Following treatment with chemotherapy and radiotherapy, patients with thoracic lymphomas may demonstrate benign residual mediastinal masses, composed of inflammatory, fibrous or necrotic tissue. ...Because of the potential risk of viable tumour cells within the mass, histological verification of the nature of these masses may be requested.
To study the outcome of thoracic lymphomas in children in order to optimise the radiological follow-up strategy of residual mediastinal masses (RMM).
A retrospective study of 39 children 24 with Hodgkin's disease (HD), 10 with non-Hodgkin's lymphoma (NHL), and 5 with anaplastic lymphoma (AL). The results of chest X-rays (CXR) and thoracic CT performed at the time of re-assessment were compared with the histology of the residual masses (n = 11) or the clinical course (n = 28).
At the time of re-evaluation, 16/39 patients had residual mediastinal enlargement (RME) on CXR, and 18/39 patients had RMM on CT. Good concordance was observed between the two imaging modalities (K = 0.69). Two children with a RMM died from extra-mediastinal progression. Two children with NHL had active residual mediastinal lesions but neither had RMM. Sixteen cases of RMM were observed in the remaining 35 children and 9 of these masses were histologically verified as benign. A favourable course was observed in these 35 cases.
RMM are frequent and generally benign. They are well shown on CXR and have a non-specific appearance on CT. Except when required by a treatment protocol, they could be submitted to further radiological follow-up before contemplating surgical verification.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OBVAL, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
OBJECTIVE: Skin involvement in children with acute monocytic leukemia or CD30-positive anaplastic large-cell lymphoma is well-known. In contrast, very little is known about the malignant cutaneous ...infiltrates in children with acute lymphoblatic leukemia (ALL) or lymphoblastic lymphoma (LBL). This study was designed to determine the frequency of these specific lesions in childhood ALL or LBL and the characteristics of such patients. DESIGN: We studied the clinical and biological findings of children with cutaneous involvement at initial diagnosis of ALL or LBL enrolled between August 1989 and March 1995 in the multicentric trial 58881 of the Children's Leukemia Cooperative Group of the European Organization of Research and Treatment of Cancer (EORTC). RESULTS: Among the 1359 children enrolled in the multicenter trial EORTC 58881, 24 presented with skin involvement at diagnosis. ALL was diagnosed in 15 patients and LBL in 9. In 15 cases, skin lesions were observed within a median time of 6 weeks (range, a few days to 8 months) before the diagnosis of the hematologic disease. Twenty-one children had at least one skin lesion located on the head. Diffuse cutaneous lesions were observed in 7 infants with high-risk ALL. Seventeen of the 24 children remain in the first complete remission (median follow-up of 3 years; range 2 months to 5 years) and 3 are in the second remission with a follow-up of 14 to 24 months. CONCLUSION: The present study demonstrates that cutaneous involvement can be an early manifestation of ALL or LBL. Cutaneous leukemic infiltrates can be observed in children with standard risk as well as in high-risk ALL. Cutaneous involvement in children with LBL is mainly associated with a B-cell precursor immunophenotype of the lymphomatous cells. The most frequent location of skin lesions in children with ALL or LBL is on the head. Further studies are needed to evaluate the prognosis of children with such involvement at diagnosis.
After LMT81 and SFOP LMT89 protocols (both modified LSA2L2), the SFOP LMT96 was designed according to BFM 86 back bone (Reiter and al, J Clin Oncol , 13:359. 1995). The aim of LMT96 protocol was to ...increase chemotherapy intensity early (day 8) and late (after the intensification phase). From February 1997 to December 2003, 83 unselected patients (pts) with newly diagnosed TLL were included in LMT96 protocol: classic corticosteroid prephase (d1 to d7); induction phase with Cyclophosphamide(CPM: 1g/m2) and high dose methotrexate (HDMTX: 3g/m2 infusion over 3h) at d8, followed by Vincristine, Daunorubicin (40mg/m2) d15, 22, 29; 8 injections of Asparaginase, 2 intrathecal injection MTX at d8 and d15 and prednisone for 4 weeks; two consolidation phases with HDMTX, Cyclophosphamide (CPM), Cytarabine and Asparaginase; one interphase with HDMTX and; an intensification phase similar to a phase II BFM study, a maintenance phase with Mercaptopurine/MTX including initially six intensified monthly pulse phases alternating either HDMTX/Asparaginase, or Cytarabine/prednisone. The total duration of treatment was 18 months for stage I, II and III and 24 months for stage IV. Cranial radiotherapy (18Gy) was only performed in pts with CNS disease. 66% were boys, median age was 10.5 y 1.9 – 17.2 and 88% had a mediastinal involvement. According to St Jude classification, 5 had a stage I or II (6%); 47 had a stage III (57%); 24 had a stage IV (63%), 18 bone marrow (BM) involvement, 4 CSF+ and 2 both. 7 pts received corticosteroid before diagnosis and were classified as stage X and treated as stage IV. After induction phase and consolidation (d 65), the complete remission rate was 95%. 1 pt had a progressive disease and died and 2 pts in partial remission are alive after salvage treatment with local radiotherapy or intensification. Up to now, 8 pts relapsed between 3.7 months and 18 months (median=10.5 months) and 1 pt who developed a glioblastoma before relapsing at 69.2 months. All relapses were mediastinal, 3 were combined with BM and 1 with BM and CSF. The median of FU is 4 years. Five-year EFS is 87% CI95%: 79–94 and OS is 89% CI95%: 82–96. In conclusion these results are as high as those of BFM experience. The intensified monthly pulse courses for 6 months did not seem decrease the relapses on therapy. Early intensification at d8 (with CPM and HDMTX) is feasible with a BFM regimen. Omission of prophylactic cranial irradiation while using a short duration (3 hours) of HDMTX infusion did not jeopardize the outcome. We suggest that our early intensification might be combined with a classic BFM regimen.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Malignant tumours of the locomotor apparatus in children mainly comprise bone tumours such as osteosarcoma or Ewing's sarcoma and soft tissue sarcomas such as rhabdomyosarcoma. The diagnosis must be ...considered in any case of progressively worsening limb pain at a fixed site, possibly associated with a soft tissue mass. Standard radiological examinations, Doppler ultrasound, then MRI confirm the diagnosis of a solid tumour, allow staging and may sometimes suggest the invasive nature of the tumour. The biopsy provides a precise histological diagnosis. A frozen section is usually necessary to confirm this diagnosis using the currently available genetic biology tools. Treatment must be adapted to the known prognostic factors, mainly tumour operability, initial size, histological type, response to neoadjuvant chemotherapy and initial staging. Treatment must comprise neoadjuvant chemotherapy followed by local treatment combining radical surgery with reconstruction if necessary and sometimes external beam radiotherapy. This treatment must be completed by postoperative adjuvant chemotherapy resulting in an average total duration of treatment between 6 and 12 months. Long-term follow-up is designed to ensure absence of disease recurrence, attentive orthopaedic follow-up and absence of late sequelae related to anticancer therapy.
