RESUMO
The term "inflammatory myofibrohistiocytic proliferation" (IMP) has been proposed to replace the conventional designations of plasma cell granuloma and inflammatory pseudotumor. Three cases of extrapulmonary IMP in children are reported, including an intracerebral lesion which has been formerly undescribed. In children, IMP may be associated with microcytic hypochromic anemia, hypergammaglobulinemia, and high erythrocyte sedimentation rate. In this clinical setting, differentiation of a rapidly growing but benign IMP from a bona fide sarcoma is of paramount importance.
Assuntos
Fibroma/diagnóstico , Granuloma de Células Plasmáticas/diagnóstico , Granuloma/diagnóstico , Sarcoma/diagnóstico , Neoplasias Abdominais/patologia , Adolescente , Neoplasias Encefálicas/patologia , Pré-Escolar , Colágeno/análise , Citoplasma/ultraestrutura , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Masculino , Terminologia como Assunto , Neoplasias Torácicas/patologia , Tomografia Computadorizada por Raios XRESUMO
An 8-year-old girl presented with cutaneous lymphoma without bone marrow or visceral involvement. The tumor cells displayed convoluted and invaginated euchromatic nuclei. The immunophenotype of these cells was non-T/non-B (Ia+, CALLA-, SIg-, TdT-, E-, Thy-). The skin lesions regressed promptly with chemotherapy including cyclophosphamide, vincristine, methotrexate, doxorubicin, and cytosine arabinoside. Six months after the completion of chemotherapy (18 months postdiagnosis), the patient had a relapse of the skin lesions with concurrent bone marrow involvement. The cutaneous infiltrate at relapse was morphologically and immunophenotypically similar to that at the onset of illness. However, the bone marrow infiltrate, although morphologically similar to the cutaneous tumor, had an immunophenotype consistent with T-cells (Ia+, CALLA-, SIg-, E-, TdT+, Thy+, OKT4+, OKT8+). As in adults, primary cutaneous non-T/non-B lymphomas in children may be derived from T-cells or their precursors.
Assuntos
Leucemia/complicações , Linfoma/complicações , Neoplasias Primárias Múltiplas , Neoplasias Cutâneas/complicações , Linfócitos T , Doença Aguda , Criança , Feminino , Humanos , Leucemia/patologia , Linfoma/patologia , Microscopia Eletrônica , Recidiva Local de Neoplasia , Neoplasias Cutâneas/patologiaRESUMO
Fourteen children with mediastinal lymphoblastic lymphoma (MLL) were treated with the six-drug APO protocol. This regimen includes aggressive intermittent chemotherapy and prophylactic central nervous system therapy. Mediastinal irradiation was given only for emergency relief of mediastinal compression (two children) or for incomplete resolution of mediastinal widening with chemotherapy (one child). All 14 patients achieved complete remission; only 1 has relapsed. Toxicities were not severe. APO is effective therapy for children with MLL. APO should receive further trials in children and adults with T-cell lymphoblastic lymphomas.