Biomedical subjects
Publications and source records attributed to R Kodet.
Three cases of 4-year-, 16-month- and 8-month-old children suffering from a disseminated form of histiocytosis X showed overlapping of Hand-Schüller-Christian syndrome and Letterer-Siwe syndrome in clinical picture, biopsy and autopsy. Morphological lesion does not develop simultaneously in all the organs. Some of them tend to steatosis and fibrosis of histiocytosis infiltrations, which the others do not.
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Malignant non-Hodgkin's lymphomas (NHL) of childhood and adolescence are a heterogeneous group of diseases originating from the lymphoid cells. Unlike adults with non-Hodgkin's lymphoma, children typically have extranodal disseminated disease of high grade (Burkitt's lymphoma, large cell lymphoma, or lymphoblastic lymphoma). This study was conducted to determine the feasibility of treating children in the Czech Republic with B-cell non-Hodgkin's lymphomas according to very intensive protocols based on the German Berlin Frankfurt Munster (BFM) NHL 90 study. Treatments are divided in the BFM studies according to "B" and "non-B" immunophenotypes. The authors report only those treated according to the BFM B-cell protocol. From 1991 through 1997 eighty-two patients less than 18 years with NHL were admitted to the department. Seventy-three of them were classified as B-cell lymphoma and 54 were thus eligible for the BFM B-cell treatment. The entire group consisted of 38 males and 16 females (ratio 2.38). Median age was 11.6 years. Twelve had stage I disease, 3 stage II, 30 stage III, and 9 stage IV lymphoma. There were 21 patients with Burkitt's lymphoma, 29 with large cell lymphoma, of which 5 were patients with MALT lymphoma. In 3 cases B-cell NHL was not further classified and one child had a mediastinal B lymphoma. Patients were further stratified according to clinical stage and lactate dehydrogenase (LDH) level. Therapy consisted of a prephase and short (2, 4, or 6 courses), intensive 5-day therapy with 6 drugs. The probability of event-free survival (pEFS) for the entire group was 74% and overall survival at 5 years was 80%. There was a significantly better outcome for children classified as stage I. No difference was observed between the EFS of stage III and IV patients. Four patients died from treatment-related complications in complete remission. Treatment results were not identical between NHL subtypes, with large cell lymphoma patients doing significantly better (pEFS 90%, p = .008). The use of protocols based on BFM 90 study was feasible at this center. The treatment results are approximately 10% lower than those reported by BFM investigators, but comparable to results from other centers.
Treatment of Hodgkin's disease in children should be directed at maximizing cures and minimizing the long-term effects of alkylating agents, anthracyclines, and bleomycin. In this study methotrexate and etoposide were used in the VAMP/VEPA regimens to treat 60 clinically staged pediatric patients with Hodgkin's disease. Twenty-nine patients with stages I-IIA received four courses of VAMP plus low-dose radiotherapy. Thirty-one IIA bulky disease and IIB-IVB patients received four or six courses of VEPA plus low-dose radiotherapy. There were 6 partial remissions after the completion of chemotherapy and all of these patients relapsed, but 4 were successfully salvaged with ABMT. Two patients have died. The 3.1-year overall survival rate is 97% (100% VAMP, 94% VEPA) and the event-free survival rate is 88% (97% VAMP, 77% VEPA). These results suggest that VAMP is a reasonable treatment for low stages of Hodgkin's disease, but more advanced disease is not adequately treated by VEPA and low-dose radiotherapy.
Epithelioid sarcoma in a rare tumor and most of the cases occur in young adults. It is rare in childhood. We have been able to obtain data and histologic material for 11 patients with this disease. The primary sites were head and neck in three patients, inguinal region in one, and extremities in seven. The age range of the patients was 12 weeks to 13 years. There was a preponderance of males over females with a ratio of 1.75. The tumors presented with a typical nodular necrotizing pattern. In three cases giant osteoclast-like cells were present. The immunohistochemistry and electron microscopy showed features consistent with previous observations on epithelioid sarcomas. In one case islands of small dark cells noted on light microscopy were surrounded by basal lamina on electron microscopy. The cells inside the nests were undifferentiated. Six tumors studied by flow cytometry were in DNA diploid range. On follow-up, five children are alive and well 2 to 7 years after treatment. Three children died of tumor progression with metastases to lymph nodes and lungs. One child had been diagnosed only recently, and in one the disease has run a protractive course with multiple recurrences. The behavior of these epithelioid sarcomas in children is similar to that seen in adults, the prognosis being dependent on radical tumor surgery preventing recurrent disease. Long-term follow-up is necessary because the tumor may recur many years after the primary tumor was removed.
A case of an adolescent girl with metastatic gastric stromal tumor is described. There were three metastatic nodules in the liver at the time of the admission. A subtotal gastrectomy was performed. The tumor had distinctly nodular appearance and was composed of a variety of cells suggestive of smooth muscle differentiation. Electron microscopy revealed cytoplasmic neural processes and densecore neurosecretory granules. Immunohistochemistry showed positive neuron-specific enolase, synaptophysin, and chromogranin A in some of the tumor cells. Similar findings in the primary tumor and its liver metastases indicated a primitive neural differentiation and enabled us to classify the lesion as a gastric autonomic nerve tumor. No other tumors that would suggest that the gastric lesion is a part of Carney's triad were detected. The child was treated with chemotherapy but the liver metastases did not change significantly. She is alive with unresectable liver metastases 10 months after the gastrectomy.