Neuroendocrine Tumors Guidelines 

Neuroendocrine Tumors Guidelines 

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Grading schemes for neuroendocrine tumors (NETs) use mitotic count; the level of the nuclear protein Ki-67, which is associated with cellular proliferation; and assessment of necrosis. The World Health Organization (WHO) and the European Neuroendocrine Tumor Society (ENETS)  both incorporate mitotic count and Ki-67 proliferation for the classification of gastroenteropancreatic NETs (GEP-NETs). [1, 2, 3]

Tumors fall into one of the following three grades:

G1: Well differentiated, low grade

G2: Well differentiated, intermediate grade

G3: Poorly differentiated, high grade

However, for NETs of the lungs and thymus, the WHO includes only mitotic count and assessment of necrosis. [4] In its 2015 consensus statement on best practices for pulmonary neuroendocrine tumors, the ENETS noted that tumor grading based on a combination of KI-67, mitotic rate, and necrosis may be of clinical importance but lacks validation. [5]

Under the WHO grading scheme, pulmonary and thymic tumors fall into one of the following three grades [4] :

The European Society for Medical Oncology (ESMO) uses only mitotic count for bronchial and thymic tumors for determining tumor grade, as follows [6] :

The National Comprehensive Cancer Network (NCCN) recommends that tumor differentiation, mitotic rate, and Ki-67 rate be included in the pathology report and that the specific classification and grading scheme be noted to avoid confusion. Clinicians are advised to view histologic grade as a general guide and use clinical judgment to make treatment decisions, particularly in cases of discordance between differentiation and Ki-67 proliferation results. [7]

NCCN guidelines recommend staging according to the 7th edition of the American Joint Committee on Cancer’s AJCC Cancer Staging Manual. [7] The AJCC uses separate staging systems for carcinoids of the stomach, duodenum/ampulla/jejunum/ileum, colon/rectum, and appendix, as well as adrenal gland tumors. Bronchopulmonary carcinoids are staged using the same system as for other pulmonary malignancies, and pancreatic NETS are staged the same as for exocrine pancreatic tumors. [8]

For staging of GEP-NETs, the ESM0 guidelines, updated in 2012, utilize the tumor-node-metastasis (TNM) classification created by the ENETS and the 2010 WHO grading system. [9] For staging of bronchopulmonary and thymus NETs, the ESMO prefers the AJCC system. [6] For adrenal carcinoma staging, the 2009 European Network for the Study of Adrenal Tumors (ENSAT) TNM system is recommended over the AJCC system. [10]

In 2012, the UK and Ireland Neuroendocrine Tumour Society (UKI NETS) released updated guidelines for the management of GEP-NETs. Recommendations for grading and staging are as follows [11] :

For grading: WHO 2010 grading system

For staging: 7th edition of the AJCC Cancer Staging Manual

Also stage NETs of the stomach, pancreas and appendix with the ENETS site-specific T-staging system

The TNM classification used should be specified

Underlying features of the T-stage classification (eg, tumor size, extent of invasion) should be documented to allow for translation between different classification systems

In 2013, the North American Neuroendocrine Tumor Society (NANETS) concluded that while the criteria differ among the various classification systems, the underlying data are similar and pathology reports should include notation of the systems and parameters used to assign the grade and stage. [12]

The following organizations have issued clinical guidelines for the treatment of carcinoid tumors:

National Comprehensive Cancer Network (NCCN)

North American Neuroendocrine Tumor Society (NANETS)

European Neuroendocrine Tumor Society (ENETS)

European Society for Medical Oncology (ESM0)

UK and Ireland Neuroendocrine Tumour Society (UKI NETS)

NCCN guidelines recommend resection as the primary treatment for most carcinoid tumors of the gastrointestinal (GI) tract, lung, and thymus. Specific recommendations vary by tumor subtype. However, for neuroendocrine tumors at any site, cholecystectomy is recommended during surgical resection if treatment with a somatostatin analog (ie, octreotide, lanreotide) is planned, due to the increased rate of biliary problems associated with long-term use of these agents. [7]

For gastric tumors, the NCCN recommendations are as follows [7] :

With hypergastrinemia and tumors ≤2 cm: Endoscopic resection with biopsy or observation; or octreotide or lanreotide for patients with Zollinger-Ellison syndrome.

With hypergastrinemia and tumors >2 cm: Endoscopic resection, if possible, or surgical resection

With normal gastrin levels: Radical gastric resection and regional lymphadenectomy; endoscopic or wedge resection can be considered for tumors ≤2 cm

Gastric carcinoids can be subclassified into the following three distinct groups [13] :

Type I – Those associated with chronic atrophic gastritis/pernicious anemia (70-80%)

Type II – Those associated with Zollinger-Ellison syndrome with multiple endocrine neoplasia type I (MEN I) (5%)

Type III – Sporadic NETs of the stomach (15-20%)

In 2013, NANETS released updated guidelines with the following recommendations for treatment of gastric carcinoid tumors [14] :

Type I or II, <1 cm: Surveillance or endoscopic removal

Type I, 1 cm to <2 cm: Surveillance with repeat endoscopy every 3 years or endoscopic resection

Type II, 1 cm to <2 cm: Endoscopic resection

Type I, ≥2 cm (≤6 polyps), or type II ≥2 cm: Endoscopic resection, if possible, or open surgical resection

Type I, ≥2 cm (>6 polyps): Individualized treatment required; surveillance, endoscopic resection, or surgical resection

Type III: Partial gastrectomy and lymph node dissection

The 2016 revised ENETS guidelines prefer conservative management strategies over surgery for type I tumors. The guidelines recommend resection of tumors ≥10 mm performed by endoscopists experienced in gastric tumors, using either endoscopic mucosal resection (EMR) or endoscopic submucosal dissection (ESD). [15]

For type II tumors, local or limited excision can be recommended, but this should be patient tailored at multidisciplinary NET centers of excellence.Type III tumors should be treated similarly to gastric adenocarcinoma with surgery (partial or total gastrectomy with lymph node dissection). Systemic therapy is required for inoperable or stage 4 disease. [15]

For duodenal tumors, the NCCN recommends the following treatments [7] :

Endoscopic resection with follow-up upper endoscopy

Transduodenal local excision with or without lymph node sampling

Pancreatoduodenectomy

ENETS guidelines provide the following recommendations for treatment of duodenal tumors [15] :

All localized tumors should be removed

Endoscopic resection for tumors ≤1 cm confined to the submucosal layer, without lymph node or distant metastasis

Surgical resection with lymphadenectomy for tumors in the periampullary region

Surgical resection for tumors >2 cm and tumors of any size with lymph node metastases

NCCN recommendations are as follows [7] :

Surgical resection with lymphadenectomy

Careful examination of the entire bowel and assessment of proximity to or involvement of the superior mesenteric artery and superior mesenteric vein

Prophylactic cholecystectomy if further treatment with octreotide or lanreotide is planned

The NANETS guidelines include the following recommendations [14] :

Tumors of the cecum: Right hemicolectomy with node dissection

Tumors of the jejunal or ileum: Resection with node dissection; full bowel examination required

Distal colon and rectum tumors <1 cm: Endoscopic resection (polypectomy, endoscopic mucosal resection, endoscopic submucosal dissection) for mucosal or submucosal tumors

Distal colon and rectum tumors 1-2 cm: Transanal excision via rigid or flexible dissection; could also be considered after endoscopic resection with positive margins

Distal colon and rectum tumors >2 cm: Surgical resection (low anterior resection or abdominoperineal resection) for larger tumors, tumors invading muscularis propria, or those with lymphadenopathy

The ENETS guidelines provide following recommendations [16, 17] :

Rectal tumors [16]

Endoscopic resection by simple polypectomy, endoscopic mucosal resection (EMR) with modified EMR band ligation, endoscopic submucosal dissection (ESD) and transanal endoscopic microsurgery (TEMS).

For lesions <10 mm and no involvement of the muscularis propria, EMR is adequate, but EMR band-assisted ligation may improve the number of complete resections 

 If EMR results in an incomplete resection, then ESD or TEMS may be indicated as salvage therapy

 Jejunum and ileum tumors [17]

NCCN recommendations for appendix NETs are as follows [7] :

Tumors ≤2 cm confined to the appendix: Appendectomy

Tumors ≤2 cm with lymphovascular or mesoappendiceal invasion or atypical histologic features: More aggressive treatment can be considered

Incomplete resection or tumors >2 cm: Staging with abdominal/pelvic CT or MRI; if no distant disease, reexploration with a right hemicolectomy

The NANETS guidelines include the following recommendations [14] :

Excision for tumors ≤2 cm; consider right hemicolectomy with node dissection if high-risk features are present

Tumors >2 cm: Right hemicolectomy with node dissection

The 2016 ENETS revised guidelines recommendations include the following [18] :

In rare cases, right hemicolectomy with node dissection may be indicated for appendiceal tumors measuring 1–2 cm. This exception applies only to tumors with one or more of the following [18] :

NCCN recommendations for the treatment of unresectable and/or metastatic carcinoid tumors of the GI tract include the following{ref6:

Somatostatin scintigraphy to assess sites of metastases and somatostatin receptor status if octreotide or lanreotide is being considered

Limited hepatic metastases: Complete resection of primary tumor and metastases with curative intent; noncurative debulking surgery in select cases

Unresectable hepatic progressive disease: Radiofrequency ablation or cryoablation or hepatic regional therapy (arterial embolization, chemoembolization, or radioembolization)

Palliative small bowel resection for patients with abdominal pain from bowel obstruction or ischemia related to the primary tumor

Octreotide or lanreotide to control tumor growth in patients with clinically significant tumor burden or progressive disease; everolimus can be considered for advanced tumors; for persistent diarrhea, telotristat 250 mg orally, three times daily, can be added; additional therapy may be used for any persistent symptoms (eg, flushing, diarrhea)

Consider capecitabine if no other options are feasible (category 3)

Consider interferon alfa-2b after octreotide or lanreotide failure (category 3)

Malignant carcinoid syndrome: Octreotide or lanreotide; cardiology consultation, and echocardiogram to assess for heart disease

Liver transplantation is investigational and not recommended as routine care

Note that the use of ablative techniques for hepatic disease is associated with increased infectious complications. Although the NCCN guidelines cite category 2b evidence for cryoablation and radiofrequency ablation, most centers use radiofrequency or microwave ablation. Cryoablation is generally used only in centers providing ablation for renal cell cancers, and it is associated with a small but definite risk of subsequent acute respiratory distress syndrome. [19]

For thymic NETs, NCCN recommendations include the following [7] :

Localized disease: Surgical resection

Locoregional disease: Reresection; if resection is incomplete, follow with radiation therapy and/or chemotherapy

The NANETS guidelines include the following additional recommendations for thymic NETs [14] :

Locoregional disease: Surgical resection including mediastinal lymphadenectomy

Metastatic or unresectable disease: Options include radiation therapy, everolimus, interferon alpha, or temozolomide

The ESMO guidelines note that a protracted follow-up should always be performed after surgical resection becuse of the high rates of recurrence. For metastatic disease, although the available chemotherapy regimens have not demonstrated good effects, cisplatinum-based regimens have been of value and temozolomide-based treatment gives some benefit. [6]

Brochopulmonary NETs

NCCN recommendations for bronchopulmonary NETs are as follows [7] :

Stage I, II, and IIIA: Lobectomy or wedge resection for peripheral low-grade neuroendocrine carcinoma and lymph node dissection or sampling

Stage IIIA low grade nonresectable tumors: Radiation therapy

Stage IIIA intermediate grade nonresectable tumors: Cisplatin/etoposide and radiation therapy

Stage IIIB (except for T4 due to multiple lung nodules): Cisplatin/etoposide with or without radiation therapy

Stage IIIBm (T4 due to multiple lung nodules) or stage IV: Systemic therapy; no preferred regimen; options include cisplatin/etoposide, temozolomide with or without capecitabine, sunitinib, or everolimus; consider octreotide for symptoms of malignant carcinoid syndrome

The NANETS guidelines and the ESMO guidelines are similar to those of the NCCN, with some minor variances. [14, 6] The ESMO guidelines include the following additional recommendations [6] :

Bronchoscopic laser excision should be considered a suboptimal treatment and be reserved for inoperable patients or performed as a preoperative disobliterating procedure

Lobectomy and sleeve resection are preferred for locoregional tumors and systemic nodal dissection should be performed

Pneumonectomy should be avoided

Additionally, NANETS suggests that interferon alpha should be considered for metastatic or unresectable disease. [14]

National Comprehensive Cancer Network (NCCN) guidelines recommend resection as the primary treatment for most localized pancreatic neuroendocrine tumors (NETs). Exceptions include patients with life-limiting comorbidities or high surgical risk. In addition, observation may be appropriate for incidentally discovered tumors <1 cm, depending on the site. [7]

The NCCN recommends that before surgery, symptoms of hormonal excess should be treated with octreotide or lanreotide; however, such treatment is contraindicated in patients with insulinoma because of the potential for fatal complications. Cholecystectomy is recommended during surgical resection if treatment with octreotide or lanreotide is planned, due to the increased rate of biliary problems associated with long-term use of these agents. [7]

For nonfunctioning pancreatic NETs, treatment recommendations are as follows:

Tumors ≤2 cm: Enucleation of the tumors with strong consideration of lymph node resection or pancreatectomy (Whipple type or proximal) with or without regional node resection, or distal pancreatectomy with or without regional node resection/splenectomy

Tumors >2 cm located at the head: Whipple-type or proximal pancreatectomy with regional node resection

Tumors >2 cm located distally: Distal pancreatectomy with regional node resection and splenectomy

Recommendations for gastrinoma treatment include the following:

Manage gastric hypersecretion with proton pump inhibitors; consider octreotide or lanreotide

Occult tumors: Observation or exploratory surgery, including duodenotomy and intraoperative ultrasound with enucleation; local resection if tumors identified, and removal of periduodenal nodes

Duodenum tumors: Duodenotomy and intraoperative ultrasound with local resection or enucleation and periduodenal node dissection

Tumors at head of pancreas: Exophytic or peripheral tumors require enucleation and periduodenal node dissection; deeper or invasive tumors or those with proximity to the main pancreatic duct require pancreatoduodenectomy

Distal tumors: Distal pancreatectomy with or without splenectomy.

Recommendations for insulinoma treatment include the following:

Stabilize glucose levels with diet and/or diazoxide; everolimus may be considered

Primary treatment is enucleation; consider laparoscopic resection for solitary tumors

Deeper or invasive tumors or those close to the main pancreatic duct require pancreatoduodenectomy if located in the head and laparoscopic distal pancreatectomy if in a distal location, with preservation of the spleen for smaller tumors

Recommendations for glucagonoma treatment include the following:

Stabilize glucose levels with IV fluids; treat hyperglycemia and diabetes

Tumors located in the head of the pancreas: Pancreatectomy with resection of peripancreatic lymph nodes

Distal tumors: Distal pancreatectomy with splenectomy and resection of peripancreatic lymph nodes

Consider perioperative anticoagulation due to increased risk of pulmonary emboli

Recommendations for treatment of NETs that secrete vasoactive intestinal peptide (VIPomas) include the following:

Stabilize with IV fluids; correct electrolyte imbalance

Tumors located in the head of the pancreas: Pancreatectomy with resection of peripancreatic lymph nodes

Distal tumors: Distal pancreatectomy with splenectomy and resection of peripancreatic lymph nodes.

The North American Neuroendocrine Tumor Society (NANETS) guidelines and the European Society for Medical Oncology (ESMO) guidelines for treatment of neuroendocrine pancreatic tumors are similar to those of the NCCN, with some minor variances. The ESMO guidelines do not recommend laparoscopic resection or surgical treatment of G2 pancreatic NETs. [9]

NCCN recommendations for the treatment of unresectable and/or metastatic pancreatic tumors include the following [7] :

Limited hepatic metastases: Complete resection of primary tumor and metastases with curative intent; noncurative debulking surgery in select cases

Asymptomatic unresectable disease: For select patients with low tumor burden and stable disease, consider observation with marker assessment and imaging every 3-12 months until significant disease progression occurs; lanreotide or octreotide can be considered

Symptomatic unresectable disease: Octreotide or lanreotide; everolimus or sunitinib; or cytotoxic chemotherapy

Hepatic-directed therapies include cytoreductive surgery or ablative therapy; bland hepatic arterial embolization, radioembolization, and chemoembolization are additional options but the optimal embolization technique has not been determined

Liver transplantation is investigational and not recommended as routine care

The European Neuroendocrine Tumor Society (ENETS) consensus guidelines are generally similar to those of the NCCN, and include the following recommendations [20] :

Surgery with curative intent, and/or loco‐regional or ablative therapies, should be considered at initial diagnosis and in the course of the disease as an alternative to systemic therapies

In patients with a functioning NET, all liver-directed therapies require prior initiation of somatostatin analog therapy (or other specific meaures for controlling symptoms)

Debulking surgery is indicated to improve symptom control in selected patients with functioning NET with predominant liver disease, even if the liver tumor burden can be reduced by less than 90%

Liver transplantation is an option in very highly selected patients, preferably young patients with functional syndromes who experience early resistance to medical therapy.

In 2014, The Endocrine Society (TES), the American Association for Clinical Chemistry (AACC), and the European Society of Endocrinology (ESE) released joint clinical practice guidelines for the management of pheochromocytoma and paraganglioma (referred to together as PPGL). The guidelines include recommendations (based on strong evidence) and suggestions (based on weaker evidence). [21]

In patients suspected of having PPGL, biochemical testing via measurement of plasma free metanephrines or urinary fractionated metanephrines is recommended. The use of liquid chromatography with mass spectrometry or electrochemical-detection methods is suggested over other laboratory methods. Patients with a known germline mutation that predisposes to PPGL should undergo periodic biochemical testing. [21]

The 2010 guidelines from the North American Neuroendocrine Tumor Society (NANETS) recommend biochemical testing for pheochromocytoma that includes measurements of fractionated metanephrines in plasma, urine, or both, as available, in the following cases [22] :

Symptomatic patients

Patients with an adrenal incidentaloma

Patients who have a hereditary risk for developing a pheochromocytoma or paraganglioma (extra-adrenal pheochromocytoma)

For imaging studies, the joint TES/AACC/ESE guidelines recommend computed tomography (CT) as first line, rather than magnetic resonance imaging (MRI). However, MRI is an option in certain patients, such as those with metastatic PPGL; those allergic to CT contrast media; and those for whom radiation exposure should be limited, such as pregnant women. [21]

For preoperative management, TES/AACC/ESE recommendations include the following:

Blockade of hormonally functional PPGL to prevent cardiovascular complications

Medical treatment to normalize blood pressure and heart rate

A high-sodium diet with supplemental fluid intake to prevent severe hypotension after removal of the tumor

The TES/AACC/ESE guidelines recommend minimally invasive (eg, laparoscopic) adrenalectomy for most adrenal pheochromocytomas, with open resection reserved for very large or invasive pheochromocytomas. Open resection is suggested for paragangliomas, although laparoscopic resection is an option for smaller tumors. Partial adrenalectomy is also a possibility for certain types of patients.

In the immediate postoperative period, the TES/AACC/ESE guidelines recommend monitoring of blood pressure, heart rate, and glucose levels. Postoperative measurement of plasma or urine metanephrine levels and lifelong annual biochemical testing are suggested.

For genetic testing, TES/AACC/ESE recommendations are as follows:

Patients with PPGLs should be engaged in shared decision-making for genetic testing

Patients with paraganglioma should undergo testing of succinate dehydrogenase (SDH) mutations

Patients with metastatic disease should undergo testing for SDHB mutations

Genetic testing should include pre- and posttest genetic counseling

The 2010 NANETS recommendations for treatment of advanced disease include the following [22] :

Surgical debulking to release tumor pressure on surrounding tissues or to decrease tumor mass

In select patients, radiofrequency ablation or cryoablation are options

Chemotherapy is preferred in patients with negative metaiodobenzylguanidine (MIBG) scintigraphy and in those with rapidly progressing tumors

External-beam irradiation of bone metastases or radiofrequency and cryoablation in selected cases only

Guidelines issued by the American Association of Clinical Endocrinologists (AACE) and American Association of Endocrine Surgeons (AAES) in 2009 for the management of adrenal incidentalomas recommend that evaluation of patients with an adrenal incidentaloma include clinical, biochemical, and radiographic testing for the following [23] :

Hypercortisolism

Aldosteronism (if hypertensive)

Pheochromocytoma or a malignant tumor

The simplest screening test for autonomous cortisol secretion from an incidentaloma is a 1-mg overnight dexamethasone suppression test. Salivary cortisol, dexamethasone suppression, and urine free cortisol testing can be used if clinical suspicion is high (eg, in patients with hypertension, obesity, diabetes mellitus, or osteoporosis).

Primary aldosteronism is confirmed by lack of aldosterone suppression on a 24-hour urine study with salt loading.

To determine the incidentaloma subtype, high-resolution computed tomography scanning should be performed in all patients. In addition, adrenal venous sampling should be performed in most patients older than 40 years.

Treatment recommendations are as follows:

Surgical resection should be reserved for those with worsening hypertension, abnormal glucose tolerance, dyslipidemia, or osteoporosis

In patients with primary aldosteronism and a unilateral source of aldosterone excess, laparoscopic total adrenalectomy is preferred over open approaches

Patients with bilateral idiopathic hyperaldosteronism (IHA) should be managed with selective and nonselective mineralocorticoid receptor blockers

Open adrenalectomy should be performed if adrenocortical carcinoma is suspected

For patients with adrenal incidentalomas who do not fulfill the criteria for surgical resection, the guidelines recommend radiographic reevaluation at 3 to 6 months and then annually for 1 to 2 years. Hormonal evaluation should be performed annually for 5 years.

National Comprehensive Cancer Network (NCCN) guidelines for treatment of adrenal carcinoma include separate recommendations for localized and metastatic disease. [7] Recommendations for localized disease are as follows:

Resection of primary tumor and adjacent lymph nodes

Open adrenalectomy recommended

External-beam radiation therapy and adjuvant mitotane may be considered in patients at high risk for recurrence

NCCN treatment recommendations for metastatic disease are as follows:

Observation for clinically indolent disease with imaging and biomarkers (if functional) every 3 months

Resection of primary tumor and metastases if >90% removable, particularly if functional

Systemic therapy, preferably within a clinical trial

The European Society for Medical Oncology (ESMO) guidelines utilize the 2005 proposed diagnostic workup of the European Network for the Study of Adrenal Tumors (ENSAT) for evaluation of possible adrenocortical carcinomas (ACC), which includes a detailed endocrine assessment and comprehensive hormonal analysis. Additional recommendations include the following [10] :

Open surgery with transperitoneal access for stages I-III when complete resection is possible

Laparoscopic adrenalectomy only for selected patients with small tumors and no evidence of invasiveness or adrenal incidentalomas

Margin-free complete resection of locally advanced ACC may require resection of parts of adjacent organs

Re-resection of ACC for recurrence, if margin-free resection is possible and the time to recurrence was >12 months

Adjuvant mitotane therapy in patients with incomplete/R1 or Rx resection and/or Ki67 expression in ≤10% of neoplastic cells

For bone metastasis, palliative radiotherapy is an option; arterial chemoembolization and radiofrequency ablation may be beneficial in selected patients

For evaluation of duodenal, jejunal, ileal, and colonic neuroendocrine tumors (NETs), the National Comprehensive Cancer Network (NCCN) recommends abdominal/pelvic multiphasic CT or MRI and any of the following as appropriate [7] :

For evaluation of T1 rectal NETs, the NCCN recommends endorectal MRI or endoscopic ultrasound (EUS); for T2-T4 rectal NETs, recommended studies are as follows:

In addition, the following are recommended to be done, as appropriate:

For evaluation of gastric NETs, NCCN recommendations are as follows:

For evaluation of bronchopulmonary NETs, the NCCN recommends chest CT and abdominal multiphasic CT or MRI, with the following as appropriate:

For evaluation of thymus NETs, the NCCN recommends chest/mediastinal multiphasic CT and abdominal multiphasic CT or MRI, the following as appropriate:

European Neuroendocrine Tumor Society (ENETS) consensus guidelines for high-grade gastroenteropancreatic neuroendocrine tumors, issued in 2016, include the following minimal consensus recommendations on diagnosis [24] :

Bosman FT, Carneiro F, Hruban RH, Theise ND. World Health Organization Classification of Tumours of the Digestive System. Lyon, France: IARC; 2010.

Rindi G, Klöppel G, Alhman H, Caplin M, Couvelard A, et al. TNM staging of foregut (neuro)endocrine tumors: a consensus proposal including a grading system. Virchows Arch. 2006 Oct. 449 (4):395-401. [Medline]. [Full Text].

Rindi G, Klöppel G, Couvelard A, Komminoth P, Körner M, Lopes JM, et al. TNM staging of midgut and hindgut (neuro) endocrine tumors: a consensus proposal including a grading system. Virchows Arch. 2007 Oct. 451 (4):757-62. [Medline]. [Full Text].

Travis WD, Brambilla E, Muller-Hermelink HK, Harris CC. World Health Organization Classification of Tumours of the Lung, Pleura, Thymus and Heart. Lyon, France: IARC; 2004.

[Guideline] Caplin ME, Baudin E, Ferolla P, Filosso P, Garcia-Yuste M, Lim E, et al. Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids. Ann Oncol. 2015 Aug. 26 (8):1604-20. [Medline]. [Full Text].

[Guideline] Öberg K, Hellman P, Ferolla P, Papotti M, ESMO Guidelines Working Group. Neuroendocrine bronchial and thymic tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2012 Oct. 23 Suppl 7:vii120-3. [Medline]. [Full Text].

[Guideline] National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Neuroendocrine Tumors. NCCN. Available at http://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. December 26, 2017; Accessed: Version 3.2017 — June 13, 2017.

Edge SB, Byrd DR, Compton CC, et al. AJCC Cancer Staging Manual. 7th Ed. New York: Springer; 2010.

[Guideline] Öberg K, Knigge U, Kwekkeboom D, Perren A, ESMO Guidelines Working Group. Neuroendocrine gastro-entero-pancreatic tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2012 Oct. 23 Suppl 7:vii124-30. [Medline]. [Full Text].

[Guideline] Berruti A, Baudin E, Gelderblom H, Haak HR, Porpiglia F, Fassnacht M, et al. Adrenal cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2012 Oct. 23 Suppl 7:vii131-8. [Medline]. [Full Text].

[Guideline] Ramage JK, Ahmed A, Ardill J, et al., UK and Ireland Neuroendocrine Tumour Society. Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs). Gut. 2012 Jan. 61 (1):6-32. [Medline]. [Full Text].

Klimstra DS, Modlin IR, Coppola D, Lloyd RV, Suster S. The pathologic classification of neuroendocrine tumors: a review of nomenclature, grading, and staging systems. Pancreas. 2010 Aug. 39 (6):707-12. [Medline]. [Full Text].

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[Guideline] Kunz PL, Reidy-Lagunes D, Anthony LB, et al; North American Neuroendocrine Tumor Society. Consensus guidelines for the management and treatment of neuroendocrine tumors. Pancreas. 2013 May. 42 (4):557-77. [Medline]. [Full Text].

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[Guideline] Niederle B, Pape UF, Costa F, Gross D, Kelestimur F, Knigge U, et al. ENETS Consensus Guidelines Update for Neuroendocrine Neoplasms of the Jejunum and Ileum. Neuroendocrinology. 2016. 103 (2):125-38. [Medline]. [Full Text].

[Guideline] Pape UF, Niederle B, Costa F, Gross D, Kelestimur F, Kianmanesh R, et al. ENETS Consensus Guidelines for Neuroendocrine Neoplasms of the Appendix (Excluding Goblet Cell Carcinomas). Neuroendocrinology. 2016. 103 (2):144-52. [Medline]. [Full Text].

Chapman WC, Debelak JP, Wright Pinson C, Washington MK, Atkinson JB, Venkatakrishnan A, et al. Hepatic cryoablation, but not radiofrequency ablation, results in lung inflammation. Ann Surg. 2000 May. 231 (5):752-61. [Medline].

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[Guideline] Lenders JW, Duh QY, Eisenhofer G, et al; Endocrine Society. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014 Jun. 99 (6):1915-42. [Medline]. [Full Text].

[Guideline] Chen H, Sippel RS, O’Dorisio MS, Vinik AI, Lloyd RV, Pacak K, et al. The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas. 2010 Aug. 39 (6):775-83. [Medline]. [Full Text].

[Guideline] Zeiger MA, Thompson GB, Duh QY, et al; American Association of Clinical Endocrinologists, American Association of Endocrine Surgeons. The American Association of Clinical Endocrinologists and American Association of Endocrine Surgeons medical guidelines for the management of adrenal incidentalomas. Endocr Pract. 2009 Jul-Aug. 15 Suppl 1:1-20. [Medline].

[Guideline] Garcia-Carbonero R, Sorbye H, Baudin E, Raymond E, Wiedenmann B, Niederle B, et al. ENETS Consensus Guidelines for High-Grade Gastroenteropancreatic Neuroendocrine Tumors and Neuroendocrine Carcinomas. Neuroendocrinology. 2016. 103 (2):186-94. [Medline]. [Full Text].

[Guideline] Falconi M, Eriksson B, Kaltsas G, Bartsch DK, Capdevila J, Caplin M, et al. ENETS Consensus Guidelines Update for the Management of Patients with Functional Pancreatic Neuroendocrine Tumors and Non-Functional Pancreatic Neuroendocrine Tumors. Neuroendocrinology. 2016. 103 (2):153-71. [Medline]. [Full Text].

Evan S Ong, MD, MS Assistant Professor of Surgery, Section of Surgical Oncology, University of Arizona College of Medicine

Evan S Ong, MD, MS is a member of the following medical societies: Society of Surgical Oncology, Americas Hepato-Pancreato-Biliary Association, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Mariclaire Cloutier Freelance editor, Medscape Drugs & Diseases

Disclosure: Nothing to disclose.

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