Mast cell sarcoma

A rare type of malignant tumor derived from mast cells

Classification
External resources

Overview

Micrograph of mast cell sarcoma showing atypical mast cells.

Mast cell sarcoma is a rare and aggressive form of sarcoma that originates from mast cells. Mast cells are a type of white blood cell that play a crucial role in the body's immune system, particularly in allergic reactions and inflammation. Unlike mastocytosis, which is a condition characterized by an abnormal accumulation of mast cells, mast cell sarcoma is a malignant tumor that can rapidly progress and metastasize.

Pathophysiology

Mast cell sarcoma arises from the neoplastic transformation of mast cells. These cells are typically found in connective tissue, especially in the skin, lungs, and digestive tract. In mast cell sarcoma, the mast cells exhibit atypical morphology and increased proliferation. The exact molecular mechanisms leading to the development of mast cell sarcoma are not fully understood, but mutations in the KIT gene are often implicated.

Clinical Presentation

Patients with mast cell sarcoma may present with a variety of symptoms depending on the location of the tumor. Common symptoms include:

• Localized pain or swelling
• Skin lesions
• Gastrointestinal symptoms such as nausea and vomiting
• Systemic symptoms like fever and weight loss

Diagnosis

Histological examination is crucial for diagnosis.

The diagnosis of mast cell sarcoma is typically made through a combination of histological examination, immunohistochemistry, and genetic testing. A biopsy of the tumor is performed to examine the morphology of the mast cells and to identify any characteristic markers such as CD117 (c-KIT) and tryptase.

Treatment

Treatment options for mast cell sarcoma are limited due to its rarity and aggressive nature. The primary treatment is often surgical resection of the tumor. Chemotherapy and radiation therapy may be used as adjunctive treatments. Targeted therapies, such as tyrosine kinase inhibitors, are being explored in clinical trials.

Prognosis

The prognosis for patients with mast cell sarcoma is generally poor due to the aggressive behavior of the tumor and its tendency to metastasize. Early detection and treatment are crucial for improving outcomes.

Related pages

• Mastocytosis
• Sarcoma
• Immune system
• Allergic reaction