Mast cell tumor medical therapy

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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

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.

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. However, Nifedipine has never been approved by the FDA for treatment of mastocytosis. 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.