Mast cell sarcoma C96.-

Horny HP, 1986

Extremely rare, aggressive variant of mastocytosis, which is initially localized and later develops multicenter or leukemic. Horny described an initial subglottic tumour which later metastasised into the skin.

Apparently, mast cell sarcoma can occur at any age (Monnier J et al. 2016).

Oropharynx¸ Bone marrow, gastrointestinal tract, lymph nodes, liver, spleen

Initially, the mast cell sarcoma is characterized by a solid nodular tumor consisting of largely undifferentiated tumor cells that infiltrate the surrounding tissue. The tumour can also mimic other tumour formations. The D816V mutation, which is common in other mastocytoses, can only be detected in 21% of cases (Monnier J et al. 2016).

The mast cell sarcoma is capable of rapid metastasis (bone marrow, skin, gastrointestinal tract and other organs). The generalisation can then become clinically evident as mast cell leukaemia (significant mast cell proliferation > 10% of leukocytes). Mast cell mediator symptoms such as fever, flush, diarrhoea, tachycardia are found in only about 1/3 of cases.

Mast cell tryptase is often significantly elevated (Wagner N et al. 2018)

The mean survival time of the patients is less than 18 months (Monnier J et al. 2016)

1. Horny HP et al (1996) Mast cell sarcoma of the larynx. J Clin Pathol 39:596-602.
2. Monnier J et al (2016) Mast cell sarcoma: new cases and literature review. Oncotarget 7: 66299-66309.
3. Weiler CR et al (2014) Mast cell sarcoma: clinical management. Immunol Allergy Clin North Am 34:423-432.