Biomedical subjects

D Harms

Publications and source records attributed to D Harms.

At least 271 records · Page 15Linked to original sources

PubMedDate not supplied

Genetic analysis of childhood germ cell tumors with comparative genomic hybridization.

BACKGROUND: Germ Cell Tumors (GCTs) in children and adolescents constitute a clinically and histologically heterogeneous group of tumors. Compared to GCTs in adults, the numbers of GCTs in children analyzed with cytogenetic and molecular genetic techniques is limited. However, the data available to date reveal a pattern of cytogenetic aberrations different from that in adults. Comparative genomic hybridization (CGH) is a valuable technique for the genetic profiling of tumors that allows screening for chromosomal imbalances consistent with amplification of oncogenes and loss of putative tumor suppressor genes. As CGH does not require tissue culture, it also allows analysing archival tissue samples. PATIENTS: This study focuses exclusively on GCTs in children younger than ten years of age and summarizes the genetic data of 51 tumors. Eighteen teratomas and 33 malignant GCTs were included. Primary sites were the testis (n=10), coccyx (n=13), mediastinum (n=20), ovary (n=5), CNS (n=2), and the face (n=1). METHODS: The experimental procedure includes differential enzymatic fluorescence labeling of tumor and control DNA followed by comparative hybridization to normal male chromosomes, karyotyping and computerized analysis of the fluorescence profiles. RESULTS: With the exception of one testicular and two ovarian tumors, malignant GCTs in children do not show chromosomal gain of 12p, which is characteristic of GCTs in adult patients. Irrespective of the primary site, childhood GCTs show chromosomal imbalances of chromosome 1 (loss of distal 1p, gain of 1q), deletion of 4q and 6q as well as gain of 20q at a high frequency. CONCLUSIONS: These studies will help guiding further investigations elucidating the role of putative tumor suppressor genes at e.g. 1p36 and 6q. In addition, further studies incorporated in prospective therapeutic protocols are necessary to evaluate the prognostic relevance of specific genetic aberrations.

Adolescent↗

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[Leprosy - An overview from a pediatric perspective].

Worldwide tourism is an increasing industry. One result of this phenomenon is the occurrence of imported infectious diseases, as recently observed even in Germany. Leprosy ranks high among dreaded infectious diseases from tropical and subtropical countries. It remains a major health threat despite marked improvements in diagnosis and therapy. This was achieved by a better understanding of bacteriological and immunological mechanisms over the past decades, resulting in a decline of Leprosy's incidence.

Age Factors↗

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Therapy of advanced ovarian juvenile granulosa cell tumors.

BACKGROUND: Gonadal sex cord-stromal tumors are rare tumors that develop from the gonadal non-germ cell component such as granulosa, Sertoli or Leydig cells. Among these, juvenile granulosa cell tumors (JGCT) constitute the largest subgroup of ovarian sex cord-stromal tumors during childhood and adolescence. In local disease (FIGO stage I), the beneficial role of tumor-ovarectomy is well established. In contrast, life expectancy in patients with advanced JGCT (FIGO stage >/= II) is short even after complete tumor resection. The current literature provides only limited and inconclusive data regarding the value of adjuvant chemotherapy in such patients with advanced disease. PATIENTS AND METHODS: Therefore, we analyzed the patients with FIGO stage >/= II JGCT who were prospectively documented as follow-up patients of the German MAKEI trials for non-testicular germ cell tumors and received the recommended cisplatin-based chemotherapy in an adjuvant setting. From 1988 until 2000, 7 patients (age, 4;2 - 18;11 years, median 14;8 years) were registered. Three patients were stage IIc, one stage IIIa, and three stage IIIc. 5 patients underwent laparatomy with adnectomy, which was complete in only two patients. Two patients received laparoscopic tumor resection, which was incomplete in both. All patients received 4 or 6 cycles of adjuvant cisplatin-based three-agent chemotherapy in analogy to the current therapeutic concept applied in malignant germ cell tumors. One patient with a large tumor and multiple peritoneal metastases additionally received 40 Gy abdominal irradiation. RESULTS: All patients achieved complete clinical remission after initial surgery and adjuvant chemotherapy. 4 out of 7 patients are currently remaining in first continuous complete remission after 15 to 111 months follow-up. One patient developed a metachronous tumor of the contralateral ovary after 126 months follow-up and is still alive but currently in therapy of another recurrence. Another patient suffered a tumor recurrence after 12 months but achieved a second complete remission with cisplatin chemotherapy after a follow-up of currently 4 months. One patient achieved complete clinical remission but suffered a diffuse peritoneal tumor recurrence with massive ascites and finally died as a result of tumor progression. In summary, at the time of this report 6 of 7 patients are alive after a median of 47 (15 - 138) months. CONCLUSION: This analysis clearly demonstrates that advanced JGCT can be successfully treated with surgery followed by adjuvant cisplatin-based chemotherapy. Therefore, this study reveals encouraging therapeutic perspectives in these otherwise fatal tumors that merit further investigation in a prospective cooperative trial.

Adolescent↗

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An approach for cure: PEI-chemotherapy and regional deep hyperthermia in children and adolescents with unresectable malignant tumors.

BACKGROUND: Elevated temperatures of 40 - 44 degrees C increase the actions of various anticancer drugs including N-lost derivatives, cytotoxic antibiotics and platinum analoga. In clinical usage thermochemotherapy (TCH) should facilitate surgical resection and ameliorate local tumor control. PATIENTS AND METHODS: From 07/1993 to 12/2002 a total of 39 patients have been enrolled onto a phase-II study (female = 24, male = 15, age 1 - 37.5 years, median 5.2). Among these, 24 patients had extracranial non-testicular germ cell tumors and 15 patients soft tissue or chondrosarcomas. INDICATION: locoregional relapse (n = 29) or unresectable tumor after neoadjuvant chemotherapy (n = 10). Among these two groups, there were ten patients with poor response or progressive disease under primary or relapse chemotherapy. Ten out of the 29 relapse patients had more than one relapse. Tumor site: pelvis (30), abdomen (4), head and neck (2), proximal leg (2) and lumbar spine (1). Thermochemotherapy (TCH): 1800 - 2000 mg ifosfamide/m (2) and 100 mg etoposide/m (2) on days 1 - 4 and 40 mg cisplatin/m (2) on days 1 + 4 combined with regional deep hyperthermia (42 - 44 degrees C, 1 h) on days 1 + 4. RESULTS: In 39 protocol patients a total of 166 TCH courses (332 heat sessions) were applied. 20 patients achieved complete response, and 10 patients achieved partial response. TCH was followed by surgical tumor resection in 28/39 patients and/or radiotherapy in 13/39 patients. At a median follow-up of 27 months, outcome in this high-risk patient population was 22 NED, 3 AWD, 12 DOD, 2 DOC. Five year event free (EFS) and overall survival (OS) for the whole study cohort was 0.39 +/- 0.11 (20/39 patients) and 0.52 +/- 0.11 (25/39 patients), respectively. CONCLUSION: TCH shows substantial therapeutic efficacy and facilitates complete tumor resection in 14 out of 28 operated patients. Multimodal treatment including TCH, surgical resection and/or radiotherapy leads to sustained remission in the majority of patients with locoregional tumor recurrence. The therapeutic effect is most pronounced, if TCH is administered at first relapse. Due to the clinical and histologic heterogeneity the number of patients eligible for TCH is limited. Therefore, a more valid assessment of treatment efficacy can only be made by a matched-pair comparison in cooperation with the clinical registers.

Abdominal Neoplasms↗

PubMedDate not supplied

Pathology and molecular biology of teratomas in childhood and adolescence.

The biologic behaviour of teratomas depends on various interdependent clinical and epidemiologic variables such as the age at diagnosis, sex, tumor site, histology which all correlate to different cytogenetic and molecular biologic aberrations. Thus, testicular teratomas of infancy are generally benign. Accordingly, prepubertal teratomas show no cytogenetic or molecular genetic aberrations. In contrast, postpubertal testicular teratomas can present as clinically malignant tumors and may show complex cytogenetic aberrations such as the isochromosome 12p, which is pathognomonic of malignant germ cell tumors. Notably, teratomas of both age groups show an at least partial erasure of the genomic imprinting, correlating with their origin from primordial germ cells. The Kiel Pediatric Tumor Registry includes 541 teratoma specimens, and among these, the most frequent tumor sites (in descending order) are: the sacrococcygeal region (33.8 %), the ovaries (31.2 %) and the testes (10.5 %). Rare localizations include the mediastinum, the retroperitoneum, the head and neck region as well as the central nervous system. The WHO classification of germ cell tumors distinguishes mature and immature teratomas as well as teratomas with malignant transformation. In immature teratomas, primitive neuroectodermal structures predominate. According to the grading system (Gonzalez-Crussi, 1982), mature teratomas (G0) are more frequent (54.5 %) than immature teratomas (G1-G3, 45.5 %). Only 7.8 % of all teratomas show the highest grade of immaturity (G3). The frequency of additional microscopic foci of malignant yolk sac tumor correlates with the grade of immaturity. In sacrococcygeal teratomas, the yolk sac tumor microfoci may give rise to a malignant relapse after incomplete resection. The rare teratomas with malignant transformation contain components with "conventional" somatic type malignancy such as leukaemia, carcinoma or sarcoma. Here, molecular genetic analysis has demonstrated the origin of the somatic malignancy from a malignant transformation within the germ cell tumor with retention of the cytogenetic changes characteristic of malignant germ cell tumors.

Adolescent↗

PubMedDate not supplied

The malignant potential of teratomas in infancy and childhood: the MAKEI experiences in non-testicular teratoma and implications for a new protocol.

UNLABELLED: Since 1982, mature and immature teratomas have been recruited into the MAHO and MAKEI protocols of the German Society for Pediatric Oncology and Hematology (GPOH) for testicular and non-testicular germ cell tumors in order to study the epidemiology and clinical behaviour of teratomas. Patients were registered in the epidemiologic German Childrens Cancer Registry and the GPOH Childrens Tumor Registry for pathological review. Patients with immaturity grade 2 and 3 according to Gonzales-Crussi were eligible for adjuvant chemotherapy. The consecutive protocols MAKEI 83/86/89 have been published previously in detail (Klin Paediatr 1997; 209: 228-234, Med Pediat Oncol 1998; 31: 8-15) and will be compared to the data of MAKEI 96. For this comparison, 274 patients from MAKEI 83/86/89 and 261 patients from MAKEI 96 are evaluable. RESULTS: 1) EFS after complete tumor resection has been estimated to 0.96 +/- 0.01 in both observation periods. 2) Incomplete tumor resection remains the main risk factor for relapse (EFS 0.55 +/- 0.09). 3) The relapse rate declined from 13.9 % in MAKEI 83/86/89 to 9.5 % in MAKEI 96. 4) In MAKEI 83/86/89 four newborns with teratoma died due to perioperative complications and nine children as a result of tumor progression, whereas in MAKEI 96 no newborn died, only one child died from tumor progression, and another child died during long time observation for another reason (meningitis). 5) In accordance to the experience of the MAKEI 83/86/89 studies, no child of the MAKEI 96 study presented with yolk sac tumor at recurrence if adjuvant chemotherapy was administered during first-line treatment because of immaturity. In contrast, more than half of the children with tumor recurrence after watch and wait strategy had yolk sac tumor in addition to teratoma.

Adolescent↗

PubMedDate not supplied

[Improved prognosis of intracranial germ cell tumors by intensified therapy: results of the MAKEI 89 therapy protocol].

Germ cell tumors of the central nervous system are histological identical to the extracranial tumor sites. According to the localisation germ cell tumors of the CNS are different in symptoms, diagnostic approaches, kind and location of metastases and stratification of therapy. Since 1986 patients with intracranial germ cell tumors are registered in the ongoing study for non-testicular germ cell tumors (MAKEI) of the German Society of Pediatric Oncology and Hematology, and are treated in accordance to therapy guidelines for extracranial sites. In MAKEI 89 therapy strategy was revised with a reduction of radiotherapy and an increased cumulative cisplatinum dose from 200 mg/m2 to 400 mg/m2. Patients with germinoma receive after histologic diagnosis radiotherapy consisting of 30 Gy craniospinal irradiation and 15 Gy tumorboost. Malignant non-germinoma receive after diagnosis by tumor marker in CSF and/or serum 2 courses bleomycin 15 mg/m2 day 1-3, Etoposide 150 mg/m2 day 1 + 2 and cisplatinum 20 mg/m2 days 4-8 (BEP), continued by 2 courses Vinblastine 3 mg/m2 day 1 + 2, Ifosfamide 1500 mg/m2 days 1-5 and cisplatinum 20 mg/m2 days 1-5 (VIP), followed by 30 Gy craniospinal irradiation and 20 Gy tumor boost. In teratoma first line therapy is complete resection. In incomplete resected cases adjuvant chemotherapy according to histological grading is administered. Until 31st January, 1993 101 patients (pts) were registered, containing 69 protocol pts. Diagnosis in protocol pts was teratoma in 8 cases, 2 pts died postnatal because of extended disease, 2/8 pts relapsed, but were salvaged by chemotherapy. 40 pts offered germinomas.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMedDate not supplied

[Therapy of testicular germ cell tumors. Current status of the MAHO studies].

The GPOH-MAHO trials designed in 3/82 and 7/88 for treatment of childhood testicular germ cell tumors registered 3/92 105 pts. In MAHO 82 study 57 pts. and in MAHO 88 study 48 pts. were treated. Histologically 60% of the tumors revealed yolk sac tumors (YST), 22% teratomas (TD) and 18% malignant teratomas (MTI, MTU, MTT). Beside unilateral orchiectomy, according to stage and histology a stratified chemotherapy was administered. Standard chemotherapy consisted of 4 courses VLB, BLM, DDP, after 2 courses standard chemotherapy if vital tumor was suspected: explorative laparatomy. According to laparatomy some patients received salvage chemotherapy of 3 courses VP 16, IFO and DDP. The following results were obtained: YST: 59 pts. with stage I. Of these 10 received adjuvant chemotherapy with VLB, BML, DDP. 49 pts. were followed according to wait and see policy and not treated by adjuvant chemotherapy; 8 pts. had a relapse. 5 were treated with standard chemotherapy and 1 pt. with salvage therapy. 1 pt. had stage II and another stage III. Both received standard chemotherapy. The survival of all 61 pts. is 100%. Median observation time is 4 years. TD: 25 pts. had stage I. No chemotherapy was given. The relapse free survival is 100%. Median observation time is 4 years. Malignant teratomas (MTI, MTU, MTT): 8 pts. had stage I. Of these 3 received adjuvant chemotherapy, 5 lymphadenectomy without chemotherapy. All patients survived without relapse. 8 pts. had stage II and received standard chemotherapy, of these 4 pts. had stage IIc and explorative laparotomy was done. According to the result 2 pts. received salvage therapy. All pts. survived relapse free.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMedDate not supplied

[Preoperative chemotherapy of nephroblastoma. Preliminary results of the SIOP-9/GPO therapy study].

The therapeutic strategy of the SIOP-9 study includes pre-operative chemotherapy for all patients with nephroblastoma diagnosed by imaging methods aged between 0.5 and 16 years. By pre-operative chemotherapy the rate of radical resectable tumors should be increased and thereby the intensity of postoperative therapy, in particular of radiotherapy, diminished. Patients with nephroblastoma stage I-III were in case of tumorresponse randomised in either a 4 weeks or 8 weeks arm of pre-operative treatment with ACT D and VCR. The question was, if a prolongation of pre-operative chemotherapy could increase the relative part of stage I. Between 1/1/89 and 6/30/91 from 49 oncologic centres of former western Germany 188 patients were registered in the SIOP-9/GPO. From the stage I-III patients between 0.5 and 16 years 80.1% were pretreated with cytostatic agents. Only 53.9% of the patients with tumorresponse were randomised. The relative frequency of intraoperative ruptures was with 3% lower in the group of pretreated patients than in the primary operated (15.3%). The stage distribution for all Wilms' tumor patients showed a prevalence of stage I with 43.3% (after pre-operative treatment 59%; after primary operation 28%). Abdominal radiotherapy was performed in 22.4%. The event-free survival rate of all nephroblastoma lay at 85% 3 years after diagnosis (stage I standard 96%; unfavorable histology all stages 45%). 7.3% of the patients developed a hepatopathy under treatment and 7.8% even a VOD according to the criteria of McDonald.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMedDate not supplied

The applicability of immunohistochemistry in the diagnosis and differential diagnosis of malignant soft tissue tumors. A reevaluation based on the material of the Kiel Pediatric Tumor Registry.

The diagnosis and differential diagnosis of malignant soft tissue tumors not infrequently poses great difficulties, especially in those cases which lack any feature of differentiation by conventional light microscopy. These difficulties have been partially resolved through the application of ultrastructural investigations. Recently considerable progress has been achieved using immunohistochemistry. At the Kiel Pediatric Tumor Registry we were able to reduce the percentage of unclassified soft tissue sarcomas from 17.6% in 1982 to 4.5% in 1989. Particularly useful were antibodies against the different types of intermediate filaments, muscle-specific actin, myoglobin, and the neural markers neuron-specific enolase and protein S-100. In contrast to the expected immunophenotype rhabdomyosarcomas, malignant peripheral neuroectodermal tumors and malignant schwannomas showed expression of cytokeratins. Moreover, in many cases rhabdomyosarcomas and synovial sarcomas expressed neural markers. Ewing's sarcoma and malignant peripheral neuroectodermal tumor are histogenetically related, but differ in grade of neural differentiation. In all soft tissue sarcomas immunohistochemistry is very useful to obtain information on the cellular heterogeneity. Despite the great achievements not every soft tissue sarcoma can be diagnosed with certainty. There will always be a baseline of unclassified cases due to problems which are not caused by the tumor itself but rather by diagnostic circumstances.

Adolescent↗

PubMedDate not supplied

[Intensive combined drug therapy and beta-interferon in the treatment of children with metastasizing neuroblastoma: GPO-NB 79/82 study].

From June 1979 to June 1982 72 children with metastatic neuroblastoma older than 1 year of age at diagnosis were treated according to the protocol NB 79 of the German Society for Paediatric Oncology. The chemotherapy included three cycles of adriamycine, cyclophosphamide, vincristine, dacarbazine and five cycles of adriamycine and cyclophosphamide. 34 patients were randomized for additional beta-Interferon (Fiblaferon) treatment (10(5) U/kg daily for 3 weeks followed by 3 times a week for 21 weeks). 38 patients were not treated with Fiblaferon. The response rate was 96% including 35% complete and 61% partial remissions. Preoperative chemotherapy improved remarkably the resectability of the primary tumor without enhancing complication rates. The cumulative proportion surviving was 21% for the interferon group and 6% for the patients not treated with interferon. The cumulative recurrence free survival rate, however, was 5% for both groups. The median survival time was 19 months and the median recurrence free survival time 10 months. A serious side effect of chemotherapy was bone marrow depression (73%) resulting in 1 week delay of drug administration per cycle. The median of administered drug dosages was 87-100% of the recommended dosages. Side effects of beta-Interferon commonly decreased with time and were fever reactions (55%), shaking chills (29%), cardiovascular reactions (16%).(ABSTRACT TRUNCATED AT 250 WORDS)

Age Factors↗

PubMedDate not supplied

[Malignant testicular tumors in children. Concept of the cooperative therapy study MAHO 88 based on MAHO 82].

The German society of Pediatric Oncology (GPO) designed in 1982 a cooperative study to improve the outlook of patients with malignant testicular germ cell tumors (MAHO 82). So far the combined modality therapy was effective to increase the relapse free survival (Haas et al. 1988). From the data the MAHO 88 protocoll was outlined as follows: Yolk sac tumor: Unilateral orchiectomy; Stage I A - wait and see. Stage I B/C-IV 4 courses standard chemotherapy. If alpha-feto-protein after 2 courses is still positive explorative laparatomy has to be performed. Malignant teratoma (MTI, MTU, MTT), seminoma: Unilateral orchiectomy. Stage I: 4 courses standard chemotherapy. Stage II-IV: 2 courses standard chemotherapy, explorative laparatomy. During explorative laparatomy 3 different approaches are recommended: Modified lymphadenectomy, radical lymphadenectomy or removal of "bulky disease". In case of presence of vital tumor cells patients with teratoma receive "salvage therapy", patients with seminoma receive instead x-irradiation. If only differentiated teratoma cells are found in addition 2 courses of standard chemotherapy are administered. Standard chemotherapy consists of Vinblastine, Bleomycine and Cis-platinum, salvage therapy of VP 16, Ifosfamide and Cis-platinum.

Adolescent↗

PubMedDate not supplied

[Immunopathology of brain tumors].

The various classifications of brain tumors are characterized by a rather disturbing diversity of tumor designations. This diversity results from the heterogeneity of human brain tumors, but also from the lack of knowledge about the histogenesis of many of these tumors. The histogenesis of some of the different types of tumor could be resolved by the application of electron microscopical studies. New aspects are gained from immunohistochemical investigations using mono- and polyclonal antibodies against intermediate filaments (GFAP, vimentin, cytokeratins), neuron-specific enolase (NSE), protein S-100, carcinoembryonic antigen (CEA), myelin-associated glycoprotein (Leu 7), synaptophysin and nuclear proliferating antigen (Ki-67). The present paper gives a survey on how immunohistochemistry can be advantageously used in the diagnosis of brain tumors.

Antigens, Differentiation, T-Lymphocyte↗

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[Rhabdomyosarcoma: morphology and cellular differentiation].

544 malignant soft tissue tumors have been collected at the Pediatric Tumor Registry in Kiel including 300 cases of rhabdomyosarcoma (55%). In 237 of the 300 cases the diagnosis is certain. Liposarcoma and malignant fibrous histiocytoma which are typical tumors of adult age are rare in our material. Among rhabdomyosarcomas embryonal rhabdomyosarcoma (eRMS) clearly predominates accounting for almost 72% of all rhabdomyosarcomas. Differentiation of tumor cells in eRMS may vary considerably. Therefore, three groups of eRMS were distinguished and analyzed for clinico-pathologic features: 1. Primitive eRMS with less than 10 rhabdomyoblasts. 2. Intermediate eRMS with 10-50% rhabdomyoblasts. 3. Well differentiated eRMS with greater than 50% rhabdomyoblasts. By immunohistochemistry, vimentin positive cells were found in all three groups. The number of desmin positive cells depended upon the grade of differentiation. Thus, there were more desmin positive cells in well differentiated eRMS. Primitive and well differentiated eRMS were predominantly located in the head and neck area, intermediate eRMS in the abdomen. Primitive eRMS were noted in higher stages than tumors of the other two groups. Response to chemotherapy as evaluated in the 7th week of treatment was better in well differentiated eRMS. Moreover, patients of this group achieved more often complete remission. It is concluded from the present study that differentiation in eRMS may have an influence on the clinical presentation and clinical course of the disease. Therefore, this question should be investigated in more detail in a larger prospective study.

Abdominal Neoplasms↗

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[Analysis of prognostic factors in rhabdomyosarcoma. Preliminary univariate and multivariate results of the Cooperative Soft Tissue Sarcoma Study (CWS-81)].

The characteristics of 129 children with rhabdomyosarcoma entered into the CWS-81 study between 1981 and 1984 were examined for their relationship to prognosis. Prognosis was defined as relapse-free survival time. The trial was stratified to clinical groups depending on the surgical procedure at onset. So, each group had to be analysed separately. The patient characteristics of group I/IIA mostly related to prognosis were primary site (disease in extremities, unfavorable; paratesticular primary, favorable) and tumor infiltration into adjacent bone (bone infiltration, unfavorable). In group IIB/III the degree of tumor response within 7-9 weeks, chemotherapy exclusively given, was the only characteristic significantly related to prognosis (greater than 2/3 tumor reduction, favorable; less than 2/3 tumor reduction, unfavorable). Other prognostic factors as histological subtype, primary site, tumor diameter, lymph node involvement, bone infiltration, sex and age had no significant influence. The tumor diameter was the only characteristic with significant influence in group IV. The larger the tumor diameter, the poorer was prognosis. Furthermore, the alveolar subtype was seen predominantly in non-responding tumors of group IV. In group III, we fitted the patient characteristics in a multivariate regression model (Cox's model). The degree of tumor response within 7-9 weeks was analysed to be the main hazard function related to prognosis. In future, the degree of tumor response within a certain time can be used as the measurement of effectiveness of chemotherapy. So, in patients of group III and IV a risk adapted therapeutic procedure can be undertaken.

Antineoplastic Combined Chemotherapy Protocols↗

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[Cooperative therapy study of nontesticular germ cell tumors MAKEI 83 of the Society of Pediatric Oncology: analysis after 3 years].

The German Society of Pediatric Oncology has initiated in 1983 a cooperative trial for the treatment of extratesticular germ cell tumors. The treatment plan is stratified according to histology, tumor site and tumor extension. Patients with unfavourable histology received depending on prognostic factors 4 courses of either vinblastine, actinomycin D and cyclophosphamide, or the combination of vinblastine, bleomycin and cisplatinum (4 courses) completed by 4 additional courses of VP 16, ifosfamide and cisplatinum. Within 3 years, 115 patients from 37 different institutions were entered into the trial. 55 of 72 protocol patients are under observation for at least 10 months since diagnosis. The disease-free survival rate according to Kaplan-Meier is 80% (+/- 6%) at 36 months. From the interim results of this ongoing study, the following conclusions are drawn: The chemotherapeutic regimens as delivered are tolerable. Radiotherapy does not seem necessary with the exception of ovarian dysgerminomas stage I a. In coccygeal teratomas the resection of the coccygeal bone decreases the hazard of local recurrences. Using the risk adapted regimen, the prognosis for teratomas with Yolk sac origin appears as favourable as for patients with mature teratomas.

Adolescent↗

PubMedDate not supplied

[Results of the cooperative Malignant Testicular Tumors 82 Study of the German Society of Pediatric Oncology on the therapy of malignant germ cell tumors in childhood].

In the German Society of Pediatric Oncology (GPO) designed in 1982 a cooperative study to improve the outlook of patients with malignant testicular germ cell tumors. According to stage and histology of the tumor different surgical approaches to retroperitoneal lymphadenectomy are outlined. Local radiotherapy is not recommended. Adjuvant chemotherapy with Vinblastine, Bleomycin and CIS-Platinum is proposed. Up to 1987 27 YST, 3 MTI, 6 MTU, 1 MTT and 9 TD were treated. Only one patient with YST had a relapse; one patient died from candida septicemia. Alternative chemotherapy was given 3 times. Overall toxicity from treatment was mild. So far the combined modality therapy was found to be effective to increase the relapse free survival.

Adolescent↗

PubMedDate not supplied

[Cooperative Ewing's Sarcoma Studies 81/86: pathologico-anatomic and immunohistochemical findings and differential diagnosis of Ewing sarcoma].

158 cases of the Cooperative Ewing's Sarcoma Trials (CESS 81/86), which have been documented at the Pediatric Tumor Registry, Kiel, were studied by conventional light microscopy and immunohistochemistry. There were 77 cases of typical Ewing's sarcoma with 70 cases being located in the skeleton and 7 in soft tissues. Of the 14 cases of atypical Ewing's sarcoma 7 cases each were localized in bone and in soft tissue, respectively. In contrast to typical Ewing's sarcoma, cells of atypical Ewing's sarcoma were larger and displayed more heterochromatin. Both, typical and atypical Ewing's sarcoma reacted positively for vimentin. Other stains were negative, notably the neuron specific enolase (NSE). In 55 cases a diagnosis of malignant peripheral neuroectodermal tumor (MPNT) was made. Histologically most of these tumors resembled atypical Ewing's sarcoma. By immunohistochemistry positive reactions were found for NSE, vimentin, protein S-100, neurofilaments and glial fibrillary acidic protein. In 3 cases a diagnosis of small cell osteosarcoma was made. There were 2 cases of undifferentiated sarcoma of bone, 2 cases of soft tissue sarcoma of undetermined histogenesis and 2 cases of rhabdomyosarcoma. Of the 4 tumors which could be investigated for response to polychemotherapy, 1 each corresponded to grade II and III, respectively, and 2 to grade IV according to the classification of histologic grade of regression established by Salzer-Kuntschik et al. (1983).

Adolescent↗