months [95% CI, 10.4–12.8] for BEV; hazard ratio [HR], 0.63; 95% CI, 0.44–0.89; p=0.007); Median PFS was 10.7 months (95% CI, 9.8–11.3) for CET vs. 8.4 months (95% CI, 7.2–9.6) for BEV (HR, 0.67; 95% CI 0.47–0.94; p=0.02). Dose reduction due to intolerable AEs occurred in 29 cases (24 [24.0%] for CET vs. 5 [4.8%] for BEV; p< 0.001). Conclusions CET tends to be superior survival benefit when compared with BEV, with tolerated AEs.

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