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J Koutecký

Publications and source records attributed to J Koutecký.

At least 19 recordsLinked to original sources

[Ovarian dysgerminoma in children and adolescents. Retrospective clinico-pathologic study].

BACKGROUND: To review the treatment strategy, follow up and outcome for all patients with pure ovarian dysgerminoma treated in childhood and adolescence. METHODS AND RESULTS: Twenty-one patients younger than 18 years were treated between 1979-2002 in Faculty Hospital Motol for newly diagnosed pure ovarian dysgerminoma. Patients were included into the cohort on the basis of revision of archival biopsy specimens deposited in Institute of Pathology and in Molecular Medicine tumor registry. The staging was reviewed retrospectively on the basis of surgical and pathological findings and on results of imaging investigations and outlined according to the TNM and International Federation of Gynecology and Obstetrics (FIGO) classification. The median age at the time of diagnosis was 12.5 years (range 6 years, 5 months--17 years, 11 months). There were ten FIGO stage IA tumors, one stage IB, two of stage IIC, one stage IIIB and seven IIIC tumors. All patients, except two girls with bilateral dysgerminoma, underwent unilateral adnexectomy or ovarectomy. Ten girls were treated postoperatively with chemotherapy, eight with chemotherapy and radiotherapy (eleven with cisplatine based chemotherapy). Three girls with dysgerminoma confined to the ovary (stage IA) have not received adjuvant chemotherapy. With a median follow up 7.1 years all girls remained continuously disease free. The 5-year overall and event free survival is 100%. Majority of patients does not have severe treatment sequelae, three pregnancies have occurred so far. CONCLUSIONS: Most patients with dysgerminoma, including those with metastases can expect cure when treated with conservative surgery and cisplatine based chemotherapy. Reduction of treatment toxicity and preservation of reproductive function is a main task. The girls with dysgerminoma confined to the ovary (stage IA) can be treated with fertility sparing surgery, other should be treated with cisplatine based chemotherapy.

Adolescent↗

[Mixed germ cell tumours of the ovary in childhood and adolescence].

Mixed germ cell tumours of the ovary are rare malignant neoplasms containing combinations of two or more types of germ cell elements. The aim of the study was to review biopsy examinations, medical records, treatment strategy, follow-up and outcome of all girls treated for mixed germ cell tumour of the ovary at the Department of Pediatric Oncology, University Hospital Motol during the period 1979-2002. Archival slides of all tumours were reviewed and tumours were classified according to the WHO system. The clinical data on surgical treatment, chemotherapy and radiotherapy used and follow-up information were obtained in all girls. The staging was reviewed retrospectively on the basis of surgical and pathological findings and results of imaging investigations, and it was outlined according to the most recent FIGO criteria and TNM classification. Sixteen girls with mixed germ cell tumour of the ovary, age range 3 years 11 months to 17 years 8 months (median 12 years) were treated. All girls presented with unilateral tumour of the ovary and all underwent surgery as an initial treatment. The most common presenting symptom was abdominal pain, occurring in ten patients. The original diagnosis of mixed histology was confirmed in all cases; in five cases the tumour contained three histologic components, in eleven cases the tumour consisted of two germ cell types. All tumours contained elements of yolk sac tumour, followed by immature teratoma, embryonal carcinoma, dysgerminoma and mature teratoma. At the time of diagnosis three patients had stage I disease, four patients stage II, seven stage III and two stage IV disease. All patients were treated with chemotherapy after surgery, predominantly with platinum-based regimens (PVB, BEP). Three patients treated initially with MAC (metothrexate, dactinomycin, cyclophosphamide) were diagnosed in the early eighties. In seven girls with advanced disease treated in the early years, radiotherapy was administered to the pelvis or whole abdomen. Overall survival and event-free survival were 80% and 81.3% respectively (median follow-up time 7.6 years). Three patients have died from the disease, two progressed on treatment (MAC), one girl relapsed three months after finishing therapy, no further therapy was administered. One girl underwent resection of tumour of her remaining ovary 24 months after original diagnosis. Histology showed mixed serous and mucinous cystadenoma. The latest examinations revealed that all other patients were in good health. Microscopic examination should be extensive and careful to find out all types of malignant germ cell elements. Platinum based chemotherapy is effective in the management of children and adolescents with mixed germ cell tumors of the ovary. Chemosensitivity of these tumours allows most girls to have conservative surgery with possible preservation of reproductive function.

Adolescent↗

[Radical surgery in the complex treatment of hepatoblastoma].

BACKGROUND: Evaluation of treatment results in children with hepatoblastoma--a retrospective study of clinical and pathological data of surgically treated children. METHODS AND RESULTS: At the clinic of authors, 28 children were operated for hepatoblastoma during 1991 to 2002. Average age of patients was 2.2 years. When tumors were classified according to PRETEX system, 3 children were in the stadium I, 12 children in stadium II, 12 children in stadium III, and one child in the stadium IV. At the diagnosis, lung metastases were found in 3 children. Till 1996 the treatment was initiated by a primary operation, since 1996 by a preoperative chemotherapy. Chemotherapy was administered according to the SIOP protocols. Primary operation was done in 13 children, 15 children were operated after the pre-operation chemotherapy. Liver resection included right-sided lobectomy in 7 cases, extended right-sided lobectomy in 4 cases, left-sided lobectomy in 8 cases, right-sided trisegmentectomy in 5 cases, left-sided trisegmentectomy in 3 cases. In our studied group the three years of event free survival was achieved in 75%, overall survival in 86%. Four children died, two of them because of the progression of the disease, two for the complication during the therapy. CONCLUSIONS: Combination of the radical surgical resection with preoperative and postoperative chemotherapy enables successful treatment of the childhood hepatoblastoma.

Child↗

[Effect of amifostine on reducing acute toxicity of megachemotherapy of tumors in children].

BACKGROUND: Amifostine (WR-2721, Ethyol) is a chemoprotective agent. There is little experiences with amifostine application in megachemotherapy in children. We evaluated amifostine effect on the reduction of the acute toxicity. METHODS AND RESULTS: Retrospective comparison of patients who received amifostine with the control group (72 vs. 72). Amifostine 750 mg/m2 was given 15 minute before cytostatic dose and regularly each eight hours if we administered cytostatics continuously. Megachemotherapy schedule included melfalan, carboplatin, cyklophosphamid, vepesid, busulfan, thiotepa and karmustin. Type of graft: peripheral stem cells 56 vs. 29, bone marrow 8 vs. 30, combination 8 vs. 13. Nonhematological toxicity: mucositis p = 0.047, hepatotoxicity p < 0.001, nephrotoxicity p = 0.005. Hematological toxicity: engraftment D + 12 vs. D + 15 (p < 0.001), amount of erythrocyte transfusions 3 vs. 6 (p < 0.001), platelet transfusions 7 vs. 9 (p = 0.06), days when number of platelets reaches 20,000 without substitution D + 15 vs. D + 22 (p < 0.001). The only statistically difference was in the in total amount of platelets (p = 0.032), when we calculated patients, who received peripheral stem cells. Number of hospitalization days 14 vs. 18 (p = 0.016), days with antibiotics 14 vs. 18 (p = 0.016), number of febrile days 6 vs. 7 (p = 0.51). CONCLUSIONS: Amifostine reduces mucosal, liver and kidney damage. The graft type could affect hematological results.

Adolescent↗

[Detection of minimal residual disease in Ewing's sarcoma using RT-PCR].

BACKGROUND: More than 90% of Ewing's sarcomas (ES) contain a fusion of the EWS and FLI-1 genes, due to the t(11;22)(q23;q12) translocation. At the molecular level, the EWS-FLI-1 rearrangement shows great diversity. Specifically, many different combinations of exons from EWS-FLI-1 encode in-frame fusion transcripts and result in differences in length and composition of the chimeric protein, which function as an oncogenic aberrant transcription factor. The finding of this translocation gives evidence for the presence of ES cells. The aim of this prospective study was to verify applicability of the RT-PCR method for the detection of minimal residual disease in patients with ES. METHODS AND RESULTS: Conditions for the detection of Ewing's sarcoma cells by means of the reverse-transcriptase polymerase chain reaction (RT-PCR) at fusion transcripts in peripheral blood, bone marrow (BM) and autologous hematopoietic stem cell grafts in patients with ES were appointed. 31 samples of BM, 5 samples of blood and 7 peripheral blood grafts obtained from 23 patients were investigated. Presence of tumor cells was identified in 7 BM samples from 7 different patients (31 samples from 16 patients), all the peripheral blood and graft samples were negative. CONCLUSIONS: The high sensitivity of RT-PCR method in detection of cells bearing t(11;22)(q23;q12) was demonstrated in the experimental model and clinical samples. Likewise the literary statements, the RT-PCR method was found to be more sensitive than cytology.

Adolescent↗

[High-dose chemotherapy in the treatment of children with malignant lymphoma].

BACKGROUND: Children with primary refractory or recurrent malignant lymphoma have usually poor prognosis. Less than 10% of those, who were treated with conventional-dose regimens had survived for 2 years. In an attempt to improve the outcome for these patients, we explored the role of consolidation high-dose chemotherapy with autografting. METHODS AND RESULTS: Forty-five patients with poor-prognosis lymphoma, of whom 27 were males, underwent megatherapy between January 1992 and December 1999. High-dose chemotherapy was indicated in patients with poor initial response to first-line chemotherapy (14 cases) or in the relapse (31 cases). The group consisted of 27 patients with Hodgkin's disease and 18 patients with non-Hodgkin's lymphoma. The median age was 14.7 years. The conditioning for Hodgkin's disease patients contained cyclophosphamide, etoposide and busulfan or carmustine. Patients with non-Hodgkin's lymphomas received cyclophosphamide, etoposide and busulfan or total body irradiation. Bone marrow was used as the source of haemopoietic stem cells in ten patients, peripheral blood in twenty-eight, and both sources were used in seven patients. After the median follow-up of 47 months, the final survival was 61%. Eleven patients died of the disease progression, four of the infectious complications, one at a car accident. Median time to relapse after the transplantation was 7.5 months. CONCLUSIONS: Further improvement of these results will require earlier transplantation, improved preparative regiments or early posttransplant immunotherapy.

Adolescent↗

Treatment of children and adolescents with non-Hodgkin's lymphoma (results based on the NHL Berlin-Frankfurt-Münster 90 protocols).

BACKGROUND: To determine the feasibility and results of treating children with non-Hodgkin's lymphomas (NHL) according to very intensive protocols based on the German Berlin Frankfurt Münster NHL 90 study. METHODS AND RESULTS: From 1991 until 1995 eighty two patients less than 18 years of age with NHL were admitted to our department. Sixty three of them were eligible for the study. The entire group consisted of 43 males and 20 females (ratio 2.1:1). Median age was 10 2/12 years. Eleven had stage I disease, 4 stage II, 29 stage III and 19 stage IV disease. Histologies represented were: large cell lymphoma 22, lymphoblastic lymphoma 19, and Burkitt lymphoma 10 patients. In 12 cases the immunophenotype was not further classified as to B-cell or T-cell subtype. Patients were stratified into the therapy groups "B" or "non B" according to histopathology, clinical stage and LDH level. Therapy for the B group consisted of 2, 4 or 6 courses of intensive 5 day pulses of 6 drugs. Patients in the non B group received the protocol for acute lymphoblastic leukemia including reinduction and CNS irradiation for advanced stages. At a median follow-up of 35 months the probability of event free survival (pEFS) at 5 years 70% and overall survival 73% for entire group. For therapy group B pEFS was 76%. The non B therapy group had a pEFS 60% (p = 0.22). There was a significantly better outcome for children classified as stage I and II. There was no statistical difference between stage III and IV. Treatment results were comparable between NHL subtypes, except for large cell lymphomas, which did significantly better (pEFS 90%). CONCLUSIONS: The use of protocols based on BFM 90 study in the Czech Republic was feasible. The pEFS are approximately 10% lower than the German study but comparable to some other studies. Outcome for large cell lymphomas was excellent. Reduction of treatment related complication and mortality rate as well as more precise classification are required.

Adolescent↗

[Advances in the diagnosis and treatment of neuroblastoma].

Neuroblastoma is the second most common solid tumor in children. The prognosis is poor for approximately 70% of patients who have widespread disease at the time of diagnosis. The use of new strategies for classification and therapy has raised expectations for cure in advanced neuroblastoma. We summarise results and experiences reached during the last decade.

Child↗

Surgical tactics in the treatment of malignant renal tumors in childhood.

The authors report the treatment results of 89 children with renal tumors (excluding carcinoma) seen from 1988 to 1992, 71 (79.8%) with favorable-histology Wilms' tumor (survival 86%) and 18 (20.2%) with unfavorable histology, including anaplastic nephroblastoma, clear-cell sarcoma, and rhabdoid tumor (survival 61%). Preoperative chemotherapy (ChT) was given to 46 patients (survival 84%); none was given to 43 with either tumors in the 1st year of life, massive hematuria, or tumor growth during ChT (survival 78%). There were 3 tumor ruptures in the latter group compared to 1 in the former group.

Chemotherapy, Adjuvant↗

[N-myc oncogene amplification in tumors of the sympathetic nervous system: preliminary study of methods and clinico-pathologic correlations].

Tumors of the sympathetic nervous system (NT), namely neuroblastoma (NB) and ganglioneuroblastoma (GNB), have a variable clinical course. Prognostic factors include clinical stage, age of the patient, histological grade, and changes at the genomic level, of which amplification of N-myc oncogene is a well recognized phenomenon. The aim of this study was to establish the status of the N-myc oncogene in NT and to compare the results with histopathological grading, with risk groups according to criteria outlined by Joshi et al., and with the clinical stage. To detect the amplification of N-myc oncogene we used differential polymerase chain reaction (D-PCR). Amplified N-myc oncogene was found in 10 out of 31 investigated children with NB (32%). The result of D-PCR could not be interpreted reliably in two patients. Of seven children with GNB the N-myc amplification was found in one case. None of seven children with ganglioneuroma was found to have amplified N-myc oncogene. The tumors with N-myc amplification were histologically poorly differentiated of undifferentiated with a high mitotic activity-8 children were classified as a high risk category; in two we had not enough surgical material to evaluate the histological prognostic factors. The correlation of N-myc status with clinical stage revealed N-myc amplification in two of 9 children classified as clinical stage III, and in eight of 11 children classified as clinical stage IV. The N-myc amplification was not found in any child diagnosed at stage I, II and IV. Five of 10 children with NB and N-myc amplification died of the disease progression. Four children died in the group of 19 children without N-myc amplification. The median of follow-up was 18 months. The patient with GNB and N-myc amplification also died of the disease progression. Although the follow-up period of the investigated group of patients was short, the results showed that the amplification of N-myc oncogene was associated with tumors of patients diagnosed at a late clinical stage (III and IV), and with tumors whose morphology and age were assessed collectively under the term "high risk group".

Child↗

[Indications for liver resection in childhood].

The authors give an account of indications for resection of the liver in childhood which comprise extensive injuries, cysts, vascular malformations and primary (benign and malignant) and secondary tumours. During the period between 1986 and 1993 34 major resections of the liver were performed and examined. Indications for resection were in 17 cases hepatoblastomas, 4x metastasis, 3x hepatocellular carcinoma, 2x haemangioendothelioma, adenoma, hamartoma and in one case each necrosis after injury, a cyst, cavernous haemangioma and sarcoma. The mean age of the operated children was 4 years and 5 months, the youngest patient was operated on the age of 4 days and the oldest was 17 years old. During the period between 1986 and 1989 the survival rate was 33%. During the period between 1990 and 1993 the survival rate was 86.5%. The authors emphasize the radical character of operations of malignant tumours which has a marked impact on the survival of patients with these disease.

Adolescent↗

[Surgical methods in the treatment of retroperitoneal neuroblastoma].

The authors submit their experience with the surgical treatment of retroperitoneal neuroblastoma, which is one of the most frequent solid tumours of child age. They describe the principles of the surgical technique with emphasis on radical removal of the tumour even in the area of large blood vessels. They emphasize the advantage of using an ultrasonic surgical aspirator (CUSA, Valleylab Co., Pfizer) which makes it possible to remove residues of the tumour without damaging the large blood vessels. During the period between 1987-1992 they treated at the Motol Faculty Hospital 69 children with retroperitoneal neuroblastoma. Of these 60 were operated. In the first stage of the disease 9 patients were operated, all survive. In the second stage 4 patients were operated, 75% survive, in the third stage 24 patients 50% survive, in the fourth stage 20 of 29 patients were operated and 11 (38%) survive. In stage IV--S three patients were operated, one survives. The total survival of patients during the investigation period is 52%.

Adolescent↗

[The solitary kidney in patients after nephrectomy in Wilm's tumor].

A single kidney in patients with agenesis or after nephrectomy is regarded as a risk factor. Primarily, the role of glomerular hyperfiltration and later possible glomerular sclerosis are emphasised. Complex treatment of Wilms tumour patients includes previous chemotherapy and radiotherapy. The aim of our study was to investigate late sequelae of this aggressive treatment on the morphology and function of a single kidney and to compare these results with a group of single kidney patients, where the diagnosis of Wilms tumour was excluded.

Adolescent↗