Malignant pleural tumor in a child mimicking a mesothelioma

Malignant tumors of pleural origin are rare in childhood and difficult to diagnose. We report a case in a white 11 1/2-year-old girl who presented with a bulky tumour in the basal left lung field. There was an area of osteolysis at the ventral extremity of the 9th left rib allowing excision of the tumor by lateral thoracotomy. Macroscopically the operation was satisfactory. Despite two complementary excisions, chemotherapy with Vin-Caepi and consolidation chemotherapy followed by bone-marrow autograft, left pleural metastases appeared two years later. A second operation, a new course of chemotherapy with stage IV SIOP MMT, followed by a second bone-marrow graft and mediastinal radio-therapy, resulted in remission. At the time of this report, after three and a half years of evolution, the child's condition is satisfactory and she leads a normal life without treatment. A probable diagnosis of pleural mesothelioma was arrived at by elimination. This kind of tumour, without specific markers, is difficult to distinguish from small-round-cell tumors. Evolution is unpredictable and often rapidly fatal. Although these tumors generally have a weak response to chemo- and radiotherapy, we think that close follow-up and active therapy, rapidly initiated at the slightest sign, can improve the quality of life of the patients and increase their survival.