Mucormycosis has emerged as an increasingly important infection in oncology centres with high mortality, especially in severely immunocompromised patients. We carried out a retrospective study of 11 children with mucormycosis treated in seven French oncology-haematology paediatric wards during the period from 1991 to 2011. Lichtheimia corymbifera and Mucor spp. were the predominant pathogens. Treatment regimens included antifungal therapy, reversal of underlying predisposing risk factors and surgical debridement. Although mucormycosis is associated with high mortality, this infection could be cured in eight of our cases of severely immunocompromised paediatric cancer patients.

Fig. 1. Serum MTX and creatinine concentrations after MTX infusion. Ã Methotrexate.

Bacteraemia and endocarditis are the most frequently reported clinical infections due to Abiotrophia defectiva species. This species has been rarely implicated in infections in neutropenic patients. We report a rare case of long-term venous catheter-related infection caused by A. defectiva that occurred in a febrile child who had neutropenia and Langerhans' cell histiocytosis.

We report the case of a very high risk metastatic rhabdomyosarcoma teenager treated with an induction therapy using only liposomal doxorubicin (Myocet ® ) because of his extremely bad health condition. A near complete remission was obtained after 2 courses. Then he received conventional chemotherapy according to the EpSSG protocol, radiotherapy and a reduced-intensity conditioning regimen followed by pheno-identical allogeneic stem cell transplantation. He is in continuous complete remission with a follow-up of nine years.

Introduction: Kasabach-Merrit syndrome (KMS) is characterized by the association of a vascular tumor, thrombocytopenia and potentially life-threatening coagulopathy. Observation: A 22-month-old child was referred to our center for incidental discovery of thrombocytopenia in a context of recurrent throat infections. Clinical examination showed petechial purpura, subicterus, no tumoral syndrome and good overall condition. His biological parameters showed regenerative hemolytic anemia (10.2 g/dL), thrombocytopenia (32 G/L), no schistocytes, moderate fibrinopenia and positive D-dimer test. The results of bone marrow analysis and autoimmune tests were normal. No treatment was undertaken given the absence of diagnosis and complication. The child was regularly followed-up in consultation. Complementary investigations ruled out Wilson's disease, thrombotic thrombocytopenic purpura and constitutional red blood cell disorders. The main hypothesis remained a thrombotic microangiopathy despite absence of schistocytes on repeated blood tests. Two months later, he was rehospitalized for acute clinical and biological worsening with splenomegaly and severe consumption coagulopathy leading to suspect a myeloproliferative syndrome, finally refuted. Steroids were started due to worsening hemorrhagic symptoms, daily transfusion requirements and increasing splenomegaly. Due to worsening health status and risk of threatening rupture, splenectomy was performed and histopathological analysis revealed a vascular lesion infiltrating the whole spleen, compatible with a littoral cell angioma. The child showed rapid clinical improvement and complete hematological normalization within 48 hours post-splenectomy. Conclusion: Littoral cell angioma is a very rare and benign vascular lesion which may be complicated by a lifethreatening KMS. This diagnosis should be suspected in patients presenting with splenomegaly and features of thrombotic microangiopathy.

Renal medullary carcinoma (RMC) is a very rare disease that is almost exclusive to young black patients with sickle cell trait. Most patients present with metastatic disease at diagnosis and have a grim prognosis. Histologic diagnosis of these tumors can be difficult in the presence of rhabdoid elements and loss of INI1 expression as observed in our case.We describe the first known reported case of a white male adolescent with sickle cell trait, who was treated for a metastatic RMC with reticular pattern of rhabdoid-like cells and absence of INI1 expression, by the European Soft Tissue Sarcoma Study Group (EpSSG) protocol designed for rhabdoid tumors and who achieved a transitory complete response. Although complete remission was achieved, overall survival was not improved.