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]

On the basis [symptom 1], [symptom 2], and [symptom 3], [disease name] must be differentiated from [disease 1], [disease 2], [disease 3], [disease 4], [disease 5], and [disease 6].

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) MRI Somatostatin receptor scintigraphy [SRS], or Octreoscan Metaiodobenzylguanidine (MIBG) scintigraphy Other diagnostic studies
  • Transthoracic echocardiography
Carcinoid Syndrome[2][3][4][5] Midgut tumours +

Mild

+
  • Intermittent
  • Secretory type
+ + +
  • Pellagra

Dermatitis

Diarrhea

Dementia

+ + + + + +
  • NT-proBNP
    • Screening of carcinoid heart disease
    • Blood Serotonin levels
+
  • Localization of carcinoid tumor
  • Positron emission tomography-computed tomography (PET-CT) using 18-fluoro-dihydroxyphenylalanine
  • Ki-67 labeling index
  • Valve thickening with retraction and reduction in the mobility of the tricuspid valve
  • Somatostatin receptor scintigraphy [SRS], or Octreoscan
  • Mesenteric fibrosis

Pathognomonic radiological sign of midgut NET.

Bronchial carcinoid
Irritable Bowel Syndrome +

Perioidic

  • Intermittent
  • Chronic history of diarhea alternating with constipation
- - -
  • Associated with other conditions
  • Fibromyalgia
  • Chronic fatigue syndrome
  • Gastroesophageal reflux disease,
  • Functional dyspepsia
  • Non-cardiac chest pain,
  • Major depression
  • ,Anxiety
- - - - - - - - - - -
  • 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 +/- +/- - - +/-
  • Constipation
  • Fatigue
  • Early satiety
- - - +/- -
  • Abdominal mas
  • Ascities
- + 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
  • Right lower quadrant pain
+/- - - -
  • oral lesions
  • Perianal lesions
- -
  • CRP may be high
Endoscopy
Benign cutaneous flushing
Systemic mastocytosis + + + + -
  • Maculopapular rash
  • Pruritus
+/- +/- + - -
  • Diffuse musculoskeletal pain
  • Neuropsychiatric symptoms
- -
  • Activating mutations of KIT
  • Serum Tryptase leevs
- -
Recurrent idiopathic anaphylaxis
Asthma exacerbation - - - + + + - - + - - - Chest X ray
Acute Urticaria
Anaphylaxis
Angioedema
Drugs causing flushing
  • Nicotinic acid
  • Levodopa
  • Bromocriptine
  • Diltiazem
Medullary Thyroid Carcinoma

References

  1. Metcalfe DD (2000). "Differential diagnosis of the patient with unexplained flushing/anaphylaxis". Allergy Asthma Proc. 21 (1): 21–4. PMID 10748948.
  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.
  5. 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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