Publications
448 results found
Clift A, Frilling A, Braat A, et al., 2020, Radioembolization for Neuroendocrine Liver Metastases: An Institutional Case Series, Systematic Review and Meta-Analysis, 17th Annual European-Neuroendocrine-Tumor-Society (ENETS) Conference for the Diagnosis and Treatment of Neuroendocrine Tumor Disease, Publisher: KARGER, Pages: 223-223, ISSN: 0028-3835
Malczewska A, Frampton A, Prado MM, et al., 2020, Diagnosis and Assessment of Effectiveness of Surgical Resection of Small Bowel Neuroendocrine Tumours: The Roles of Circulating MicroRNAs, 17th Annual European-Neuroendocrine-Tumor-Society (ENETS) Conference for the Diagnosis and Treatment of Neuroendocrine Tumor Disease, Publisher: KARGER, Pages: 145-145, ISSN: 0028-3835
Oberg K, Califano A, Strosberg J, et al., 2020, A Meta-Analysis of the Accuracy of a Neuroendocrine Tumor mRNA Genomic Biomarker (NETest) in Blood, 17th Annual European-Neuroendocrine-Tumor-Society (ENETS) Conference for the Diagnosis and Treatment of Neuroendocrine Tumor Disease, Publisher: KARGER, Pages: 149-149, ISSN: 0028-3835
Oberg K, Califano A, Strosberg JR, et al., 2020, A meta-analysis of the accuracy of a neuroendocrine tumor mRNA genomic biomarker (NETest) in blood, Annals of Oncology, Vol: 31, Pages: 202-212, ISSN: 0923-7534
BackgroundThe lack of an accurate blood biomarker in neuroendocrine tumor (NET) disease has hindered management. The advance of genomic medicine and the development of molecular biomarkers has provided a strategy—liquid biopsy—to facilitate real-time management. We reviewed the role of a blood mRNA-based NET biomarker, the NETest, as an in vitro diagnostic (IVD).Patients and methodsA systematic review of the literature using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines was undertaken. The methodological quality was evaluated using the QUADAS-2 tool. We identified ten original scientific papers that met the inclusion criteria. These were assessed by qualitative analysis and thereafter meta-analysis. Data were pooled and a median [95% confidence interval (CI)] diagnostic odds ratio (DOR), positive likelihood ratio (+LR), and negative likelihood ratio (−LR) were calculated. For the meta-analysis, a generic inverse variance method was undertaken using the accuracy and area under the curve (AUC) data.ResultsThe ten studies exhibited moderate to high methodological quality. They evaluated NETest usage both as a diagnostic and as a monitoring tool. The meta-analysis identified the diagnostic accuracy of the NETest to be 95%–96% with a mean DOR of 5 853, +LR of 195, and −LR of 0.06. The NETest was 84.5%–85.5% accurate in differentiating stable disease from progressive disease. As a marker of natural history, the accuracy was 91.5%–97.8%. As an interventional/response biomarker, the accuracy was 93.7%–97.4%. The pooled AUC for the NETest was 0.954 ± 0.005, with a z-statistic of 175.06 (P < 0.001).ConclusionsThe NETest is an accurate biomarker suitable for clinical use in NET disease management. The meta-analysis supports the utility of the NETest as an IVD to establish a diagnosis and monitor therapeutic efficacy. The use of this as a biomarker provides information relevan
Oberg KE, Califano A, Strosberg JR, et al., 2020, A meta-analysis of the accuracy of a neuroendocrine tumor mRNA biomarker (NETest) in the blood, Gastrointestinal Cancers Symposium of the American-Society-of-Clinical-Oncology, Publisher: AMER SOC CLINICAL ONCOLOGY, ISSN: 0732-183X
Sharma R, Wang WM, Yusuf S, et al., 2019, 68Ga-DOTATATE PET/CT parameters predict response to peptide receptor radionuclide therapy in neuroendocrine tumours, Radiotherapy and Oncology, Vol: 141, Pages: 108-115, ISSN: 0167-8140
PURPOSE: [177Lu]DOTATATE prolongs progression free survival (PFS) in metastatic neuroendocrine tumours (NETs). However, objective response rate is low. This, coupled with long duration of therapy and expense suggest need for better selection. We aim to assess whether baseline [68Ga]DOTATATE-PET/CT parameters, and whether response assessment by PET accurately predicts clinical outcome to [177Lu]DOTATATE. EXPERIMENTAL DESIGN: Retrospective study of patients receiving [177Lu]DOTATATE was conducted. Patients were followed 3-monthly until disease progression. Four [68Ga]DOTATATE-PET parameters (single lesion SUVmax, tumour to spleen and liver SUV ratios, and SUVmax-av using up to five target lesions in multiple organ sites) were determined at baseline and follow-up. The association between these PET parameters either at baseline, or any changes following treatment, and PET response criteria (PERCIST and modified PERCIST) to predict PFS were determined. Patients were followed 3-monthly until disease progression. Response was determined using RECIST 1.1. Baseline SSTR2 expression was assessed and compared with PET parameters. RESULTS: 55 patients with metastatic NETs were identified predominantly small bowel (N = 18) and pancreatic (N = 8) in origin. 16 were low grade, 15 intermediate and 3 high grade. Response to PRRT (N = 47): partial response (PR) 28%, stable disease (SD) 60% progressive disease (PD) 13%. Response to PRRT predicted PFS: PR 71.8 months (95%CI: not achieved), SD 29.1 months (95%CI: 15.2-43.1), and PD 9.7 months (95%CI: 0-21.02). Baseline, single lesion SUVmax predicted both response and PFS with SUV cut-off of 13.0 giving high sensitivity and specificity. Tumoural SUVmax correlated with SSTR2 expression, Spearman's rho - 0.69, p < 0.01. CONCLUSIONS: Baseline single lesion SUVmax and SUVmax-av predicts response to [177Lu]DOTATATE. Objective response following PRRT defines a subset of patients with markedly improved PFSBaseline SUVmax 13.0 defines a thre
Braat AJAT, Ahmadzadehfar H, Kappadath SC, et al., 2019, Radioembolization with Y-90 resin microspheres of neuroendocrine liver metastases after initial peptide receptor radionuclide therapy, Cardiovascular and Interventional Radiology, Vol: 43, Pages: 246-253, ISSN: 0174-1551
PurposePeptide receptor radionuclide therapy (PRRT) and radioembolization are increasingly used in neuroendocrine neoplasms patients. However, concerns have been raised on cumulative hepatotoxicity. The aim of this sub-analysis was to investigate hepatotoxicity of yttrium-90 resin microspheres radioembolization in patients who were previously treated with PRRT.MethodsPatients treated with radioembolization after systemic radionuclide treatment were retrospectively analysed. Imaging response according to response evaluation criteria in solid tumours (RECIST) v1.1 and clinical response after 3 months were collected. Clinical, biochemical and haematological toxicities according to common terminology criteria for adverse events (CTCAE) v4.03 were also collected. Specifics on prior PRRT, subsequent radioembolization treatments, treatments after radioembolization and overall survival (OS) were collected.ResultsForty-four patients were included, who underwent a total of 58 radioembolization procedures, of which 55% whole liver treatments, at a median of 353 days after prior PRRT. According to RECIST 1.1, an objective response rate of 16% and disease control rate of 91% were found after 3 months. Clinical response was seen in 65% (15/23) of symptomatic patients after 3 months. Within 3 months, clinical toxicities occurred in 26%. Biochemical and haematological toxicities CTCAE grade 3–4 occurred in ≤ 10%, apart from lymphocytopenia (42%). Radioembolization-related complications occurred in 5% and fatal radioembolization-induced liver disease in 2% (one patient). A median OS of 3.5 years [95% confidence interval 1.8–5.1 years] after radioembolization for the entire study population was found.ConclusionRadioembolization after systemic radionuclide treatments is safe, and the occurrence of radioembolization-induced liver disease is rare.
Sharma R, Wang W, Yusuf S, et al., 2019, [<SUP>68</SUP>Ga]-DOTATATE PET/CT parameters predict response to peptide receptor radionuclide therapy in neuroendocrine tumours, 32nd Annual Congress of the European-Association-of-Nuclear-Medicine (EANM), Publisher: SPRINGER, Pages: S640-S640, ISSN: 1619-7070
Clift AK, Kidd M, Bodei L, et al., 2019, Neuroendocrine neoplasms of the small bowel and pancreas, Neuroendocrinology: international journal for basic and clinical studies on neuroendocrine relationships, Vol: 110, Pages: 444-476, ISSN: 0028-3835
The traditionally promulgated perspectives of neuroendocrine neoplasms as rare, indolent tumours are blunt and have been outdated for the last two decades. Clear increments in their incidence over the past decades render them increasingly clinically relevant, and at initial diagnosis many present with nodal and/or distant metastases (notably hepatic). The molecular pathogenesis of these tumours is increasingly yet incompletely understood. Those arising from the small bowel or pancreas typically occur sporadically; the latter may occur within the context of hereditary tumour predisposition syndromes. Neuroendocrine neoplasms can also be associated with endocrinopathy of hormonal hypersecretion. Tangible advances in the development of novel biomarkers, functional imaging modalities and therapy are especially applicable to this sub-set of tumours. The management of small bowel and pancreatic neuroendocrine tumours may be challenging, and often comprises a multidisciplinary approach wherein surgical, medical, interventional radiological and radiotherapeutic modalities are implemented. This review provides a comprehensive overview of the epidemiology, pathophysiology, diagnosis and treatment of small bowel and pancreatic neuroendocrine tumours. Moreover, we provide an outlook of the future in these tumor types which will include the development of precision oncology frameworks for individualised therapy, multi-analyte predictive biomarkers, artificial intelligence-derived clinical decision support tools and elucidation of the role of the microbiome in neuroendocrine neoplasm development and clinical behaviour.
Frilling A, Clift AK, Braat AJAT, et al., 2019, Radioembolisation with 90Y microspheres for neuroendocrine liver metastases: an institutional case series, systematic review and meta-analysis, HPB, Vol: 21, Pages: 773-783, ISSN: 1365-182X
BACKGROUND: Neuroendocrine liver metastases are clinically challenging due to their frequent disseminated distribution. This study aims to present a British experience with an emerging modality, radioembolisation with yttrium-90 labelled microspheres, and embed this within a meta-analysis of response and survival outcomes. METHODS: A retrospective case series of patients treated with SIR-Spheres (radiolabelled resin microspheres) was performed. Results were included in a systematic review and meta-analysis of published results with glass or resin microspheres. Objective response rate (ORR) was defined as complete or partial response. Disease control rate (DCR) was defined as complete/partial response or stable disease. RESULTS: Twenty-four patients were identified. ORR and DCR in the institutional series was 14/24 and 21/24 at 3 months. Overall survival and progression-free survival at 3-years was 77.6% and 50.4%, respectively. There were no grade 3/4 toxicities post-procedure. A fixed-effects pooled estimate of ORR of 51% (95% CI: 47%-54%) was identified from meta-analysis of 27 studies. The fixed-effects weighted average DCR was 88% (95% CI: 85%-90%, 27 studies). CONCLUSION: Current data demonstrate evidence of the clinical effectiveness and safety of radioembolisation for neuroendocrine liver metastases. Prospective randomised studies to compare radioembolisation with other liver directed treatment modalities are needed.
Frilling A, Clift A, Al-Nahhas A, et al., 2019, Surgery and peptide receptor radionuclide therapy: An effective multimodal approach for metastatic neuroendocrine tumors., Annual Meeting of the American-Society-of-Clinical-Oncology (ASCO), Publisher: AMER SOC CLINICAL ONCOLOGY, ISSN: 0732-183X
Drymousis P, Clift AK, Leaman S, et al., 2019, SURGERY COMBINED WITH MULTIMODAL THERAPY - A NOVEL CONCEPT IN THE TREATMENT OF ADVANCED SMALL BOWEL NEUROENDOCRINE NEOPLASMS, Digestive Disease Week (DDW), Publisher: W B SAUNDERS CO-ELSEVIER INC, Pages: S1409-S1409, ISSN: 0016-5085
Braat AJAT, Kappadath SC, Ahmadzadehfar H, et al., 2019, Radioembolization with <SUP>90</SUP>Y Resin Microspheres of Neuroendocrine Liver Metastases: International Multicenter Study on Efficacy and Toxicity, CARDIOVASCULAR AND INTERVENTIONAL RADIOLOGY, Vol: 42, Pages: 413-425, ISSN: 0174-1551
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- Citations: 48
Carlsen EA, Fazio N, Granberg D, et al., 2019, Peptide receptor radionuclide therapy in gastroenteropancreatic NEN G3: a multicenter cohort study, Endocrine-Related Cancer, Vol: 26, Pages: 227-239, ISSN: 1351-0088
Peptide receptor radionuclide therapy (PRRT) is an established treatment of metastatic neuroendocrine tumors grade 1-2 (G1-G2). However, its possible benefit in high-grade gastroenteropancreatic (GEP) neuroendocrine neoplasms (NEN G3) is largely unknown. We therefore aimed to assess the benefits and side effects of PRRT in patients with GEP NEN G3. We performed a retrospective cohort study at 12 centers to assess the efficacy and toxicity of PRRT in patients with GEP NEN G3. Outcomes were response rate, disease control rate, progression-free survival (PFS), overall survival (OS) and toxicity. We included 149 patients (primary tumor: pancreatic n = 89, gastrointestinal n = 34, unknown n = 26). PRRT was first-line (n = 30), second-line (n = 62) or later-line treatment (n = 57). Of 114 patients evaluated, 1% had complete response, 41% partial response, 38% stable disease and 20% progressive disease. Of 104 patients with documented progressive disease before PRRT, disease control rate was 69%. The total cohort had median PFS of 14 months and OS of 29 months. Ki-67 21-54% (n = 125) vs Ki-67 ≥55% (n = 23): PFS 16 vs 6 months (P < 0.001) and OS 31 vs 9 months (P < 0.001). Well (n = 60) vs poorly differentiated NEN (n = 62): PFS 19 vs 8 months (P < 0.001) and OS 44 vs 19 months (P < 0.001). Grade 3-4 hematological or renal toxicity occurred in 17% of patients. This large multicenter cohort of patients with GEP NEN G3 treated with PRRT demonstrates promising response rates, disease control rates, PFS and OS as well as toxicity in patients with mainly progressive disease. Based on these results, PRRT may be considered for patients with GEP NEN G3.
Sorbye H, Baudin E, Borbath I, et al., 2019, Unmet needs in high-grade gastroenteropancreatic neuroendocrine neoplasms (WHO G3), Neuroendocrinology, Vol: 108, Pages: 54-62, ISSN: 0028-3835
Gastroenteropancreatic (GEP) neuroendocrine neoplasms (NEN) are classified based upon morphology and graded upon proliferation rate as either well-differentiated low-grade (G1-G2) neuroendocrine tumours (NET) or poorly differentiated high-grade (G3) neuroendocrine carcinoma (NEC). Recently, a new subgroup of well-differentiated high-grade pancreatic tumours (NET G3) has been defined. The GEP NEN G3 group consisting of both NEC and NET G3, has recently been shown to be a quite heterogeneous patient group concerning prognosis and treatment benefit, depending on factors such as primary tumour site, differentiation, proliferation rate and molecular alterations. In this review we discuss the existing data on diagnostics, treatment and biomarkers on this patient group, the unmet needs and the future perspectives.
Ramage JK, Punia P, Faluyi O, et al., 2019, Observational study to assess quality of life in patients with pancreatic neuroendocrine tumours receiving treatment with Everolimus: The OBLIQUE Study (UK Phase IV trial), Neuroendocrinology, Vol: 108, Pages: 317-327, ISSN: 0028-3835
Background/Aims. To assess health-related quality of life (HRQoL), treatment patterns and clinical outcomes of adult (≥18 years) patients with advanced (unresectable or metastatic) pancreatic neuroendocrine neoplasms (PanNENs) treated with everolimus in routine clinical practice. METHODS: In a prospective, non-interventional, multicentre study patients administered at least one 10 mg dose of everolimus were evaluated for change in HRQoL (EORTC QLQ-C30 Global Health Status scale) from baseline after 6 months treatment (primary endpoint). Secondary endpoints included disease-specific HRQoL measures (EORTC QLQ-G.I.NET21), clinical outcomes and everolimus treatment patterns and safety. RESULTS: Forty-eight patients were recruited (between Aug-2013 and Mar-2015); the median treatment duration was 27.8 months. EORTC QLQ-C30 Global Health score was not significantly different from baseline after 6 months of treatment (mean difference -1.9 points, p=0.660, n=30). In pairwise analyses, the only significant changes in HRQoL from baseline were for EORTC QLQ-C30 physical functioning score at Month 3 (adjusted mean difference -8.8 points, p=0.002, n=36), and the EORTC QLQ-G.I.NET21 disease-related worries scores at months 1 and 2 (adjusted mean differences: -11.5 points [p=0.001, n=44] and -8.8 points [p=0.017, n=43], respectively). Disease progression or death was recorded for 44.4% (n=20/45) patients during follow-up; median progression-free survival was 25.1 months and the cumulative survival rate at 3 years was 71%. No new safety signals were detected. CONCLUSIONS: The OBLIQUE study demonstrates that HRQoL is maintained in patients with PanNENs during treatment with everolimus in a UK real-world setting. This study adds to the limited HRQoL data available in this patient group.
Baudin E, Hayes A, Scoazec J-Y, et al., 2019, Unmet medical needs in pulmonary neuroendocrine (carcinoid) neoplasms, Neuroendocrinology, Vol: 108, Pages: 7-17, ISSN: 0028-3835
Pulmonary carcinoids (PCs) display the common features of all well-differentiated neuroendocrine neoplasms (NEN) and are classified as low- and intermediate-grade malignant tumours (i.e. typical (TC) and atypical carcinoid (AC), respectively). There is a paucity of randomised studies dedicated to advanced PCs and management principles are drawn from the larger gastroenteropancreatic (GEP) NEN experience. There is growing evidence that NEN anatomic subgroups have different biology and different responses to treatment and, therefore, should be investigated as separate entities in clinical trials. In this review, we discuss the existing evidence and limitations of tumour classification, diagnostics and staging, prognostication and treatment in the setting of PC with focus on unmet medical needs and directions for the future.
Capdevila J, Bodei L, Davies P, et al., 2019, Unmet medical needs in metastatic lung and digestive neuroendocrine neoplasms, Neuroendocrinology, Vol: 108, Pages: 18-25, ISSN: 0028-3835
Unmet medical needs are not infrequent in oncology, and these needs are usually of higher magnitude in rare cancers. The field of neuroendocrine neoplasms (NENs) has evolved rapidly during the last decade, and, currently, a new WHO classification is being implemented and several treatment options are available in the metastatic setting after the results of prospective phase III clinical trials. However, several questions are still unanswered, and decisions in our daily clinical practice should be made with limited evidence. In the 2016 meeting of the advisory board of the European Neuroendocrine Tumor Society (ENETS), the main unmet medical needs in the metastatic NENs setting were deeply discussed, and several proposals to try to solve them are presented in this article, including biomarkers, imaging, and therapy.
Ramage JK, Valle JW, van Dijkum EJMN, et al., 2019, Colorectal Neuroendocrine Neoplasms: Areas of Unmet Need, NEUROENDOCRINOLOGY, Vol: 108, Pages: 45-53, ISSN: 0028-3835
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- Citations: 14
Frilling A, Clift AK, 2018, Surgical Approaches to the Management of Neuroendocrine Liver Metastases, ENDOCRINOLOGY AND METABOLISM CLINICS OF NORTH AMERICA, Vol: 47, Pages: 627-+, ISSN: 0889-8529
Alvarez MJ, Subramaniam PS, Tang LH, et al., 2018, A precision oncology approach to the pharmacological targeting of mechanistic dependencies in neuroendocrine tumors., Nature Genetics, Vol: 50, Pages: 979-989, ISSN: 1061-4036
We introduce and validate a new precision oncology framework for the systematic prioritization of drugs targeting mechanistic tumor dependencies in individual patients. Compounds are prioritized on the basis of their ability to invert the concerted activity of master regulator proteins that mechanistically regulate tumor cell state, as assessed from systematic drug perturbation assays. We validated the approach on a cohort of 212 gastroenteropancreatic neuroendocrine tumors (GEP-NETs), a rare malignancy originating in the pancreas and gastrointestinal tract. The analysis identified several master regulator proteins, including key regulators of neuroendocrine lineage progenitor state and immunoevasion, whose role as critical tumor dependencies was experimentally confirmed. Transcriptome analysis of GEP-NET-derived cells, perturbed with a library of 107 compounds, identified the HDAC class I inhibitor entinostat as a potent inhibitor of master regulator activity for 42% of metastatic GEP-NET patients, abrogating tumor growth in vivo. This approach may thus complement current efforts in precision oncology.
Clift A, Frilling A, 2018, Liver transplantation and multivisceral transplantation in the management of patients with advanced neuroendocrine tumours, World Journal of Gastroenterology, Vol: 24, Pages: 2152-2162, ISSN: 1007-9327
Orthotopic liver transplantation (OLT) represents a generally accepted albeit somewhat controversially discussed therapeutic strategy in highly selected patients with non-resectable hepatic metastases from neuroendocrine tumours (NET). Whilst there are some exclusion criteria, these are not universally followed, and the optimal set of inclusion parameters for deeming patients eligible has not yet been elucidated. This is due to heterogeneity in the study populations, as well differing approaches employed and also divergences in selection criteria between centres. Recent data have suggested that OLT may represent the most efficacious approach in terms of overall and disease-free survival to the management of NET metastatic to the liver when conducted in accordance with the modified Milan criteria. Therefore, a consensus set of selection criteria requires definition to facilitate stringent and fair allocation of deceased-donor organs, as well as consideration for living-donor organs. In the context of classically non-resectable metastatic tumour bulk, multivisceral transplantation with or without the liver may also be indicated, yet experience is very limited. In this review, we discuss the diagnostic work-up of patients in whom the aforementioned transplantation approaches are being considered, critically analyse the published experience and also anticipate future developments in this field, including a discussion of immediate and longer-term research priorities.
Dubash SR, Barwick T, Mauri FA, et al., 2018, [<SUP>18</SUP>F]FET-βAG-TOCA versus [<SUP>68</SUP>Ga]DOTATATE PET/CT in functional imaging of neuroendocrine tumours., Publisher: AMER SOC CLINICAL ONCOLOGY, ISSN: 0732-183X
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- Citations: 3
Carter P, Alifrangis C, Cereser B, et al., 2018, Correction: Assessing tumor molecular profiling to guide treatments for patients with advanced female genital tract malignancy, Oncotarget, Vol: 9, Pages: 15164-15164, ISSN: 1949-2553
Correction to article DOI: https://dx.doi.org/10.18632/oncotarget.23675
Iacovazzo D, Flanagan SE, Walker E, et al., 2018, MAFA missense mutation causes familial insulinomatosis and diabetes mellitus., Proceedings of the National Academy of Sciences, Vol: 1151, Pages: 1027-1032, ISSN: 0027-8424
The β-cell-enriched MAFA transcription factor plays a central role in regulating glucose-stimulated insulin secretion while also demonstrating oncogenic transformation potential in vitro. No disease-causing MAFA variants have been previously described. We investigated a large pedigree with autosomal dominant inheritance of diabetes mellitus or insulinomatosis, an adult-onset condition of recurrent hyperinsulinemic hypoglycemia caused by multiple insulin-secreting neuroendocrine tumors of the pancreas. Using exome sequencing, we identified a missense MAFA mutation (p.Ser64Phe, c.191C>T) segregating with both phenotypes of insulinomatosis and diabetes. This mutation was also found in a second unrelated family with the same clinical phenotype, while no germline or somatic MAFA mutations were identified in nine patients with sporadic insulinomatosis. In the two families, insulinomatosis presented more frequently in females (eight females/two males) and diabetes more often in males (12 males/four females). Four patients from the index family, including two homozygotes, had a history of congenital cataract and/or glaucoma. The p.Ser64Phe mutation was found to impair phosphorylation within the transactivation domain of MAFA and profoundly increased MAFA protein stability under both high and low glucose concentrations in β-cell lines. In addition, the transactivation potential of p.Ser64Phe MAFA in β-cell lines was enhanced compared with wild-type MAFA. In summary, the p.Ser64Phe missense MAFA mutation leads to familial insulinomatosis or diabetes by impacting MAFA protein stability and transactivation ability. The human phenotypes associated with the p.Ser64Phe MAFA missense mutation reflect both the oncogenic capacity of MAFA and its key role in islet β-cell activity.
Clift AK, Kornasiewicz O, Drymousis P, et al., 2018, Goblet cell carcinomas of the appendix: rare but aggressive neoplasms with challenging management., Endocrine Connections, Vol: 7, Pages: 268-277, ISSN: 2049-3614
Goblet cell carcinomas (GCC) are a rare, aggressive sub-type of appendiceal tumours with neuroendocrine features, and controversy exists with regards to therapeutic strategy. We undertook a retrospective review of GCC patients surgically treated at two tertiary referral centres. Clinical and histopathological data were extracted from a prospectively maintained database. Survival analyses utilised Kaplan-Meier methodology. Twenty-one patients were identified (9 females). Median age at diagnosis was 55years (range 32-77). There were 3, 6 and 9 grade 1, 2 and 3 tumours, respectively. One, 10, 5 and 5 patients had stage I, II, III and IV disease at diagnosis, respectively. There were 8, 10 and 3 Tang class A, B and C tumours, respectively. Index operation was appendectomy (n=12), right hemicolectomy (n=6) or resections including appendix/right colon, omentum and the gynaecological system (n=3). Eight patients underwent completion right hemicolectomy. Surgery for recurrence included small bowel resection (n=2), debulking with peritonectomy and heated intraperitoneal chemotherapy, and hysterectomy and bilateral salpingo-oophorectomy (all n=1). Median follow-up was 30months (range 2.5-123). One-, 3- and 5-year OS was 79.4%, 60% and 60%, respectively. Mean OS (1-, 3-, and 5-year OS) for Tang class A, B and C tumours were 73.1months (85.7%, 85.7%, 51.4%), 83.7months (all 66.7%) and 28.5months (66.7%, 66.7%, not reached), respectively. Chromogranin A/B and 68Ga-DOTATATE PET/CT were not useful in follow-up, but CEA, CA 19-9, CA 125 and 18F-FDG PET/CT identified tumour recurrence. GCC must be clearly discriminated from relatively indolent appendiceal neuroendocrine neoplasms. 18F-FDG PET/CT and CEA/CA19-9/CA-125 are useful in detecting recurrence of GCC.
Braat A, Kappadath C, Ahmadzadehfar H, et al., 2018, International Multicentre Retrospective Study on the Safety of Radioembolization with Yttrium-90 Resin Microspheres after Systemic Radionuclide Therapy in Neuroendocrine Tumors, 15th Annual ENETS Conference for the Diagnosis and Treatment of Neuroendocrine Tumor Disease, Publisher: KARGER, Pages: 242-242, ISSN: 0028-3835
Clift AK, Pawa N, Osmani H, et al., 2018, Appropriate Surgical Strategy in Appendiceal Neuroendocrine Tumors: Is Right Hemicolectomy Oncologically Justified or Overtreatment?, 15th Annual ENETS Conference for the Diagnosis and Treatment of Neuroendocrine Tumor Disease, Publisher: KARGER, Pages: 269-269, ISSN: 0028-3835
Skovgaard D, Fazio N, Granberg D, et al., 2018, Peptide Receptor Radionuclide Therapy (PRRT) in Gastroenteropancreatic Grade 3 Neuroendocrine Neoplasms: A Retrospective International Multicenter Study, 15th Annual ENETS Conference for the Diagnosis and Treatment of Neuroendocrine Tumor Disease, Publisher: KARGER, Pages: 258-258, ISSN: 0028-3835
Carter P, Alifrangis C, Cereser B, et al., 2017, Assessing tumor molecular profiling to guide treatments for patients with advanced female genital tract malignancy, Oncotarget, Vol: 9, Pages: 6007-6014, ISSN: 1949-2553
Tumor molecular profiling has enabled selection of targeted therapies in a host of solid tumors. Here we used a retrospective clinical cohort, to evaluate the benefit of tailoring treatments for female genital tract malignancy, using tumor molecular profiles. Clinical outcome data for 112 patients was retrospectively separated into two groups. These either followed a matched treatment plan that incorporated at least one drug recommended according to their tumor profile and none that were expected to have no benefit (64 patients), or was unmatched with suggested treatments and received at least one drug that was anticipated to lack benefit for that tumor (48 patients).In the group of patients whose drugs matched those recommended by molecular profiling of their tumor, their overall survival was 593 days on average, compared to 449 days for patients that did not; removing drugs predicted to have no benefit from treatment regimens received after profiling increased survival by 144 days on average (P = 0.0265). In the matched treatment group, 30% of patients had died by the last time of monitoring, whereas this was 40% in the unmatched group (P = 0.2778). The IHC biomarker for the progesterone receptor was demonstrated to be prognostic for survival.
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