Gallbladder polyps are common findings in abdominal ultrasound exams, appearing in about 4.5% of adults. While most of them do not have malignant potential, a small percentage – between 4% and 10% – are adenomas, which can become malignant.

Studies show that the size of the polyp is the main risk factor for the development of cancer, especially when adenomatous polyps are 10 millimeters or more, presenting a chance of malignancy between 37% and 55%.

However, it is difficult to differentiate between adenomatous polyps and polyps without malignant potential in preoperative exams. Therefore, it is important for the gastroenterologist to know the correct indication for surgery in patients with gallbladder polyps in order to avoid an unnecessary surgical procedure in patients without risk and, mainly, correctly indicating the procedure in the population with a higher risk of malignancy.

In this article, we will summarize the indications for follow-up and treatment of gallbladder polyps.

SYMPTOMATIC PATIENTS

Gallbladder polyps rarely cause symptoms, however some studies have reported an association between gallbladder polyps and undetected stones on ultrasound and/or cholecystitis. The joint European guideline of 2022 recommends cholecystectomy for patients who present symptoms such as biliary colic or complications (example: pancreatitis) and who have favorable clinical conditions for surgery [1]. The rate of symptom improvement is variable in the literature (40-90% improvement).

Patients with nonspecific dyspeptic symptoms without biliary colic should be treated conservatively (unless there are other indications for polyp removal), since the pathogenesis of these symptoms is not clear and cholecystectomy may not relieve symptoms. These patients should be treated symptomatically, as with other patients with functional dyspepsia.

ASYMPTOMATIC PATIENTS WITH RISK FACTORS FOR GALLBLADDER CANCER

Risk factors for gallbladder cancer include:

• age >60 years
• primary sclerosing cholangitis
• Asian ethnicity
• sessile polyps with focal gallbladder wall thickness >4 mm

The approach will depend on the size of the polyp:

• Polyps ≤5 mm: surveillance ultrasound at 6 months, 1 year, and 2 years. Follow-up can be discontinued if there is no growth during this period.
• Polyps 6 to 9 mm: cholecystectomy is recommended if the patient is clinically fit and accepts surgery.
• Polyps 10 to 20 mm: Polyps 10 to 20 mm should be considered as possibly malignant. Laparoscopic cholecystectomy is recommended.
• Polyps >20 mm: are generally malignant. Patients should undergo preoperative staging with computed tomography or endoscopic ultrasound. Radical treatment consists of extended cholecystectomy with lymph node dissection and partial hepatic resection at the gallbladder bed.

ASYMPTOMATIC PATIENTS WITHOUT RISK FACTORS FOR GALLBLADDER CANCER

In asymptomatic patients without risk factors for gallbladder cancer, surveillance recommendations vary according to the size of the polyp.

• For polyps ≤ 5 mm: no follow-up is necessary. *
• For polyps 6 to 9 mm: perform abdominal ultrasound at 6 months, 1 year, and 2 years. Surveillance can be discontinued if there is no growth during this period.

* This strategy is aligned with the practices of the American College of Radiology [2] and the Canadian Association of Radiologists Incidental Findings Working Group [3], which recommend that polyps smaller than 7 mm do not require follow-up.

IMPORTANT CONSIDERATIONS IN PATIENTS UNDERGOING SURVEILLANCE

1. Increase in polyp size

The joint European guideline of 2017 recommended that:

• An increase in size greater than 2 mm in the images probably represents a clinically significant increase and should prompt referral to a surgeon for cholecystectomy.

The update of this guideline in 2022 recommends that:

• If the polypoid lesion grows 2 mm or more during the 2-year follow-up period, then the current size of the polypoid lesion should be considered along with the patient’s risk factors. Multidisciplinary discussion should be held to decide whether to continue surveillance or if cholecystectomy is indicated.

An important retrospective study published in 2019 including more than 600,000 adults undergoing cholecystectomy showed that:

• The growth of 2 mm or more seems to be part of the natural history of gallbladder polyps.
  • The likelihood of a polyp growing at least 2 mm in 10 years was 66% for polyps smaller than 6 mm and 53% for polyps between 6-10mm.
  • Important: this growth does not seem to be associated with future gallbladder cancer. None of the 507 patients with polyps that grew to 10 mm or more were subsequently diagnosed with cancer.
• The first year is the most important:
  • Most cases of gallbladder cancer were diagnosed in the first year, probably representing neoplasms already present at the time of diagnosis.
  • Polyps initially smaller than 10 mm were almost never associated with future cases of gallbladder cancer (rate 1.05 per 100,000 person-years)
  • Polyps with ≥ 10 mm at diagnosis were rarely associated with gallbladder cancer after the first year.

The cherry on top of this study:

• In addition, we observed that similar proportions of adults were diagnosed with gallbladder Ca (0.053% vs. 0.054%), whether an initial ultrasound showed a gallbladder polyp or not. These findings suggest that there may not be a general link between gallbladder polyps and gallbladder neoplasia, and that gallbladder polyps are an incidental finding.

2. Duration of surveillance

The duration of surveillance in patients with gallbladder cancer is not clear. The updated joint European guidelines recommend discontinuing surveillance in two years if there is no growth of the polyps. Some authors recommend maintaining surveillance for at least five years. However, in patients with risk factors for gallbladder cancer, we should maintain surveillance for gallbladder cancer with abdominal USG indefinitely.

3. Adenomyomatosis

Patients with typical features of adenomyomatosis on ultrasound do not require surveillance or cholecystectomy.

4. If the gallbladder polyp disappears during follow-up

If the gallbladder polyp disappears during follow-up, the follow-up surveillance can be discontinued.

References

1. Foley KG, Lahaye MJ, Thoeni RF, Soltes M, Dewhurst C, Barbu ST, Vashist YK, Rafaelsen SR, Arvanitakis M, Perinel J, Wiles R, Roberts SA. Management and follow-up of gallbladder polyps: updated joint guidelines between the ESGAR, EAES, EFISDS and ESGE. Eur Radiol. 2022 May;32(5):3358-3368. doi: 10.1007/s00330-021-08384-w. Epub 2021 Dec 17. PMID: 34918177; PMCID: PMC9038818.
2. Sebastian S, Araujo C, Neitlich JD, Berland LL (2013) Manag- ing incidental findings on abdominal and pelvic CT and MRI, Part 4: white paper of the ACR Incidental Findings Commit- tee II on gallbladder and biliary findings. J Am Coll Radiol 10(12):953–956
3. Bird JR, Brahm GL, Fung C, Sebastian S, Kirkpatrick IDC (2020) Recommendations for the management of incidental hepatobiliary findings in adults: endorsement and adaptation of the 2017 and 2013 ACR Incidental Findings Committee White Papers by the Canadian Association of Radiologists Incidental Findings Working Group. Can Assoc Radiol J 71(4):437–447
4. Szpakowski JL, Tucker LY. Outcomes of Gallbladder Polyps and Their Association With Gallbladder Cancer in a 20-Year Cohort. JAMA Netw Open. 2020 May 1;3(5):e205143. doi: 10.1001/jamanetworkopen.2020.5143. PMID: 32421183; PMCID: PMC7235691.

How to cite this article

Martins BC. Management of Gallbladder Polyps: When to Follow Up and When to Recommend Cholecystectomy? Gastropedia 2024; vol 1. Available at: https://gastropedia.pub/en/surgery/management-of-gallbladder-polyps-when-to-follow-up-and-when-to-recommend-cholecystectomy

Introduction

Gallbladder polyps are usually incidental findings diagnosed during abdominal ultrasound exams or during cholecystectomy. They usually do not present symptoms, but occasionally they can cause discomforts similar to those caused by gallstones.

Most of these lesions are not neoplastic, but rather hyperplastic or represent lipid deposits.

With the widespread use of ultrasound, polypoid lesions in the gallbladder are being increasingly detected. However, often the image is not enough to rule out the possibility of neoplasia or pre-malignant adenomas. In this article, we will review the clinical importance and differential diagnosis of gallbladder polyps.

Classification

Polypoid lesions in the gallbladder can be categorized as benign or malignant. Benign lesions can be subdivided into neoplastic and non-neoplastic.

Non-neoplastic benign polyps

The most common benign non-neoplastic lesions are cholesterol polyps, followed by adenomyomatosis and inflammatory polyps.

• Cholesterol polyps and cholesterosis:
  • it is a benign condition characterized by the accumulation of lipids in the mucosa of the gallbladder wall.
  • they are the most common types of gallbladder polyps, reaching up to 10% or more.
  • It can be of the diffuse or polypoid type.
  • The term cholesterosis refers to the diffuse type, which is usually diagnosed incidentally during cholecystectomy, causing the appearance of a “strawberry gallbladder” due to the contrast it makes with the gallbladder mucosa.
  • Cholesterol polyps are the polypoid form of cholesterosis, being the most common gallbladder polyp, usually diagnosed incidentally on ultrasound.
  • Although usually asymptomatic, in some patients it can cause symptoms and complications similar to those caused by gallstones.
• Adenomyomatosis:
  • it is an abnormality of the gallbladder characterized by excessive growth of the mucosa, thickening of the muscular wall and intramural diverticula.
  • The prevalence of gallbladder adenomyosis is low, but it appears to have a higher prevalence in women than in men.
• Inflammatory polyps
  • Inflammatory polyps are the least common non-neoplastic polyps.
  • They appear as sessile or pedunculated and are composed of granulation and fibrous tissue with plasma cells and lymphocytes.
  • The polyps are usually 5 to 10 mm in diameter, although inflammatory polyps larger than 1 cm have been described

Neoplastic benign polyps

• Adenomas:
  • Adenomatous polyps of the gallbladder are the most common benign neoplastic lesions. Although the true incidence is unknown, in most series it is less than 0.5 percent.
  • Gallbladder adenomas are benign epithelial tumors composed of cells that resemble the epithelium of the bile ducts.
  • The risk of cancer increases with the size of the polyp, with larger adenomatous polyps having a risk of malignancy.
• Others — Other neoplastic lesions of the gallbladder such as fibromas, lipomas and leiomyomas, are rare. The natural history of these polyps is not well defined.

Malignant polyps:

• Most malignant polyps in the gallbladder are adenocarcinomas.
• The adenocarcinomas of the gallbladder are much more common than gallbladder adenomas, unlike the colon, where adenomas are much more common than adenocarcinomas.
• Squamous cell carcinoma, mucinous cystadenoma and gallbladder adenoacanthomas are rare

CANCER RISK

Most gallbladder polyps are benign, and most benign polyps, with the exception of adenomas, do not have malignant potential. The overall risk of gallbladder cancer in patients with gallbladder polyps appears to be low.

• In a large cohort study with over 35,000 adults with gallbladder polyps diagnosed by USG, 0.053% had gallbladder cancer, similar to the population without polyps (0.054%). [ref]
• The risk of progression to neoplasia varies according to the size of the polyps, occurring in 128/100,000 people for polyps > 10mm, but only in 1.3/100,000 people for polyps < 6mm.

Established risk factors for cancer

• Polyp size — The incidence of gallbladder cancer varies from 43 to 77% in polyps larger than 1 cm and 100% in polyps larger than 2 cm.
• Sessile polyp — sessile polyps are an independent risk factor for malignancy, with a 7x higher risk of gallbladder cancer. [ref]
• Age > 60 years: this is the cut-off adopted in guidelines for risk stratification and treatment guidance.
• Others: Indian ethnicity, primary sclerosing cholangitis

Conditions with uncertain risk

• Concomitant gallstones
• Adenomyomatosis — There is no evidence that the presence of adenomyosis increases the risk of gallbladder cancer. If the risk is increased, the magnitude of the increase appears to be small.

DIAGNOSIS

Gallbladder polyps are usually discovered incidentally on abdominal ultrasound exams. None of the available imaging modalities can unequivocally distinguish benign from malignant polyps. This can only be confirmed by histopathology after cholecystectomy.

Characteristics of gallbladder polyps on abdominal ultrasound:

• They can be single or multiple
• Sensitivity 84% and specificity 96% (meta-analysis with 16,260 patients)
• CHOLESTEROL POLYPS are usually multiple, homogeneous, polypoid and pedunculated, with echogenicity greater than the liver parenchyma.
  • They may or may not contain hyperechoic points.
  • Cholesterol polyps usually measure less than 1 cm.
  • In contrast to cholesterol polyps, diffuse cholesterosis does not have specific ultrasonographic findings, and its diagnosis is usually made after surgery.
• ADENOMAS are homogeneous lesions, isoechoic in relation to the liver parenchyma, have a smooth surface and usually do not have a pedicle.
  • The sessile morphology and focal thickening of the gallbladder wall greater than 4 mm are risk factors for malignancy.
• ADENOCARCINOMAS are homogeneous or heterogeneous polypoid structures that are usually isoechoic in relation to the liver parenchyma.
• The ADENOMYOMATOSIS can also cause a diffuse thickening with round anechoic f

Celiac disease is an autoimmune disease caused by an abnormal immune response to gluten peptides in the upper small intestine. It is important to understand its pathophysiology to know how to interpret the serological tests that assist in the diagnosis of celiac disease.

Gluten is a protein found in wheat, barley, and rye. In the small intestine, gluten is digested and breaks down into gliadin.

In celiac disease, gliadin manages to cross the epithelial barrier in the small intestine and reach the underlying lamina propria. The cause of gliadin epithelial permeability is uncertain, but it may be due to an underlying pathological process (for example, infection) or changes in intercellular junctions (tight junctions).

Immune response in the mucosa

When gliadin comes into contact with the lamina propria, it is deaminated by tissue transglutaminase (TTG). The deaminated gliadin then reacts with HLA-DQ2 or HLA-DQ8 receptors on antigen-presenting cells that stimulate the activation of T and B cells, leading to the release of cytokines, antibody production, and lymphocyte infiltration. Over time, inflammation causes villous atrophy, crypt hyperplasia in epithelial cells, and intraepithelial lymphocytosis.

Genetic Factors

The familial occurrence of celiac disease suggests that there is a genetic influence in its pathogenesis. Celiac disease does not develop unless a person has alleles that encode for the HLA-DQ2 or HLA-DQ8 proteins, products of two of the HLA genes.

However, many people, most of whom do not have celiac disease, carry these alleles; therefore, their presence is necessary, but not sufficient for the development of the disease.

Studies in siblings and identical twins suggest that the contribution of HLA genes to the genetic component of celiac disease is less than 50%.14 Several non-HLA genes that may influence susceptibility to the disease have been identified, but their influence has not been confirmed.

Environmental Factors

Epidemiological studies have suggested that environmental factors play an important role in the development of celiac disease. These include a protective effect of breastfeeding and the introduction of gluten in relation to weaning. Initial administration of gluten before 4 months of age is associated with an increased risk of developing the disease, and the introduction of gluten after 7 months is associated with a marginal risk. However, the overlap of gluten introduction with breastfeeding may be a more important protective factor in minimizing the risk of celiac disease.

The occurrence of certain gastrointestinal infections, such as rotavirus infection, also increases the risk of celiac disease in childhood.

Now understand how the research of autoantibodies can help in the diagnosis of celiac disease: The role of autoantibodies in the diagnosis of celiac disease

References

1. Green PH, Cellier C. Celiac disease. N Engl J Med. 2007 Oct 25;357(17):1731-43. doi: 10.1056/NEJMra071600. PMID: 17960014.
2. Kaminarskaya Yu?. Celiac disease, wheat allergy, and nonceliac sensitivity to gluten: topical issues of the pathogenesis and diagnosis of gluten-associated diseases. Clinical nutrition and metabolism. 2021;2(3):113–124.

How to Cite this article

Martins BC. Pathogenesis of Celiac Disease. Gastropedia, 2023, vol I. Available at: https://gastropedia.com.br/gastroenterology/intestine/pathogenesis-of-celiac-disease

Obesity is a public health problem with increasing incidence. In this article we will discuss its concept, etiology, classification and consequences.

1. Concept and epidemiology
2. Consequences of obesity
   • Metabolic syndrome
3. Classification
4. Etiology

1. Concept and epidemiology

Obesity can be defined by the accumulation of localized or generalized fatty tissue, caused by nutritional imbalance, associated or not with genetic or endocrine-metabolic disorder.

Obesity is a chronic disease whose prevalence is increasing in adults, children and adolescents and is currently considered a global epidemic. Once considered a problem of developed countries, obesity is now becoming a major health problem also in developing countries.

Obesity in adults is related to reduced life expectancy

The sedentary lifestyle associated with diets high in calories including not only carbohydrates, but also saturated fats, sugar and salt, has contributed to the increase in obesity, especially after the 80s.

• According to the WHO, in 2015 there were 600 million adults with obesity.

• In the USA, 9.2% of the population are morbidly obese (class III), (BMI > 40 kg/m2).

• In Brazil obesity affected 12.2% of the adult population in 2002-2003 and rose to 26.8% in 2020, according to IBGE

• 29.5% of women are obese — practically one in three — compared to 21.8 of men.

• Overweight, on the other hand, was found in 62.6% of women and 57.5% of men.

2. Consequences of obesity

Severe obesity (type III) is associated with a significant increase in morbidity and mortality. On the other hand, weight loss is associated with a reduction in morbidity associated with obesity.

These are pathological states aggravated by the presence of obesity and that are improved by its control, among the most frequent:

• HAS
• DM II
• Peripheral vascular insufficiency
• Cholelithiasis
• Arthropathies
• Coronary insufficiency
• Dyslipidemias
• Hepatic steatosis
• Sleep apnea
• Urinary incontinence
• GERD
• Physical limitation conditions and others.

The mortality of severe obese is 250% higher than non-severe.

Mortality from cancer, especially endometrial, is also increased for obese.

Metabolic syndrome

Metabolic Syndrome corresponds to a set of diseases whose basis is insulin resistance. When present, Metabolic Syndrome is related to a general mortality twice as high as in the normal population and cardiovascular mortality three times higher.

According to Brazilian Consensus, Metabolic Syndrome occurs when three of the five criteria below are present:

• Central obesity – waist circumference greater than 88 cm in women and 102 cm in men;
• Arterial Hypertension – systolic blood pressure ? 130 and/or diastolic blood pressure ? 85 mmHg;
• Altered glycemia (glycemia ? 110 mg/dl) or diagnosis of Diabetes;
• Triglycerides ? 150 mg/dl;
• HDL cholesterol ? 40 mg/dl in men and ? 50 mg/dl in women

* If BMI >30, the abdominal circumference does not need to be determined as central obesity is presumed.

3. Classification

The main index to measure and classify the degree of obesity is the BMI, due to its ease of application and correlation with morbidity and mortality risks.

Another useful measure, especially in Asians and patients with BMI between 25-35 is the measurement of abdominal circumference, since central obesity (associated with higher cardiometabolic risks) may not be captured in these patients.

• AC > 102 cm male sex
• AC > 88 cm female sex

Note: Asian population admits > 90 (male) and >80 (female)

4. Etiology

There are multiple factors that can contribute to the development of obesity

• Genetics: child with an obese parent has a 3-4 x higher risk of developing obesity. Two obese parents, the risk is 10 x higher
• Age: tendency to weight gain
• Habits and lifestyle: consumption of caloric, fatty foods, salt, sugar, sedentary lifestyle
• Medications: some antidepressants, antipsychotics, anticonvulsants, hypoglycemic agents (insulin and sulfonylureas), contraceptive hormones
• Comorbidities: hypothyroidism, cushing’s syndrome
• Intestinal microbiota: increasing evidence of the role of the microbiota

How to cite this article

Martins BC. Obesity: concept, consequences and classification. Gastropedia, vol I, 2023. Available at: gastropedia.com.br/cirurgia/obesidade/obesidade-conceito-consequencia-classificacao

Introduction

The incidence of neuroendocrine tumors of the pancreas is increasing, possibly due to more frequent imaging tests and the quality of these tests. However, their prevalence is fortunately still rare. This post from Therapeutic Endoscopy is intended to serve as a reference guide when we eventually come across one of these situations in our daily lives. If you want to know about duodenal neuroendocrine tumors check out this other article.

Important general concepts about neuroendocrine tumors of the gastrointestinal tract

The NETs correspond to a heterogeneous group of neoplasms that originate from neuroendocrine cells (enterochromaffin-like cells), with secretory characteristics.

All gastroenteropancreatic (GEP) NETs are potentially malignant and behavior and prognosis are correlated with histological types.

The NETs can be sporadic (90%) or associated with hereditary syndromes (10%), such as multiple endocrine neoplasia type 1 (MEN-1), SD von Hippel-Lindau, neurofibromatosis and tuberous sclerosis.

The NETs are mostly indolent, but can determine symptoms. Thus, they can be divided into functioning and non-functioning:

• Functioning: secretion of active hormones or neurotransmitters: serotonin, glucagon, insulin, somatostatin, gastrin, histamine, VIP or catecholamines. They can cause a variety of symptoms
• Non-functioning: they may not secrete any peptide/hormones or secrete non-active peptides or neurotransmitters, so as not to cause clinical manifestations.

Pancreatic neuroendocrine tumors (TNE-P)

The functioning TNEs of the pancreas are: insulinoma, gastrinoma, glucagonoma, vipoma and somatostatinoma.

Most TNE-Ps are malignant, except for insulinomas and TNE-NFs smaller than 2 cm.

Surgery is the only curative modality for sporadic TNE-P, and resection of the primary tumor in patients with localized, regional and even metastatic disease, can improve patient survival.

In general, functioning TNEs of the pancreas should be resected to control symptoms whenever possible. TNE-NF depends on size (see below).

Multiple pancreatic tumors are rare and should raise suspicion of MEN1.

NEXT WE WILL SEE THE MAIN CHARACTERISTICS OF EACH HISTOLOGICAL SUBTYPE

INSULINOMAS

• It is the most frequent TNE of the pancreatic islets.
• 90% are benign, but they are symptomatic even when small.
• About 10% are associated with MEN.
• They are hypervascularized and solitary lesions, often < 2 cm.
• Whipple’s triad:
  • hypoglycemia (< 50)
  • neuroglycopenic symptoms (blurred vision, weakness, fatigue, headache, drowsiness)
  • disappearance of symptoms with glucose replacement
• serum insulin > 6 IU/ml
• C-peptide > 0.2 mmol/l
• Pro-insulin > 5 IU/ml
• Positive prolonged fasting test (99% of cases)
• Learn more about insulinoma in this other article

GASTRINOMAS

• It is more common in the duodenum, but 30% of cases are in the pancreas
• They are the most frequent TNEs of the pancreas after insulinomas.
• They are associated with MEN 1 syndrome in 30%, and in these cases they present as small and multifocal lesions.
• They cause hypergastrinemia and Zollinger-Ellison syndrome.
• 60% are malignant.
• Treatment: surgical in sporadic cases (DPT).
• In MEN 1, there is controversy in the surgical indication, since gastrinemia may not be controlled even with DPT (tumors are usually multiple)

GLUCAGONOMAS

• Rare; most are sporadic.
• They are usually large and solitary, with a size between 3-7 cm occurring mainly in the tail of the pancreas.
• Symptoms: migratory necrolytic erythema (80%), DM, malnutrition, weight loss, thrombophlebitis, glossitis, angular cheilitis, anemia
• Slow growth and long survival
• Lymph node or hepatic metastasis occurs in 60-75% of cases.

VIPOMAS

• Extremely rare
• Like glucagonomas, located in the tail, large and solitary.
• Most are malignant and metastatic
• In 10% of cases it can be extra-pancreatic.
• Clinical picture related to VIP secretion (vasoactive intestinal peptide):
  • diarrhea (more than 3L liters per day) – rice washing water
  • Hydro-electrolyte disorders: hypokalemia, hypochloridria, metabolic acidosis
  • Blushing
• Excellent response to treatment with somatostatin analogues.

SOMATOSTATINOMAS

• It is the least common of all
• Somatostatin leads to inhibition of endocrine and exocrine secretion and affects intestinal motility.
• Solitary lesion, large, sporadic, mostly malignant and metastatic
• Clinical picture:
  • Diabetes (75%)
  • Gallstones (60%)
  • Steatorrhea (60%)
  • Weight loss

NON-FUNCTIONING PANCREATIC TNE

• 20% of all pancreatic TNEs.
• 50% are malignant.
• The main differential diagnosis is with adenocarcinoma

Well-differentiated TNE-NF smaller than 2 cm: two societies (ENETS and NCCN) suggest observation if it is well differentiated. However, the North American society NETS recommends observation in tumors smaller than 1 cm and individualized conduct, between 1-2 cm.

PANCREATIC TNE RELATED TO HEREDITARY SYNDROMES

• 10% of TNE-Ps are related to MEN-1
• Often multicentric,
• Usually affecting younger people.
• Usually of benign behavior, but they present malignant potential
• Gastrinoma 30-40%; Insulinoma 10%; TNE-NF 20-50%; others 2%
• Surgical treatment is controversial, because sometimes it does not control gastrinemia (multiple tumors)

The multiple endocrine neoplasia (MEN) syndromes comprise 3 genetically distinct familial diseases involving adenomatous hyperplasia and malignant tumors in several endocrine glands. They are autosomal dominant diseases.

MEN-1

• Autosomal dominant disease
• Predisposes to TU (3Ps): Parathyroid; Pituitary (pituitary); Pancreas,
• Usually of benign behavior, but they present malignant potential
• Gastrinoma 30-40%; Insulinoma 10%; TNE-NF 20-50%; others 2%
• Surgical treatment is controversial, because sometimes it does not control gastrinemia (multiple tumors)

MEN-2A:

• Medullary thyroid carcinoma,
• Pheochromocytoma,
• Hyperplasia or adenomas of the parathyroid glands (with consequent hyperparathyroidism).

MEN-2B:

• Medullary thyroid carcinoma,
• Pheochromocytoma
• Multiple mucous and intestinal neuromas

References:

1. Pathology, classification, and grading of neuroendocrine neoplasms arising in the digestive system – UpToDate ; 2021
2. Guidelines for the management of neuroendocrine tumours by the Brazilian gastrointestinal tumour group. ecancer 2017,11:716 DOI: 10.3332/ecancer.2017.716

How to cite this article:

Martins BC, de Moura DTH. Pancreatic neuroendocrine tumors. Gasstropedia. 2022; vol I. Available at: gastropedia.com.br/surgery/pancreatic-neuroendocrine-tumors