Differentiating carcinoid syndrome from other diseases: Difference between revisions

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* [[CRP]]  may be high
* [[CRP]]  may be high
* pANCA and ASCA
* [[p-ANCA]] and [[Anti saccharomyces cerevisiae antibodies|ASCA]]
*  
*  
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* Activating mutations of ''KIT''  
* Activating [[mutations]] of ''[[KIT]]''  
* Serum Tryptase leevs
* Serum [[Tryptase]] levels
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* Basal serum calcitonin concentrations  
* Basal serum [[calcitonin]] concentrations  
* Carcinoembryonic antigen (CEA) concentration
* [[Carcinoembryonic antigen peptide-1|Carcinoembryonic antigen (CEA) concentration]]
* Thyroid function tests: normal
* [[Thyroid function tests]]: normal
* germline ''RET'' testing
* [[Germline]] ''[[RET gene|RET]]'' testing
* Serum calcium
* [[Calcium|Serum calcium]]
* Plasma fractionated metanephrines
* Plasma fractionated [[Metanephrine|metanephrines]]
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Revision as of 15:25, 2 May 2019

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]

Overview

Carcinoid syndrome must be differentiated from systemic mastocytosis, medullary thyroid carcinoma, irritable bowel syndrome, malignant neoplasms of the small intestine, benign cutaneous flushing, and recurrent idiopathic anaphylaxis.

Differentiating Carcinoid Syndrome from other Diseases

Carcinoid syndrome must be differentiated from:[1]

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging Histopathology
Abdominal pain Diarrhea Flushing Dyspnea Palpitations Other symptoms Wheezing Telangiectasia Hypotension Tachycardia Systolic murmur of tricuspid regurgitation Other physical findings Urinary 5-hydroxyindoleacetic acid (5-HIAA) Serum Chromogranin A (CgA) Other markers Abdominal computed tomography (CT) Abdominal MRI Somatostatin receptor scintigraphy [SRS], or Octreoscan Metaiodobenzylguanidine (MIBG) scintigraphy Other diagnostic studies
  • Transthoracic echocardiography
Carcinoid Syndrome[2][3][4][5][6][7][8][9][10] Neuroendocrine tumor of midgut [11][12][13][14] +

Mild

+ + + +

Dermatitis

Diarrhea

Dementia

Metastatic tumors in the liver: Right upper quadrant pain, hepatomegaly, and early satiety

+ +/- +/- + + - + +
  • Neuroendocrine tumor of midgut are difficult to identify on CT because of their small size.
  • Findinga mass-like process with soft tissue "spokes" radiating into the mesenteric fat toward the small bowel causing retraction.
  • Liver metastases
  • Sensitive for detection of liver metastases
+
  • Localization of carcinoid tumor
+
  • 68-Ga DOTATATE PET scan
  • Positron emission tomography-computed tomography (PET-CT) using 18-fluoro-dihydroxyphenylalanine
  • Ki-67 labeling index
  • Endoscopy for metastatic Neuroendocrine tumour with an unknown primary site.
  • Valve thickening with retraction and reduction in the mobility of the tricuspid valve
  • Somatostatin receptor scintigraphy [SRS], or Octreoscan
  • Biopsy and histopathology
  • Mesenteric fibrosis

Pathognomonic radiological sign of midgut NET.

Neuroendocrine tumor of lung[15][16][17][18] + + + + +
+ +/- +/- + + - + +
  • Atypical neuroendocrine tumors have a greater tendency to metastasize to liver,hypervascular, and becomies isodense relative to the liver parenchyma after contrast administration.
Sensitive for detection of liver metastases if present + +
  • Chest X ray: round or oval opacities from 2-5cm with sharp and notched margins
  • Chest CT : Hilar or Perihilar Masses, Endobronchial Nodules,Related to Bronchial Obstruction:peripheral atelectasis and postobstructive pneumoniaPeripheral Nodules.
  • Pulmonary funcation test
  • Bronchoscopy
  • 68-Ga DOTATATE PET scan
  • Fluorodeoxyglucose PET scans for atypical lung NETs .
- Typical low-grade:bland cells containing regular round nuclei with finely dispersed chromatin and inconspicuous small nucleoli.Mitotic figures are scarce and necrosis is absent.

Intermediate-grade atypical: presence of Neuroendocrine morphology and either necrosis or 2 to 10 mitoses per 10 HPF

  • Biopsy and histopathology
Lung neuroendocrine tumor can be a/s with:
  • Cushing syndrome
  • Acromegaly
  • SIADH Complications:
  • Recuurent Pneiumonia
Irritable Bowel Syndrome +

Perioidic

- - - - - - - - - - - - - -
  • Bristol stool form scale should to record stool consistency
  • Abdominal radiograph to assess for stool accumulation and determine the severity.
  • Age-appropriate colorectal cancer screening in all patients
- - Rome IV criteria
  • recurrent abdominal pain, at least 1day/week in the last 3 months, a/s with 2 or more of the following criteria:

•Related to defecation

•Associated with a change in stool frequency

•Associated with a change in stool form (appearance)

Malignant neoplasms of small intestine +/- +/- - - +/- - - - +/- - - + Abdominal CT scan may be diagnostic of small intestine cancer. Findings on CT scan suggestive of small intestine cancer include intrinsic mass with a short segment of bowel wall thickening MRI and MRI enteroscopy are other advance modalities to diagnose and stage small intestinal cancers - - Enteroscopy, capsule endoscopy and double balloon enteroscopy Biopsy and histopathology
Crohn disease[19] +/- - - - - - - - - - -
  • CT enterography :Small bowel inflammation by displaying mural hyperenhancement and thickening; engorged vasa recta; and perienteric inflammatory changes.
- -
  • CBC
  • Blood chemistry including electrolytes*
  • Renal function tests
  • liver enzymes
  • blood glucose
  • ESR
  • CRP
  • Serum iron
  • Vitamin D & vitamin B12 levels
  • stool D/R and culture for ova and parasites,
  • C. difficile toxin
-
  • Focal ulcerations and acute and chronic inflammation
  • Granulomas
  • Colonoscopy:focal ulcerations adjacent to areas of normal appearing mucosa along with polypoid mucosal,skip lesions,pseudopolyps,
Benign cutaneous flushing - - + - - - - - - - - - - - - - - - - - - -
Systemic mastocytosis + + + + - +/- +/- + - - - - - -
Asthma exacerbation - - - + + + - - + - - - Chest X ray
Anaphylaxis + -/+ + + + +/- - + + - - - - - -
  • plasma tryptase
  • Plasma histamine levels
  • Take proper clinical history and medication history specicially beta blockrs,ACE-inhibots,opioids
  • Skin testing with allergen extracts
  • enzyme-linked immunosorbent assays (ELISAs) for quantification of allergen-specific IgE levels
- - History of exposure to insect stings,food alllergy,rubber latex,food additives,,allergy to medications,physical factors such s excercise and cold
Histaminergic Angioedema +/- +/- + + + + - + + - - - -
  • Bowel wall edema
  • Circumferential thickening of the small bowel wall with ascites or incomplete obstruction
- - -
  • CBC with differential,
  • Basic chemistry panel
  • Liver function tests
  • C-reactive protein
  • Erythrocyte sedimentation rate
  • Levels of the complement protein C4
  • Serum total tryptase
  • Allergen-specific IgE immunoassays
- - -
  • Take proper clinical history of previous similar episodes
  • Medication history
  • Any allergy to insects stings , foods or any ingestion within previous 24 hours
Medullary Thyroid Carcinoma - +/- +/- +/- - - - - - - - - - - -
  • Ultrasonography of the neck

For metastasis

  • Chest computed tomography
  • Neck CT
  • Three-phase contrast-enhanced liver CT
  • Axial MRI
  • Bone scintigraphy.
-
  • immunohistochemical staining for calcitonin
  • Spindle-shaped and frequently pleomorphic cells without follicle development
  • Fine-needle aspiration (FNA) biopsy
  • TNM staging
  • American Thyroid Association (ATA) Guidelines for Management and evaluation of Medullary Thyroid Cancer

References

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  2. Rubin de Celis Ferrari AC, Glasberg J, Riechelmann RP (August 2018). "Carcinoid syndrome: update on the pathophysiology and treatment". Clinics (Sao Paulo). 73 (suppl 1): e490s. doi:10.6061/clinics/2018/e490s. PMC 6096975. PMID 30133565.
  3. Hegyi J, Schwartz RA, Hegyi V (January 2004). "Pellagra: dermatitis, dementia, and diarrhea". Int. J. Dermatol. 43 (1): 1–5. PMID 14693013.
  4. Savelli G, Lucignani G, Seregni E, Marchianò A, Serafini G, Aliberti G, Villano C, Maccauro M, Bombardieri E (May 2004). "Feasibility of somatostatin receptor scintigraphy in the detection of occult primary gastro-entero-pancreatic (GEP) neuroendocrine tumours". Nucl Med Commun. 25 (5): 445–9. PMID 15100502.
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  6. Bora, ManashKumar; Vithiavathi, S (2012). "Primary bronchial carcinoid: A rare differential diagnosis of pulmonary koch in young adult patient". Lung India. 29 (1): 59. doi:10.4103/0970-2113.92366. ISSN 0970-2113.
  7. Yazıcıoğlu A, Yekeler E, Bıcakcıoğlu P, Ozaydın E, Karaoğlanoğlu N (December 2012). "Synchronous bilateral multiple typical pulmonary carcinoid tumors: a unique case with 10 typical carcinoids". Balkan Med J. 29 (4): 450–2. doi:10.5152/balkanmedj.2012.081. PMC 4115868. PMID 25207053.
  8. Krausz Y, Keidar Z, Kogan I, Even-Sapir E, Bar-Shalom R, Engel A, Rubinstein R, Sachs J, Bocher M, Agranovicz S, Chisin R, Israel O (November 2003). "SPECT/CT hybrid imaging with 111In-pentetreotide in assessment of neuroendocrine tumours". Clin. Endocrinol. (Oxf). 59 (5): 565–73. PMID 14616879.
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  11. Sjöblom SM (September 1988). "Clinical presentation and prognosis of gastrointestinal carcinoid tumours". Scand. J. Gastroenterol. 23 (7): 779–87. PMID 3227292.
  12. Ganeshan D, Bhosale P, Yang T, Kundra V (October 2013). "Imaging features of carcinoid tumors of the gastrointestinal tract". AJR Am J Roentgenol. 201 (4): 773–86. doi:10.2214/AJR.12.9758. PMID 24059366.
  13. Signs and symptoms of carcinoid syndrome. National Cancer Institute. http://www.cancer.gov/types/gi-carcinoid-tumors/patient/gi-carcinoid-treatment-pdq
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  15. Gustafsson BI, Kidd M, Chan A, Malfertheiner MV, Modlin IM (July 2008). "Bronchopulmonary neuroendocrine tumors". Cancer. 113 (1): 5–21. doi:10.1002/cncr.23542. PMID 18473355.
  16. Jeung, Mi-Young; Gasser, Bernard; Gangi, Afshin; Charneau, Dominique; Ducroq, Xavier; Kessler, Romain; Quoix, Elisabeth; Roy, Catherine (2002). "Bronchial Carcinoid Tumors of the Thorax: Spectrum of Radiologic Findings". RadioGraphics. 22 (2): 351–365. doi:10.1148/radiographics.22.2.g02mr01351. ISSN 0271-5333.
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