scholarly journals Innovative approach to a functional mediastinal paraganglioma with anomalous coronary supply: a case report

2020 ◽  
Vol 4 (3) ◽  
pp. 1-6 ◽  
Author(s):  
Maria Trêpa ◽  
Inês Silveira ◽  
Cláudia Amaral ◽  
André Luz

Abstract Background Mediastinal paragangliomas (PGs) are rare and particularly challenging neuroendocrine tumours. Clinical presentation is heterogeneous and tumour resection can be challenging due to bleeding and the risk of catecholamine surges in functional tumours. Case Summary A 36-year-old man with multiple cardiovascular risk factors was admitted with subacute heart failure. Investigations revealed a large non-metastatic functional mediastinal PG irrigated mainly by a left circumflex coronary anomalous feeder branch. The surgical risk was deemed very high due to patient comorbidities, tumour vascularization, and close relation to major thoracic structures. A multidisciplinary team decided to perform embolization of the anomalous coronary branch followed by peptide-receptor radionuclide therapy with 177-LuDOTATE aiming to decrease tumour size and perioperative risk. Follow-up studies showed a reduction in tumour vascularization, size, and hormonal production. Discussion The innovative strategy of combining embolization of the anomalous feeder branch with radionuclide therapy proved to be a promising approach.

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e15697-e15697
Author(s):  
Andreja Frilling ◽  
Daniel Kaemmerer ◽  
Mark S. Kidd ◽  
Ashley K Clift ◽  
Justin Stebbing ◽  
...  

e15697 Background: Neuroendocrine tumours (NET) of the pancreas (PNET) or small bowel (SBNET) 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) in a substantial number of non-surgical patients with advanced NET. Our aim was to prospectively determine the efficacy of a combination of radical loco-regional surgery and PRRT in patients with metastasised NET. Methods: A set of inclusion criteria was defined (e.g. G1/G2 NET, initial tumour diagnosis, treatment naïve patient, stage IV NET, positivity on 68Ga DOTA- PET/CT, eligibility for surgery and PRRT). Patients underwent PRRT within 3 months following surgery. Follow-up included biochemistry and imaging. In a sub-cohort, blood-based neuroendocrine gene transcript analysis of 51 genes (NETest) was used to define the effectiveness of treatment. Outcome measures included 5-year overall survival (OS) and PFS from initial diagnosis. Results: Twenty-five patients (5 PNET, 20 SBNET) met eligibility criteria and were included. There were 13 men (52%) and mean age was 57.1 years. All patients with SB NET underwent right hemicolectomy, terminal ileal resection and mesenteric lympadenectomy. In all PNET patients only limited pancreatic resection was required. The median number of PRRT cycles was 4 (range 2-6). Post-treatment mortality was 0%. Surgical morbidity was 12% (all Clavien-Dindo grade 1). Transient grade 1 toxicity occurred post-PRRT in 40%. NETest scores were increased in 8 patients (100%) pre-operatively and decreased in all following treatment. NETest decreases correlated with diminished tumour volume (R2=0.37, p=0.02). Median follow-up was 48 months (range 12-72months). Five-year OS was 90% and 5-year PFS was 84.3%. Conclusions: Radical loco-regional surgery for primary tumours combined with PRRT provides a novel, highly efficacious approach in metastasised NET. The NETest accurately measures the effectiveness of treatment.


2020 ◽  
Vol 38 (4_suppl) ◽  
pp. 604-604 ◽  
Author(s):  
Nick Pavlakis ◽  
David Turner Ransom ◽  
David Wyld ◽  
Katrin Marie Sjoquist ◽  
Rebecca Asher ◽  
...  

604 Background: Single agent 177Lu-octreotate peptide receptor radionuclide therapy is now a standard of care for progressive mNETS. High activity was seen with LuTate and concurrent CAPTEM chemotherapy in a single arm Phase I/II trial. This study was undertaken to determine the relative activity of adding CAPTEM to LuTate PRRT in patients with mNETs. Methods: Non-comparative randomised open label phase II trial of PRRT +/- CAPTEM in patients with mNETs, with 2:1 randomisation: PRRT /CAPTEM (experimental arm) vs. PRRT (control). PRRT /CAPTEM: 7.8GBq LuTate day(D) 10, 8 weekly (wkly) x 4, with b.i.d. oral CAP 750mg/m2 D1-14 & TEM 75mg/m2 D10-14, 8 wkly x 4, vs. PRRT 8 wkly x 4. Primary endpoint: progression free survival (PFS) at 15 months assuming 15 month PFS of 66.4% in the control arm, aiming for PFS rate > 80%; secondary endpoints: objective tumour response rate (complete or partial response) (OTRR), clinical benefit rate (complete or partial response, stable disease) (CBR), toxicity, and QOL. Results: 47 patients enrolled (Dec 2015 - Feb 2018): 33 PRRT/CAPTEM and 14 PRRT. Two patients withdrew prior to treatment. Patient characteristics were balanced except gender (female 58% vs. 14%). Two patients received 2 prior systemic regimens. After a median follow-up of 32 months, the 15 month PFS was 90% (95% CI: 73-97%) v 92% (95% CI: 57-99%); OTRR 25% vs 15%; and CBR 97% vs 92% for PRRT/CAPTEM v PRRT respectively. For treatment related adverse events 22/32 CAPTEM patients experienced one Grade 3 event (69%) vs 5/13 (38%, PRRT); 4/32 pts experienced one Grade 4 event (13%) v 1/13 (8%) respectively. Only one patient failed to complete therapy due to toxicity (PRRT/CAPTEM). Conclusions: This initial planned analysis demonstrates similarly high 15 month PFS for CAPTEM/PRRT relative to PRRT alone. OTRR is numerically higher but at the cost of greater toxicity. Longer follow up is required to determine if the activity of PRRT/CAPTEM is sufficient to warrant Phase III evaluation. Clinical trial information: ACTRN12615000909527.


2017 ◽  
Vol 24 (5) ◽  
pp. 243-251 ◽  
Author(s):  
Tessa Brabander ◽  
Wouter A van der Zwan ◽  
Jaap J M Teunissen ◽  
Boen L R Kam ◽  
Wouter W de Herder ◽  
...  

Peptide receptor radionuclide therapy (PRRT) with [177Lu-DOTA0,Tyr3]octreotate (177Lu-DOTATATE) is a treatment with good results in patients with metastatic gastroenteropancreatic neuroendocrine tumours (GEPNETs). However, there are some pitfalls that should be taken into consideration when evaluating the treatment response after PRRT. 354 Dutch patients with GEPNETs who were treated with 177Lu-DOTATATE between March 2000 and December 2011 were retrospectively selected. Liver function parameters and chromogranin A were measured before each therapy and in follow-up. Anatomical imaging was performed before therapy and in follow-up. An increase in aminotransferases by ≥20% compared to baseline was observed in 83 of 351 patients (24%). In patients with an objective response (OR) and stable disease (SD) this increase was observed in 71/297 (24%) and in patients with progressive disease (PD) it was observed in 12/54 patients (22%). An increase in chromogranin A by ≥20% compared to baseline was observed in 76 patients (29%). This was present in 34% of patients who eventually had PD and 27% of patients who had OR/SD. In 70% of patients this tumour marker returned to baseline levels after therapy. An increase in liver enzymes and chromogranin A is not uncommon after PRRT. In the vast majority of patients this will resolve in follow-up. Clinicians should be aware that these changes may occur due to radiation-induced inflammation or disease progression and that repeated measurements over time are necessary to differentiate between the two.


2021 ◽  
Author(s):  
Esmail Jafari ◽  
Abdul Latif Amini ◽  
Hojjat Ahmadzadehfar ◽  
Dara Bagheri ◽  
Majid Assadi

Abstract Background The aim of this study was to determine the probable cardiotoxicity following radionuclide therapy (RNT), specifically peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE and radioligand therapy (RLT) with 177Lu-PSMA by evaluation of serum troponin I and cardiac profile change during a follow-up time. Materials and Methods Patients with prostate cancer and neuroendocrine tumours (NETs) referred for PRRT and RLT, respectively, were enrolled in this study. The cardiac profiles of the patients were evaluated by a cardiologist and a cardiac history was obtained from all patients. Also, troponin I was measured before and 48 hours after treatment. Results In this retrospective study for assessment of RLT associated cardiotoxicity, 24 patients were evaluated with a median age of 64 years (27–99 years) including 13 NET patients and 11 prostate cancer patients. Patients were followed up for 4 to 31 months which no cardiovascular problem was observed. In evaluation of troponin I, 39 RNT cycles were evaluated. In all patients, the value of troponin I was in normal range. In all patients, the median values of serum troponin I before and after treatment were 0.2 ± 0.02 (range: 0.00–0.42) and 0.28 ± 0.02 (range: 0.00–0.46) ng/ml, respectively (p > 0.05). In the prostate cancer patients, the median values of serum troponin I before and after treatment were 0.26 ± 0.04 (0.04–0.42) and 0.30 ± 0.04 (0.00–0.41) ng/ml, respectively (p > 0.05). In the NET patients, the median values of serum troponin I before and after treatment were 0.18 ± 0.03 (0.00–0.42) and 0.17 ± 0.03 (0.00–0.46) ng/ml, respectively (p > 0.05). Conclusion PRRT with 177Lu-DOTATATE and RLT with 177Lu-PSMA as emerging therapeutic modalities have no significant cardiotoxicity. However, further well-designed studies are recommended.


2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Sander C. Ebbers ◽  
Muriël Heimgartner ◽  
Maarten W. Barentsz ◽  
Rachel S. van Leeuwaarde ◽  
Mark J. C. van Treijen ◽  
...  

Abstract Background Early [68Ga]Ga-DOTA-TOC PET/CT imaging after peptide receptor radionuclide therapy (PRRT) in neuroendocrine neoplasm patients is often used as a prognosticator for survival, but lacks validity. This study investigates the prognostic value of changes in PET parameters after PRRT. Methods Baseline and follow-up [68Ga]Ga-DOTA-TOC PET/CT scans of all patients treated with PRRT were delineated automatically. Total lesion somatostatin receptor expression (TL-SSTR) and somatostatin receptor expressing tumor volume (SSTR-TV) were used as covariates in Cox proportional hazard models to predict time-to-new treatment. Results In twenty patients, median time-to-new treatment was 19.3 months (range [3.8; 36.2]). Absolute and percentual changes in both PET parameters were not associated with time-to-new treatment. A significant relation between independent baseline and follow-up SSTR-TV and follow-up TL-SSTR, and time-to-new treatment was identified. Conclusions Automatically derived [68Ga]Ga-DOTA-TOC PET/CT parameters are easy to acquire and may be of prognostic value after completing PRRT. Acquiring SSTR-TV or TL-SSTR parameters at baseline and during follow-up can be of value in identifying a patient’s prognosis.


2012 ◽  
Vol 30 (15_suppl) ◽  
pp. e14513-e14513
Author(s):  
Dieter Hörsch ◽  

e14513 Background: Peptide receptor radionuclide therapy is an effective treatment option for patients with well differentiated somatostatin receptor expressing neuroendocrine tumors. However, published data results mainly from retrospective monocentric studies. Methods: We initiated a multi-institutional, prospective and board reviewed registry for patients treated with Peptide receptor radionuclide therapy in Germany in 2009. Results: In five centers, 297 patients were registered. Primary tumors were mainly derived from pancreas (117/297), small intestine (80/297) whereas 56 were of unknown primary. Most tumors were well differentiated with a median Ki67 proliferation rate of 5% (range 0.9 to 70). Peptide receptor radionuclide therapy was performed using mainly Yttrium-90 and/or Lutetium-177 as radionuclides in 1-8 cycles. Mean overall survival was estimated at 213 months with a follow up between 1 and 230 months after initial diagnosis and 87 months with a follow up between 1 and 92 months after start of Peptide receptor radionuclide therapy. Median overall survival was not yet reached. Subgroup analysis demonstrated that best results were obtained in neuroendocrine tumors with a proliferation rate below 20%. Conclusions: Our results indicate that Peptide receptor radionuclide therapy is an effective treatment well and moderately differentiated neuroendocrine tumors irrespective of previous therapies and should be regarded a one of the primary treatment options for patients with neuroendocrine tumors.


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