SMARCA4-deficient thoracic sarcoma (SMARCA4-DTS) is a recently identified aggressive subtype of sarcoma. We present the case of a 44-year-old man who underwent a surgery for a perforated small intestine. Compued tomography scan revealed a tissular mediastino–pulmonary mass.Histopathological examination of the intestinal mass shown a malignant tumour with a typical epithelioid and rhabdoid cells, numerous mitoses and large necrosis. A large panel of immunohistochemistry revealed loss of SMARCA4 and SMARCA2 and allowed the diagnosis of SMARCA4-DTS. It is important to consider SMARCA4-deficient thoracic sarcoma in the differential diagnosis of tumours showing suggestive morphologic features in patients of all ages, especially in the case of metastasis associated with thoracic mass.

BackgroundCardiac tumors are a very rare entity. Leiomyosarcoma represents less than 1% of cases.Case presentationa 51-year-old woman diagnosed with primary left atrium leiomyosarcoma. She was treated by optimal surgery and adjuvant chemotherapy. She is still alive after a follow-up of 24 months without evidence of local or distant recurrence.ConclusionsCardiac leiomyosarcoma is a rare tumor with a dismal prognosis. Surgery is the mainstay of treatment. Adjuvant treatment is still controversial.

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