Journal of Vascular and Interventional Radiology
Volume 17, Issue 8,
, Pages 1235-1250
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Neuroendocrine tumors (NETs) are rare and represent a diverse collection of malignancies that occur in many organ systems throughout the body, including the gastrointestinal and respiratory tracts. Unfortunately, the majority of patients with NETs have hepatic metastases at the time of diagnosis. Although some patients may be asymptomatic, others have unusual clinical presentations and variable tumor growth patterns. Although many patients have long indolent courses, without treatment, most patients die within 5 years of diagnosis. This article reviews the care of patients with NETs and hepatic metastases, with emphasis on the increasingly important role of oncologic image-guided interventions.
TYPES OF NETs, EPIDEMIOLOGY, AND PATHOLOGY
NETs consist of a group of carcinomas with variable clinical courses that arise from neuroendocrine cells dispersed throughout the body. NETs are often indolent but frequently present with incurable metastatic disease. The most common NETs, carcinoid tumors, usually arise from the small bowel, lung, and bronchi, occurring less often in the appendix and very rarely in the pancreas (7). Carcinoid tumors can secrete serotonin as well as other bioactive amines that cause the characteristic
Low-grade NETs frequently present with an unknown primary site. The goals of diagnostic testing are to identify the location of the primary tumor and to establish the extent of disease. In 2005, a panel of experts published comprehensive guidelines and recommendations for the detailed workup of patients with gastroenteropancreatic NETs to aid clinicians in this purpose (12). In addition to a routine history and physical examination, blood and urine laboratory studies (eg, thyroid function
MEDICAL MANAGEMENT OF HORMONAL SYNDROMES
Low-grade NETs are capable of making a variety of bioactive amines that can cause hormonal syndromes. Importantly, secretion from many NETs is inhibited by natural somatostatin. Before the introduction of somatostatin analogues (eg, octreotide) in the United States, refractory carcinoid syndrome was a frequent cause of morbidity and mortality. Analyses of the Surveillance, Epidemiology, and End Results 9 database show an improvement in overall survival (OS) among patients with metastatic
Current systemic therapies for bulky metastatic carcinoid tumors have low biologic activity, an unfavorable toxicity profile, or both. In the recently reported Eastern Cooperative Oncology Group phase III study (29) of systemic chemotherapy in carcinoid tumors (study E1281), the response rates for streptozocin plus doxorubicin or 5-fluorouracil were both 16%, and the median progression-free survival (PFS) durations were 4.5 and 5.3 months, respectively. IFN-α has also been widely studied in
Primary Tumor Resection
Little controversy exists regarding the role of surgical resection in the treatment of isolated primary NETs. Excision is safe, effective, associated with low recurrence rates, and therefore potentially curative in patients with isolated, sporadic pancreatic islet cell tumors (45). However, when multiple tumors are present, MEN-1 syndrome should be considered when the role of surgery in the treatment of patients with multiple islet cell tumors and MEN-1 syndrome is not clear. Also, resection of
PERCUTANEOUS LIVER-DIRECTED THERAPIES
As described earlier, patients with NET hepatic metastases present a difficult problem for medical and surgical oncologists alike. Unfortunately, hepatic resection is possible in fewer than 10% of patients, and systemic chemotherapy has limited effectiveness, particularly for patients with carcinoid tumors. In addition, although somatostatin analogues have been shown to be effective in controlling symptoms in many of these patients, the disease can become refractory to treatment. Also, despite
Despite increasing interest in the field of thermal ablation and application of these therapies to primary and metastatic liver tumors, only a few reports of radiofrequency (RF) ablation in patients with NET liver metastases have been published.
The largest series consisted of 34 patients with 234 tumors who were treated in 42 sessions with laparoscopic RF ablation (93). The intent of therapy was palliative in 28 patients (82%) and curative in six others. Of these 34 patients, 19 had
FOLLOW-UP AFTER TREATMENT
Given the diversity of NET types and clinical features, presently there is no consensus as to how these patients are monitored after therapy. A resource used by clinicians is the National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology updated for 2006 (109), which presents management algorithms for various types of NETs. Follow-up for patients with NET hepatic metastases is individualized on the basis of the patient's clinical status (including symptoms), laboratory and
Significant medical and surgical advances have been made in the understanding and management of NETs metastatic to the liver. However, in most instances, this type of neoplasm remains incurable. Interventional radiologists with specific expertise in liver-directed therapies currently play a critical role in the care of patients with incurable, symptomatic, and nonresectable disease. Recent studies of systemic agents that target angiogenesis (31, 44) or the epidermal growth factor receptor (110)
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Cryoablation of unresectable malignant liver tumors
Am J Surg
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Radiofrequency ablation combined with chemoembolization in hepatocellular carcinoma: treatment response based on tumor size and morphology
J Vasc Interv Radiol
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Yttrium 90 resin microspheres for the treatment of unresectable colorectal hepatic metastases after failure of multiple chemotherapy regimens: preliminary results
J Vasc Interv Radiol
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90Y microsphere (TheraSphere) treatment for unresectable colorectal cancer metastases of the liver: response to treatment at targeted doses of 135-150 Gy as measured by [18F] fluorodeoxyglucose positron emission tomography and computed tomographic imaging
J Vasc Interv Radiol
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Treatment of unresectable hepatocellular carcinoma with use of 90Y microspheres (TheraSphere): safety, tumor response, and survival
J Vasc Interv Radiol
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Surgery as primary treatment in patients with liver metastases from carcinoid tumors: a retrospective, unicentric study over 13 years
(2001)(Video) Treatment of Neuroendocrine Tumor Liver Metastases
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Hepatic arterial chemoembolization for metastatic endocrine tumors
J Vasc Interv Radiol
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Hepatic artery chemoembolization for management of patients with advanced metastatic carcinoid tumors
Am J Surg
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Effective palliative treatment of metastatic carcinoid tumors with intra-arterial chemotherapy/chemoembolization combined with octreotide acetate
Am J Surg
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Portal vein embolization in preparation for major hepatic resection: evolution of a new standard of care
J Vasc Interv Radiol
Transhepatic ipsilateral right portal vein embolization extended to segment IV: improving hypertrophy and resection outcomes with spherical particles and coils
J Vasc Interv Radiol
Isolated liver metastases from neuroendocrine tumors: does resection prolong survival?
J Am Coll Surg
Surgical treatment of neuroendocrine metastases to the liver: a plea for resection to increase survival
J Am Coll Surg
Phase II trial of dacarbazine (DTIC) in advanced pancreatic islet cell carcinoma. Study of the Eastern Cooperative Oncology Group-E6282
Prospective study of chemotherapy in patients with metastatic gastrinoma
Combination therapy with octreotide and alpha-interferon: effect on tumor growth in metastatic endocrine gastroenteropancreatic tumors
Am J Gastroenterol
Octreotide and interferon alfa: a new combination for the treatment of malignant carcinoid tumours
Eur J Cancer
Hepatic neuroendocrine metastases: does intervention alter outcomes?
J Am Coll Surg
Karnofsky Memorial Lecture: an odyssey in the land of small tumors
J Clin Oncol
Prognosis and survival in patients with gastrointestinal tract carcinoid tumors
Malignant carcinoid tumors
An analysis of 8305 cases of carcinoid tumors
N Engl J Med
Neuroendocrine tumors of the gastrointestinal tract: how aggressive should we be?
Fluorouracil, doxorubicin, and streptozocin in the treatment of patients with locally advanced and metastatic pancreatic endocrine carcinomas
J Clin Oncol
Embolization and chemoembolization therapy for neuroendocrine tumors
Curr Opin Oncol
Carcinoid: a comprehensive review
Revised classification of neuroendocrine tumors of the lung, pancreas and gut
Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours
Multidetector computed tomography and neuroendocrine pancreaticoduodenal tumors
Multi-detector row CT of pancreatic islet cell tumors
Preoperative detection of pancreatic insulinomas on multiphasic helical CT
AJR Am J Roentgenol
Dual phase spiral CT in the detection of small insulinomas of the pancreas
Br J Radiol
Islet cell tumor of the pancreas: biphasic CT versus MR imaging in tumor detection
Helical CT for the preoperative localization of islet cell tumors of the pancreas: value of arterial and parenchymal phase images
AJR Am J Roentgenol
Detection of liver metastases from endocrine tumors: a prospective comparison of somatostatin receptor scintigraphy, computed tomography, and magnetic resonance imaging
J Clin Oncol
Radiotherapy with a radiolabeled somatostatin analogue, [111InDTPA-D-Phe1]-octreotide
Ann N Y Acad Sci
Somatostatin analogue octreotide and inhibition of tumour growth in metastatic endocrine gastroenteropancreatic tumours
Octreotide as an antineoplastic agent in the treatment of functional and nonfunctional neuroendocrine tumors
Inhibition of vascular endothelial growth factor by octreotide in colorectal cancer patients
Somatostatin analogue SMS 201-995 reduces serum IGF-I levels in patients with neoplasms potentially dependent on IGF-I
Octreotide acetate long-acting formulation versus open-label subcutaneous octreotide acetate in malignant carcinoid syndrome
J Clin Oncol
Long-term management of the carcinoid syndrome: treatment with octreotide alone and in combination with alpha-interferon
Phase II/III study of doxorubicin with fluorouracil compared with streptozocin with fluorouracil or dacarbazine in the treatment of advanced carcinoid tumors: Eastern Cooperative Oncology Group Study E1281
J Clin Oncol
Randomized clinical trial of the effect of interferon alpha on survival in patients with disseminated midgut carcinoid tumours
Br J Surg
Improved progression free survival (PFS), and rapid, sustained decrease in tumor perfusion among patients with advanced carcinoid treated with bevacizumab [abstract]
J Clin Oncol
A 5-decade analysis of 13,715 carcinoid tumors
Combination chemotherapy for islet cell carcinoma and metastatic carcinoid tumors with 5-fluorouracil and streptozotocin
Cancer Treat Rep
Streptozocin alone compared with streptozocin plus fluorouracil in the treatment of advanced islet-cell carcinoma
N Engl J Med
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Hypovascular pancreatic neuroendocrine tumor with hepatic metastases: A case report and literature review
2021, Radiology Case Reports(Video) Neuroendocrine liver metastases - the power of DWI and PRRT - Dr. Aarti Sekhar
Hypovascular pancreatic neuroendocrine tumors are uncommon pancreatic tumors and commonly misdiagnosed as pancreatic ductal adenocarcinoma or chronic mass-forming pancreatitis. The liver is the organ most commonly affected by neuroendocrine tumor metastases but hepatic neuroendocrine tumor metastases are quite difficult to discriminate from other hepatic metastases and primary hepatic tumors. We describe a case of a 47-year-old man with incidentally detected multiple hepatic lesions on ultrasound. On further imaging technique including computed tomography and magnetic resonance imaging, the patient had an abnormal hypoenhancing lesion at the pancreatic tail and multiple hyperenhancing hepatic metastases that were diagnosed as hypovascular pancreatic well-differentiated neuroendocrine tumor Grade 2 with multiple hypervascular hepatic metastases after liver biopsy and surgery. Neuroendocrine tumor is a rare etiology among hypoenhancing pancreatic tumors, and must be considered to discriminate from pancreatic adenocarcinomas in cases there are multiple hyperenhancing hepatic metastases on the arterial phase without typical washout on the portal venous phase.
A rational approach to postoperative surveillance for resected non-functional pancreatic neuro-endocrine tumours
Non-functional pancreatic neuroendocrine tumours (NF-PNETs) are rare and have highly variable outcomes. Current guidelines recommend surveillance for NF-PNETs <2 cm. Patients who ultimately have surgical resection are at risk of disease recurrence, and data to support postoperative surveillance protocols are lacking. The aims of this study were to i) identify post-operative predictors of recurrence and ii) risk stratify patients at risk of recurrence.
Consecutive patients who underwent surgery for NF-PNETs between 2002 and 2015 were identified retrospectively. Data were collected on demographics, pre-operative laboratory results and histopathological tumour characteristics. Statistical analyses were based on penalised Cox-regression modelling and a decision-tree model. Comparison of the variables identified was performed using ROC curves to identify the most sensitive and specific variable associated with disease recurrence.
We identified 73 patients (38 males) with a median age of 61.5 years (range: 31–79). The median period of follow-up was 49 months (5–131). During follow up, 10 deaths (13.9%) were recorded and disease recurrence occurred in 12 patients (16.4%). The Kaplan-Meier predicted 1-,3- and 5-year recurrence–free survival rates were 98.6% (95% CI = 95.9, 100%), 85.4% (76.9–94.8%) and 72% (58.7–88.2%) respectively. Cox multivariate analysis identified poor tumour differentiation (WHO G3 grade) and lymph node ratio (LNR) as independent predictors for recurrence (p < 0.05). A simple criterion of ‘tumour grade G3 or LNR ≥0.1’ was found to be sensitive and specific in detecting disease recurrence.
Our results have identified a simple and sensitive criterion for risk stratifying post-resection surveillance. Prospective validation in larger patient cohort is now warranted.
Yttrium-90 radioembolization for unresectable metastatic neuroendocrine liver tumor: A systematic review
2018, European Journal of Radiology
Citation Excerpt :
TABE and TACE differ from each other in that TACE uses chemotherapeutic agents along with the conventional embolizing particles. There is still controversy regarding whether the addition of these cytotoxic drugs increases the effectiveness of bland embolization; in general, TABE demonstrates disease control and survival rates similar to those seen with TACE [33–35]. Previous studies of TABE/TACE for the treatment of unresectable liver metastases of NETs have demonstrated a median disease control rate of 50% (range, 11.1%–100%) and a median survival of 36 months (range, 9.3–80 months) [8,36,37].
To evaluate the value of yttrium-90 (90Y) microspheres in the management of unresectable liver metastases secondary to neuroendocrine tumors (NETs).
PubMed, EMBASE, the Cochrane Database of Systematic Reviews, and the “gray” literature (Google Scholar) were searched for all studies related to 90Y therapy for unresectable liver metastases of NETs.
A total of 11 studies and 7 abstracts involving 870 patients were included in the final analysis. In 11 of these studies, 19.8% (77/388) of patients had undergone transarterial bland embolization (TABE) or transarterial chemoembolization (TACE) before 90Y therapy. The median disease control rate among all patients was 86% at 3 months after 90Y therapy. The median survival was 28 months, with 1-, 2-, and 3-year survival rates of 72.5%, 57%, and 45%, respectively. The median survival values for patients who received resin- and glass-based 90Y treatment were 27.6 and 31.7 months, respectively. The survival values for patients with carcinoid, pancreatic, and unclassified origin of NETs were 56, 31, and 28 months, respectively; the survival values for patients with grade I, II, and III NETs were 71, 56, and 28 months, respectively. Carcinoid syndrome was reported in 52.4% (55/105) of patients, and 69.1% of those with clinical symptoms demonstrated improvement in symptoms after 90Y radioembolization. Complications were reported in 9 studies, including radiation gastritis (n = 4), duodenal ulcer (n = 2), death due to liver failure (n = 1), and radiation cholecystitis (n = 1). The most common side effects were abdominal pain (median, 32.6%), nausea/vomiting (median, 32.5%), and fatigue (median, 30.4%).
90Y radioembolization can be used as an alternative therapy for unresectable liver metastases of NETs, with an improved survival rate and tumor response. This treatment is also effective for patients who have undergone unsuccessful TABE/TACE therapy and for the relief of symptoms in patients with carcinoid syndrome.
Tumor Dose Response in Yttrium-90 Resin Microsphere Embolization for Neuroendocrine Liver Metastases: A Tumor-Specific Analysis with Dose Estimation Using SPECT-CT
2017, Journal of Vascular and Interventional Radiology
To evaluate dose-response relationship in yttrium-90 (90Y) resin microsphere radioembolization for neuroendocrine tumor(NET) liver metastases using a tumor-specific dose estimation based on technetium-99m–labeled macroaggregated albumin (99mTc MAA) single photon emission computed tomography (SPECT)-CT.
Fifty-five tumors (mean size 3.9 cm) in 15 patients (10 women; mean age 57 y) were evaluated. Tumor-specific absorbed dose was estimated using a partition model. Initial (median 2.3 months) follow-up data were available for all tumors; last (median 7.6 months) follow-up data were available for 45 tumors. Tumor response was evaluated using Modified Response Evaluation Criteria in Solid Tumors (mRECIST) on follow-up CT. Tumors with complete or partial response were considered responders. Mean tumor absorbed dose was 231.4 Gy ± 184.3, and mean nontumor liver absorbed dose was 39.0 Gy ± 18.0.
Thirty-six (65.5%) and 30 (66.7%) tumors showed response at initial and last follow-up, respectively. Mean absorbed doses in responders and nonresponders at initial and last follow-up were 285.8 Gy ± 191.1 and 128.1 Gy ± 117.1 (P= .0004) and 314.3 Gy ± 195.8 and 115.7 Gy ± 117.4 (P= .0001). Cutoff value of ≥ 191.3 Gy for tumor-specific absorbed dose predicted tumor response with 93% specificity, whereas < 72.8 Gy predicted nonresponse with 100% specificity at last follow-up. Estimated mean absorbed tumor dose per patient was significantly higher in responders versus nonresponders over the follow-up period (224.5 Gy ± 90.3 vs 70.0 Gy ± 28.0; P= .007).
Tumor-specific absorbed dose, estimated with a partition model, was significantly associated with tumor response in NET liver metastases. An estimated dose ≥ 191.3 Gy predicted treatment response with high sensitivity and specificity.
ACR Appropriateness Criteria Radiologic Management of Hepatic Malignancy
2016, Journal of the American College of Radiology
Management of primary and secondary hepatic malignancy is a complex problem. Achieving optimal care for this challenging population often requires the involvement of multiple medical and surgical disciplines. Because of the wide variety of potential therapies, treatment protocols for various malignancies continue to evolve. Consequently, development of appropriate therapeutic algorithms necessitates consideration of medical options, such as systemic chemotherapy; surgical options, such as resection or transplantation; and loco-regional therapies, such as thermal ablation and transarterial embolization techniques. This article provides a review of treatment strategies for the three most common subtypes of hepatic malignancy treated with loco-regional therapies: hepatocellular carcinoma, neuroendocrine metastases, and colorectal metastases. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed every three years by a multidisciplinary expert panel. The guideline development and review include an extensive analysis of current medical literature from peer reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances where evidence is lacking or not definitive, expert opinion may be used to recommend imaging or treatment.
Radiofrequency ablation for neuroendocrine liver metastases: A systematic review
2015, Journal of Vascular and Interventional Radiology
To determine the efficacy of radiofrequency (RF) ablation in neuroendocrine tumor (NET) liver metastases. A systematic review was performed following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Eight studies were included (N = 301). Twenty-six percent of RF ablation procedures were percutaneous (n = 156), with the remainder conducted at surgery. Forty-eight percent of patients had a concomitant liver resection. Fifty-four percent of patients presented with symptoms, with 92% reporting symptom improvement following RF ablation (alone or in combination with surgery). The median duration of symptom improvement was 14–27 months. However, recurrence was common (63%–87%). RF ablation can provide symptomatic relief in NET liver metastases alone or in combination with surgery.
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Long-term analysis of 2 prospective studies that incorporate mitomycin C into an adjuvant chemoradiation regimen for pancreatic and periampullary cancers
Advances in Radiation Oncology, Volume 3, Issue 1, 2018, pp. 42-51
The purpose of this study was to report toxicity and long-term survival outcomes of 2 prospective trials evaluating mitomycin C (MMC) with 5-fluorouracil–based adjuvant chemoradiation in resected periampullary adenocarcinoma.
From 1996 to 2002, 119 patients received an adjuvant 4-drug chemotherapy regimen of 5-fluorouracil, leucovorin, MMC, and dipyridamole with chemoradiation on 2 consecutive trials (trials A and B). Trial A patients received upfront chemoradiation (50 Gy split-course, 2.5 Gy/fraction) followed by 4 cycles of the 4-drug chemotherapy with bolus 5-fluorouracil. Trial B patients received 1 cycle of the 4-drug chemotherapy with continuous infusion 5-fluorouracil followed by continuous chemoradiation (45-54 Gy, 1.8 Gy/fraction) and 2 additional cycles of chemotherapy. Cox proportional hazards models were performed to identify prognostic factors for overall survival (OS).
Of the 62 trial A patients, 61% had pancreatic and 39% nonpancreatic periampullary carcinomas. Trial B (n = 57) consisted of 68% pancreatic and 32% nonpancreatic periampullary carcinomas. Resection margin and lymph node status were similar for both trials. Median follow-up was longer for trial A than trial B (197.5 vs 107.0 months), with median OS of 32.2 and 24.2 months, respectively. Rates of 3-, 5-, and 10-year OS were 48%, 31%, and 26% in trial A and 32%, 23%, and 9% in trial B. On multivariate analysis, lymph node–positive resection was the strongest prognostic factor for OS. A pancreatic primary and positive margin status were also associated with inferior survival (P < .05). Rates of grade ≥3 treatment-related toxicity in trials A and B were 2% and 7%, respectively.
This is the first study to report long-term outcomes of MMC with 5-fluorouracil–based adjuvant chemoradiation in periampullary cancers. Because MMC may be considered in DNA repair-deficient carcinomas, randomized trials are needed to determine the true benefit of adjuvant MMC.
Treatment of acute pouchitis
Seminars in Colon and Rectal Surgery, Volume 28, Issue 3, 2017, pp. 132-137
Acute pouchitis is a common disease that affects many patients with an ileal pouch-anal anastomosis. The management of acute pouchitis remains largely empiric with the mainstay of therapy being the antibiotics ciprofloxacin and metronidazole. Probiotics may have a role in the primary and secondary prophylaxis of acute pouchitis. In addition, there are modest data that probiotics are effective in the treatment of mild acute pouchitis. There are limited data for other treatments of acute pouchitis including oral and rectal budesonide and glutamine suppositories. Diet plays a largely undefined role in the development and management of acute pouchitis, and, although many have been studied, no specific diet can currently be recommended. Larger well-designed clinical trials are necessary to confirm the efficacy of current treatments and to investigate new treatments for the management of acute pouchitis.
Robotic Liver Resection
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Comparison of no-touch multi-bipolar vs. monopolar radiofrequency ablation for small HCC
Journal of Hepatology, Volume 66, Issue 1, 2017, pp. 67-74
The primary aim of this study was to compare the rate of global radiofrequency ablation (RFA) failure between monopolar RFA (MonoRFA) vs. no-touch multi-bipolar RFA (NTmbpRFA) for small hepatocellular carcinoma (HCC) ⩽5cm in cirrhotic patients.
A total of 362 cirrhotic patients were included retrospectively across four French centres (181 per treatment group). Global RFA failure (primary RFA failure or local tumour progression) was analysed using the Kaplan-Meier method after coarsened exact matching. Cox regression models were used to identify factors associated with global RFA failure and overall survival (OS).
Patients were well matched according to tumour size (⩽30/>30mm); tumour number (one/several); tumour location (subcapsular and near large vessel); serum AFP (<10; 10–100; >100ng/ml); Child-Pugh score (A/B) and platelet count (</⩾100G/L), p=1 for all. One case of perioperative mortality was observed in the NTmbpRFA group and the rate of major complications was 7.2% in both groups (p=1). The cumulative rates of global RFA failure at 1, 3 and 5years were respectively 13.3%, 31% and 36.7% for MonoRFA vs. 0.02%, 7.9% and 9.2% for NTmbpRFA, p<0.001. Monopolar RFA, tumour size >30mm and HCC near large vessel were independent factors associated with global RFA failure. Five-year OS was 37.2% following MonoRFA vs. 46.4% following NTmbpRFA p=0.378.
This large multicentre case-matched study showed that NTmbpRFA provided better primary RFA success and sustained local tumour response without increasing severe complications rates, for HCC ⩽5cm.
Using no-touch multi-bipolar radiofrequency ablation for hepatocellular carcinoma ⩽5cm provide a better sustained local tumour control compared to monopolar radiofrequency ablation.
R.M. is a paid proctor for Sirtex Medical and J.C.Y has financial relationships with Novartis and Genentech. None of the other authors have identified a conflict of interest.(Video) Updates in the management of neuroendocrine cancers
Copyright © 2006 Society of Interventional Radiology. Published by Elsevier Inc. All rights reserved.
What is the prognosis for neuroendocrine tumor with liver metastases? ›
Even more so, liver metastases are often referred to as the “secondary liver cancer.” These patients have a significantly worse prognosis and a “5-year survival.” Without treatment, such as with Kathleen's original prognosis, “patients who develop liver metastases live an average of 8.7 months.”Who is the longest survivor of neuroendocrine cancer? ›
Marilyn Thurston of Colorado may be the longest-surviving carcinoid patient in the United States and possibly the world, having been diagnosed over 30 years ago. Her physician, Dr. Nicholas DiBella, created a special pin recognizing her tenacity.Where is the best place for treatment of neuroendocrine cancer? ›
Mayo Clinic doctors have extensive experience diagnosing and treating neuroendocrine tumors. Each year, Mayo Clinic doctors care for more than 1,900 people with neuroendocrine tumors, including people with very rare types. Nationally recognized expertise.How do you treat neuroendocrine cancer in the liver? ›
If neuroendocrine tumours (NETs) spread, they often spread to the liver (called liver metastases). Liver directed therapy directly targets the cancer in the liver and is often used to treat NETs that have spread to the liver. Liver directed therapy is mainly used when surgery can't be done.What is the 5-year survival for neuroendocrine tumors? ›
If the tumor has spread to nearby tissue or the regional lymph nodes, the 5-year relative survival rate is 96%. If the tumor has spread to distant areas of the body, the relative 5-year survival rate is 68%.Can you live a long life with a neuroendocrine tumor? ›
It depends on your individual condition, type of neuroendocrine tumour (NET), treatment and level of fitness. So no one can tell you exactly how long you will live. These are general statistics based on large groups of people. Remember, they can't tell you what will happen in your individual case.Can neuroendocrine cancer go into remission? ›
A remission is when a NET cannot be detected in the body and there are no symptoms. This may also be called having “no evidence of disease” or NED. A remission may be temporary or permanent. This uncertainty causes many people to worry that the tumor will come back.What celebrity has neuroendocrine cancer? ›
A few high profile celebrities, Aretha Franklin and Steve Jobs, have died from pancreatic neuroendocrine tumors. Following their deaths, their cancer was often called “pancreatic cancer” because it was cancer and it occurred in the pancreas.Which actor died of neuroendocrine tumor? ›
How Irrfan Khan died of a neuroendocrine tumour what is it symptoms and treatment.What foods should be avoided with neuroendocrine tumors? ›
- aged cheese.
- alcohol and fermented drinks such as beer.
- smoked and salted fish and meats such as sausages and corned beef.
- soybean products such as tofu and soy sauce.
What is the new treatment for neuroendocrine cancer? ›
The new drug consists of a radioactive isotope, Lu-177, attached to dotatate—a molecule that binds to GEP-NET cells that have a molecule called a somatostatin receptor on their surface. The drug then enters these somatostatin receptor−positive tumor cells, and radiation emitted by Lu-177 helps kill the cells.What is the best chemo for neuroendocrine cancer? ›
CapTem Chemo and Other Neuroendocrine Tumor Chemotherapy
It typically works most effectively on more aggressive tumors, those with poorly differentiated cells. But a newer combination, capecitabine and temozolomide (CapTem), can provide some control of well-differentiated pancreatic tumors.
The exact cause of neuroendocrine tumors isn't known. These cancers begin in neuroendocrine cells that have traits similar to those of nerve cells and hormone-producing cells. Neuroendocrine cells are found throughout your body.Does immunotherapy work for neuroendocrine cancer? ›
The most significant finding was that a subset of patients with neuroendocrine tumors, those with high-grade carcinoma, seem to benefit most from dual immunotherapy, with a response rate approaching 44%. Many of those responses, at least initially, appear to be long-lasting and continuous.Is Stage 4 neuroendocrine cancer curable? ›
Many neuroendocrine tumors have spread at the time of diagnosis or recurred after surgery. Most metastatic tumors (that have spread) are not curable but can be treatable with multiple forms of therapy.What is the average age for neuroendocrine? ›
The average age of diagnosis for a pancreas NET is 60. However, when these types of tumors are the result of a genetic syndrome (see Risk Factors), the age of diagnosis is generally earlier. Men are slightly more likely to develop a pancreas NET than women.Does chemo shrink neuroendocrine tumors? ›
Chemotherapy uses anti cancer (cytotoxic) drugs to destroy neuroendocrine tumour (NET) cells. The drugs circulate throughout your body in the bloodstream. Chemotherapy isn't usually the first choice of treatment for NETs.How aggressive are neuroendocrine tumors? ›
Large cell neuroendocrine tumours tend to be aggressive tumours that grow quickly. They are more likely to spread to other parts of the body. Small cell lung neuroendocrine carcinomas, or small cell lung cancers, are also poorly differentiated cancerous tumours.Do neuroendocrine tumors go to the brain? ›
In rare instances, NENs can metastasize to the CNS. The incidence of patients with NENs having brain metastases is <5%. Furthermore, only 1.4% of metastatic brain tumours are NENs, and the majority of these lesions originate from primaries in the lung.What is a Stage 4 neuroendocrine tumor? ›
Stage IV. Stage IV means the lung NET has spread to more than 1 area in the opposite lung, the fluid surrounding the lung or the heart, or distant parts of the body through the bloodstream. Once cancer cells get into the blood, the cancer can spread anywhere in the body.
What is a Stage 3 neuroendocrine tumor? ›
Stage III: The tumor is any size, and the cancer has spread to regional lymph nodes (any T, N1 or N2, M0). Or, the tumor has spread to the peritoneum or to other organs or structures, but the cancer has not spread to the lymph nodes or elsewhere (T4, N0, M0).What is the follow up for a neuroendocrine tumor? ›
How often you have appointments. You are likely to have a follow up appointment every 6 to 12 months if you have had surgery to fully remove your NET. If your tumour was grade 3 and you have had surgery to fully remove the tumour, you are seen every 3 months.Who is most likely to get neuroendocrine cancer? ›
- Age. Pheochromocytoma is most common between 40 and 60 years of age, and Merkel's cell cancer commonly affects people over the age of 70.
- Gender. ...
- Race/ethnicity. ...
- Family history. ...
- Immune suppression. ...
- Sun exposure.
Many neuroendocrine tumors can be successfully treated with surgery and chemotherapy, especially if the tumor is localized and has not spread to the lymph nodes or other organs in the body.What kind of neuroendocrine tumor did Steve Jobs have? ›
Jobs was diagnosed with a rare form of pancreatic cancer, called an islet cell tumor or gasteroenteropancreatic neuroendocrine tumor (GEP-NET), which is a different form of pancreatic cancer than the highly aggressive and often rapidly fatal pancreatic adenocarcinoma.What neuroendocrine tumor did Steve Jobs have? ›
Pancreatic neuroendocrine tumors (or PNETs) account for only 7% of all pancreatic cancer tumors and tend to grow slower than exocrine tumors, the most common type of pancreas tumors. Jobs survived eight years before dying of the disease on Oct. 5, 2011.Does neuroendocrine cancer run in families? ›
Most of the time, we don't know why neuroendocrine tumors form. Sometimes, though, a NET develops because of a genetic mutation (also known as a variant) inherited from a parent. Overall, these hereditary tumors represent a small share of NETs — just 10 percent.How many people get neuroendocrine tumors? ›
How many people are diagnosed with a NET? Overall, it is estimated that more than 12,000 people in the United States are diagnosed with a NET each year, and approximately 171,000 people are living with this diagnosis.Are neuroendocrine tumors staged? ›
Neuroendocrine tumors are staged according to the TNM staging system: tumor (T), node (N), metastasis (M). The World Health Organization (WHO) classifies neuroendocrine tumors according to the malignant potential of the tumor: Well-differentiated neuroendocrine tumors (grade 1 and 2)What is the name of neuroendocrine tumor? ›
Some examples of neuroendocrine tumors are carcinoid tumors, islet cell tumors, medullary thyroid cancer, pheochromocytomas, neuroendocrine carcinoma of the skin (Merkel cell cancer), small cell lung cancer, and large cell neuroendocrine carcinoma (a rare type of lung cancer).
What foods feed tumours? ›
- Processed meat. According to the World Health Organization (WHO), there is “convincing evidence” that processed meat causes cancer. ...
- Red meat. ...
- Alcohol. ...
- Salted fish (Chinese style) ...
- Sugary drinks or non-diet soda. ...
- Fast food or processed foods. ...
- Fruit and vegetables. ...
The neuroendocrine mechanisms of chronic stress. Chronic stress produces stress hormones during the activation of the neuroendocrine system (hypothalamus-pituitary-adrenal axis) and the sympathetic nervous system, which can promote tumor development and regulate the tumor microenvironment.Does alcohol affect neuroendocrine tumors? ›
According to state-wide scientific research conducted in Utah between 1996 and 2014, smoking tobacco and alcohol use may be risk factors for neuroendocrine tumors of the small intestine, independent of family history.Does Keytruda work for neuroendocrine tumors? ›
The study drug, pembrolizumab has been FDA approved for treating a type of skin cancer called melanoma and for metastatic non-small cell lung cancer. However, it is not approved for treatment of metastatic high-grade neuroendocrine tumors. Other Names: Keytruda.Does neuroendocrine cancer always recur? ›
The chance that neuroendocrine cancer will come back (recur) is greatest within 5 years, so close follow-up is needed during this time.How do you treat Stage 4 neuroendocrine cancer? ›
Somatostatin analogs, like octreotide or lanreotide, chemotherapy, targeted therapy, and PRRT are often used to treat stage IV GI tract NETs. Surgery is sometimes used to relieve symptoms rather than eliminate the cancer. If distant metastases are not causing symptoms, then surgery may not be needed.What is the monthly shot for neuroendocrine tumor? ›
Octreotide LAR or lanreotide is standard treatment for the long-term control of symptoms and tumour growth. The drug is given once a month. It is usually taken for as long as it works. It may be taken for the rest of your life.What is the hormone treatment for neuroendocrine cancer? ›
Hormone therapy for NETs centers around somatostatin, a hormone that plays a part in controlling other hormones such as insulin. Somatostatin analogs are drugs that act like the hormone somatostatin to reduce the symptoms caused by an NET. The two somatostatin analogs are: Sandostatin® (octreotide)What is neuroendocrine cancer of the liver? ›
Primary hepatic neuroendocrine carcinoma (PHNEC) is a rare hepatic tumor that may manifest with abdominal pain or fullness, as well as diarrhea or weight loss. More than 10% of cases are asymptomatic and in rare cases a carcinoid syndrome may be observed.Where does neuroendocrine cancer spread to? ›
Neuroendocrine tumors are classified as primary or secondary tumors. A primary tumor is cancer that hasn't spread to other areas of your body. A secondary tumor is a NET that has spread, usually to your lymph nodes in your lymphatic system, your liver or your bones.
What part of the body does neuroendocrine cancer affect? ›
Types of neuroendocrine tumours
Pancreatic neuroendocrine tumours (pNETs) develop in the pancreas. Pulmonary neuroendocrine tumours develop in the lungs. Rarely, NETs can also develop in other parts of the body, including in the liver, gallbladder, bile ducts, kidneys, ovaries, or testicles.
Medullary thyroid cancer is a rapidly growing but still rare cancer that occurs in neuroendocrine cells in the thyroid gland. The thyroid gland is at the base of the throat. The cells, called C cells, help make a hormone called calcitonin that controls calcium levels in the blood.What cancers do not respond to immunotherapy? ›
Certain cancers, including pancreatic cancer, prostate cancer and glioblastoma, have been especially resistant to this approach.Which cancers are most successfully treated with immunotherapy? ›
- Mesothelioma. ...
- Multiple myeloma. ...
- Non-Hodgkin lymphoma. ...
- Prostate cancer. ...
- Skin cancer. ...
- Soft tissue sarcoma. ...
- Stomach cancer. ...
- Endometrial cancer. For some patients with advanced uterine cancer, treatment with checkpoint inhibitors may be an option:
Even more so, liver metastases are often referred to as the “secondary liver cancer.” These patients have a significantly worse prognosis and a “5-year survival.” Without treatment, such as with Kathleen's original prognosis, “patients who develop liver metastases live an average of 8.7 months.”What is the best hospital for neuroendocrine cancer? ›
Mayo Clinic doctors have extensive experience diagnosing and treating neuroendocrine tumors. Each year, Mayo Clinic doctors care for more than 1,900 people with neuroendocrine tumors, including people with very rare types. Nationally recognized expertise.What is the 10 year survival rate for neuroendocrine cancer? ›
The median survival duration was 41 months. The 1-, 3-, 5-, and 10-year overall survival rates for patients with NETs were 72.8%, 52.7%, 39.4%, and 18.1%, respectively.What is metastatic neuroendocrine tumor in liver? ›
Neuroendocrine liver metastases are usually multiple and of varying size. In most cases both liver lobes are affected, but miliary seeding throughout the liver is seen only occasionally. The carcinoid is the most common neuroendocrine tumor causing liver metastases, especially when of midgut origin.Do neuroendocrine tumors spread fast? ›
Some neuroendocrine tumors grow very slowly. Others are aggressive cancers that invade and destroy normal body tissue or spread (metastasize) to other parts of the body.How treatable is a neuroendocrine tumor? ›
Most localized NETs are successfully treated with surgery alone. The surgeon will usually remove some tissue surrounding the tumor, called a margin, in an effort to leave no traces of cancer in the body. When completely removing the tumor is not possible, "debulking surgery" is sometimes recommended.
What is metastatic high grade neuroendocrine carcinoma stage 4? ›
Stage IV means the lung NET has spread to more than 1 area in the opposite lung, the fluid surrounding the lung or the heart, or distant parts of the body through the bloodstream. Once cancer cells get into the blood, the cancer can spread anywhere in the body.How bad is neuroendocrine tumor? ›
Compared with more common malignant tumors, neuroendocrine tumors are slow-growing but can produce amino acids that cause severe symptoms. Aggressive therapy is recommended to lessen the severity of symptoms or to prevent possible harm to the liver.Should neuroendocrine tumors be removed? ›
Surgery frequently plays a central role in the management of neuroendocrine tumors (NETs). Like other tumors, the goal is to remove NETs in an attempt to provide a cure whenever possible. But even when NETs have metastasized (spread), surgery may still provide a significant benefit.Does Lanreotide shrink tumors? ›
LANREOTIDE (lan REE oh tide) is used to reduce blood levels of growth hormone in patients with a condition called acromegaly. It also works to slow or stop tumor growth in patients with neuroendocrine tumors and treat carcinoid syndrome.What are the stages of neuroendocrine tumor? ›
- Be contained in a particular area of the body (localized)
- Have spread to nearby tissues or lymph nodes (regional)
- Have spread throughout the body (metastatic)
Pancreatic neuroendocrine tumors (PNETs), a group of endocrine tumors arising in the pancreas, are among the most common neuroendocrine tumors.What is the new treatment for neuroendocrine tumors? ›
On January 29, the Food and Drug Administration (FDA) approved a new targeted treatment, lutetium Lu 177 dotatate (Lutathera®), for adult patients with advanced NETs that affect the pancreas or gastrointestinal tract, known as GEP-NETs.How do you treat a metastatic neuroendocrine tumor? ›
- Surgery. Surgery is used to remove the tumor. ...
- Chemotherapy. Chemotherapy uses strong drugs to kill tumor cells. ...
- Targeted drug therapy. ...
- Peptide receptor radionuclide therapy (PRRT). ...
- Medications to control excess hormones. ...
- Radiation therapy.
Although the overall prognosis may be poor based on cases with previous patients and older treatments, many patients with stage 4 cancer can live for years. A few factors to keep in mind: Many treatments are available to help fight cancer. The body's response to treatment may differ from that of others.Can high grade neuroendocrine carcinoma be cured? ›
Many neuroendocrine tumors have spread at the time of diagnosis or recurred after surgery. Most metastatic tumors (that have spread) are not curable but can be treatable with multiple forms of therapy.
What does stage 4 metastatic mean? ›
Cancer that spreads from where it started to a distant part of the body is called metastatic cancer. For many types of cancer, it is also called stage IV (4) cancer. The process by which cancer cells spread to other parts of the body is called metastasis.