Mast cell tumor medical therapy

Mast cell tumor Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Mast Cell Tumor from other Diseases
Epidemiology and Demographics
Risk Factors
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Study of Choice
Diagnostic criteria
Staging
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X-ray
Echocardiography or Ultrasound
CT Scan
MRI
Biopsy
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Mast cell tumor medical therapy On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Mast cell tumor medical therapy All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Mast cell tumor medical therapy
CDC on Mast cell tumor medical therapy
Mast cell tumor medical therapy in the news
Blogs on Mast cell tumor medical therapy
Directions to Hospitals Treating Mast cell tumor
Risk calculators and risk factors for Mast cell tumor medical therapy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Medical Therapy

The treatment of mast cell tumor is mainly focused on avoidance of triggering factors (e.g. physical stimuli such as heat or cold, alcohol, drugs such as aspirin and other NSAIDS) and symptomatic therapy. Commonly used medications for symptomatic relief include:

Medication	Indications
H1 antihistamine	Pruritus Flushing Anaphylaxis
H2 antihistamine	Pruritus Anaphylaxis Diarrhea Nausea Vomiting Abdominal discomfort
Mast cell stabilizers	Abdominal cramping Malabsorption
Proton pump inhibitors	Peptic ulcers
Epinephrine	Anaphylaxis
Steroids	Anaphylaxis Malabsorption

Antihistamines

• Antihistamines block receptors targeted by histamine released from mast cells. Both H1 and H2 blockers may be helpful.
• Leukotriene antagonists block receptors targeted by leukotrienes released from mast cells.
• Antihistamines frequently treat itching and other skin complaints.
• Certain antihistamines work specifically against ulcers

Mast cell stabilizers

• Mast cell stabilizers help prevent mast cells from releasing their chemical contents.
• Cromolyn sodium oral solution (Gastrocrom® / Cromoglicate) is the only medicine specifically approved by the U.S. FDA for the treatment of mastocytosis.
• Ketotifen is available in Canada and Europe, but is only available in the U.S. as ophthalmic drops (Zaditor®).
• Cromolyn sodium may help reduce cramping in the abdomen.

Proton pump inhibitors

• Proton pump inhibitors help reduce production of gastric acid, which is often increased in patients with mastocytosis.
• Excess gastric acid can harm the stomach, esophagus, and small intestine.

Epinephrine

• Epinephrine constricts blood vessels and opens airways to maintain adequate circulation and ventilation when excessive mast cell degranulation has caused anaphylaxis.
• Epinephrine treats symptom flares which occur with shock, referred to as "anaphylaxis".

Albuterol

• Albuterol and other beta-2 agonists open airways that can constrict in the presence of histamine.

Steroid

• Corticosteroids can be used topically, inhaled, or systemically to reduce inflammation associated with mastocytosis.
• Steroids treat malabsorption or impaired ability to take in nutrients.

Antidepressants

• Antidepressants are an important and often overlooked tool in the treatment of mastocytosis.
• The stress and physical discomfort of any chronic disease may increase the likelihood of a patient developing depression.
• Depression and other neurological symptoms have been noted in mastocytosis. Some antidepressants such as doxepin are themselves potent antihistamines and can help relieve physical as well as cognitive symptoms.

Calcium channel blocker

• Dihydropyridines are calcium channel blockers that are sometimes used to treat high blood pressure.
• At least one clinical study suggested that nifedipine, one of the dihydropyridines, may reduce mast cell degranulation in patients that exhibit urticaria pigmentosa.
• However, Nifedipine has never been approved by the FDA for treatment of mastocytosis.

A 1984 study by Fairly et al. included a patient with symptomatic urticaria pigmentosa who responded to nifedipine at dose of 10 mg po tid. . In aggressive forms of systemic mastocytosis, treatments such as interferon alpha, cladribin, and imatinib mesylate should to be considered. Imatinib seems to be more effective in patients without the D816V C-kit mutation.

Chemotherapy

• In rare cases in which mastocytosis is cancerous or associated with a blood disorder, the patient may have to use steroids and/or chemotherapy.
• The novel agent imatinib (Glivec® or Gleevec®) has been found to be effective in certain types of mastocytosis.
• Recent literature shows that C-Kit (D816V) lends some resistance to imatinib and sorafenib but these cells are still sensitive to nilotinib, dasatinib and rapamycin. Cladribine and interferon have also been found to be effective.

Cutaneous Mastocytosis

• Usually, Isolated cutaneous mastocytosis needs no treatment or action to be taken.
• Cutaneous mastocytosis with cosmetic complaints should be treated by a topical therapy only in children older than 2 years.
• When cutaneous mastocytosis is confined to about 10% of body surface, occlusive dressing is used.
• When the extension is larger, (more than 10%) the occlusive dressing is optional, and usually the corticosteroid cream is diluted.
• In cutaneous mastocytosis with complaints of itch, redness, and swelling, avoiding foods with a possible triggering role has to be suggested.

Diffuse cutaneous mastocytosis

• Diffuse cutaneous mastocytosis is self-limiting, and the need for a treatment is questionable.
• If diffuse cutaneous mastocytosis causes significant distress, leads to frequent hospitalizations, or is life-threatening, treatment with imatinib is a good option.
• Imatinib mesylate, a type II kinase inhibitor, inhibits cell proliferation and induces apoptosis by binding the adenosine triphosphate pocket and the adjacent site of the kinase.
• The nonselectivity of imatinib also explains side effects such as cardiomyopathy and reduced growth velocity, the latter being a relevant side effect in children.
• A dose of 200 mg/m2/day have been used in children, and reducing the dosage as soon as sustained healing of the skin lesions is noted is sufficient to obtain remission.