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Primary cardiac sarcomas are rare tumors with poor prognosis. Intimal sarcoma, a mesenchymal malignant tumor described mainly in the great vessels, may rarely involve the heart. Herein we describe the case of a 70-years-old female who was found to have a left atrial mass during an investigation of a new onset dyspnea. The patient underwent surgery and the resected mass was found to be an intimal sarcoma. The objectives of this report were to describe a case of this rare disease entity and to discuss its pathological and molecular findings based on relevant literature. ispartof: ANNALES DE PATHOLOGIE vol:39 issue:6 pages:440-443 ispartof: location:France status: published

Primary angiosarcoma of the breast is a rare but aggressive disease with a poor 5-year survival. An early and precise diagnosis has to be made in order to improve prognosis. A large vascular breast mass should therefore always be considered as an angiosarcoma until proven otherwise. Referral to tertiary care centre and multidisciplinary management are strongly recommended. There is no standard therapeutic approach but surgery remains the mainstay of angiosarcoma treatment. Patients need a close follow-up because recurrence is frequent and often precocious. We report the rare case of a 16-year-old patient presenting a primary angiosarcoma of the breast with MYC amplification. Such cases should always be reported, as MYC amplification could in a close future be routinely used as a marker of disease aggressiveness and possibly as a therapeutic target.

Desmoid tumors or aggressive fibromatosis of the breast, are a rare entity, representing less than 0.2% of all primary breast tumors. The clinical presentation and evolution can mimic a malignant carcinoma, with the notable difference that a desmoid tumor cannot generate distant metastases. The aim of the treatment is to achieve local control of this tumor, which can be highly aggressive by deeply infiltrating surrounding structures, and frequently reoccurs after resection. Both the tumor and its treatment may cause significant morbidity, causing a real therapeutic challenge. We here report the case of a 63-year-old woman who underwent a tumorectomy for left breast cancer and developed six years later a large desmoid tumor in the same breast. First medically treated with selective estrogen receptor modulators and non-steroidal anti-inflammatory drugs, it progressed to an ulcerative exophytic and necrotic tumor requiring surgery. To our knowledge, this is the first description of such a spectacular evolution in the literature. After reporting on this uncommon evolution of a rare disease, we will provide a short review of current treatment options.