Differentiating carcinoid syndrome from other diseases

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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

+ +/- +/- + + - + + + +
  • Valve thickening with retraction and reduction in the mobility of the tricuspid valve

Pathognomonic radiological sign of midgut NET.

Neuroendocrine tumor of lung[15][16][17][18] + + + + +
+ +/- +/- + + - + + Sensitive for detection of liver metastases if present + + - 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

Irritable Bowel Syndrome[19][20][21][22] +

Perioidic

- - - - - - - - - - - - - - - - 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[23][24][25] +/- +/- - - +/- - - +/- - * Abdominal mass - + 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[26][27][28][29] +/- - - - - - - - - - - - - -
  • Focal ulcerations and acute and chronic inflammation
Benign cutaneous flushing[30][31][32] - - + - - - - - - - - - - - - - - - - - - -
Systemic mastocytosis[33][34][35][36][37][38][39] + + + + - +/- +/- + - - - - - -
Asthma exacerbation[40][41][42][43][44][45] - - - + + + - - + -
  • Tachypnea
  • Prolonged expiratory phase of respiration (decreased I:E ratio)
  • Seated position with use of extended arms to support the upper chest (tripod position)
  • +/- Pulsus paradoxus
- - - -- - - - Chest X ray -
  • Loss of the normal pseudostratified structure of airway epithelium
  • Increase in the proportion of goblet cells
  • Fibrotic thickening of the sub-epithelial reticular basement membrane
  • Increased numbers of myofibroblasts
  • Increased vascularity
  • Increased airway smooth muscle mass
  • Increased extracellular matrix
Anaphylaxis[46][47][48][49][50] + -/+ + + + +/- - + + - - - - - - - - - - History of exposure to insect stings,food alllergy,rubber latex,food additives,,allergy to medications,physical factors such s excercise and cold
Histaminergic Angioedema[51][52][53][54][55][56] +/- +/- + + + + - + + - - - - - - - - - -
  • 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[57][58][59][60][61] - +/- +/- +/- - - - - - - - - - - - -

For metastasis

-

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