Primary synovial sarcoma of the mediastinum: a poor prognosis in a 14-year-old girl.

Synovial sarcoma is a soft tissue sarcoma. Its occurrence as a primary mediastinal neoplasm is very rare. We report a case of mediastinal synovial sarcoma in a 14-year-old girl with exertive dyspnoea at presentation. On physical examination, there was a superior vena cava syndrome and café-au-lait spots. Chest X-ray revealed a white left hemithorax with mediastinal deviation to the opposite side. Thoraco-abdomino-pelvic scanner showed a voluminous posterior cervico-mediastinal tissue mass. Computed tomography (CT)-guided biopsy of the mediastino-pleural mass was performed. Histological examination reported fusocellular malignant mesenchymal proliferation. A complementary immunohistological study with a broad range of antibodies was performed with a high-grade single-phase spindle cell synovial sarcoma of the mediastinum. She presented a respiratory distress, did not respond to resuscitation, and died. Mediastinal synovial sarcoma is a rare tumour that is difficult to diagnose. Its slow progression and delay in diagnosis may lead to a fatal evolution.

The emergent use of Cardiopulmonary bypass and extracorporeal membrane oxygenator in a child with sickle cell disease.

The use of Cardiopulmonary bypass (CPB) and extracorporeal membrane oxygenator (ECMO) in patients suffering from Sickle cell disease (SCD) needs specific precautions. Whereas, no consensual protocols have been established to clarify therapeutic management. CASE REPORT A 7-year-old boy was admitted to the hospital for surgery of advanced endocarditis.  Major dyspnea, hemodynamic distress and fever were noted on physical examination. Biological tests exploring anaemia revealed Haemoglobin (Hb) S levels of 39.1%. Echocardiography showed important right heart cavities dilation with multiple aortic vegetations. The child was accepted for emergent surgery. Ten minutes after anesthetic induction, serious hemodynamic distress was established. The patient was put on normothermic CPB when he received four packed red-blood-cell. After surgery, he was placed on ECMO support for 2 days than he succumbed. CONCLUSION Urgent cardiac surgery in patients suffering from SCD poses a major therapeutic dilemma. Multiplying case reports and encouraging prospective studies are necessary to define the right place of cardio-pulmonary assistance in treatment protocols for better management.