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Biomedical subjects

P Gutjahr

Publications and source records attributed to P Gutjahr.

At least 19 recordsLinked to original sources

[Wilms tumor].

Wilms tumor is the most frequent malignancy of the urogenital tract in children. Its treatment gives an excellent example of the efficacy of multimodal therapeutic strategies in oncological diseases. In particular, the performance of international, randomized multicenter studies has led to a continuous increase in disease-free survival among the affected patients in recent decades, thanks to increasingly detailed stratification of individual elements of the therapy. The pediatric urologist has a definite place in interdisciplinary teams treating Wilms tumor, being needed for the operative component of the treatment. For further improvement of the prognosis in this disease and to reduce the comorbidity caused by the treatment, the major challenge for pediatric urologists in coming years will be to make the surgery less traumatic and thus also to reduce the incidence and severity of perioperative complications.

Child↗

Flow-cytometric DNA analysis of intracranial tumors in children.

The objective of this study was to investigate flow-cytometric DNA values of pediatric intracranial tumors, and to establish DNA analysis as a potential prognostic parameter. Twenty-nine brain tumor specimens from 26 pediatric patients were cryo-preserved within a 3-year period. The DNA content was measured by flow cytometry. Six of the tumor specimens had aneuploid DNA patterns. The median of the proliferation index was lower in the survivor group compared with the non-survivor group (36.4% and 47.5%, respectively). Ten of the 26 patients are still alive, eight were lost to follow up, and eight died. Flow-cytometric DNA analysis may be a helpful tool for examining brain tumors in children. The small size of this study could not establish flow cytometry as a definite prognostic factor, but further prospective multicenter studies will evaluate the prognostic significance of flow-cytometric DNA analysis.

Adolescent↗

Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery.

OBJECTIVE: To retrospectively analyse the outcome of children with rhabdomyosarcoma (RMS) of the bladder, prostate or vagina who were treated with chemotherapy, with or without radical surgery or additional radiotherapy, at our institution since 1968. PATIENTS AND METHODS: From a total of 107 children with RMS seen between 1968 and December 2001, 22 (mean age 5.9, range 0.5-18) had RMS of bladder/prostate or vagina. Twenty of the patients received primary polychemotherapy (vincristine, actinomycin D, cyclophosphamide, adriamycin, and more recently including etoposide and ifosfamide), two had primary surgery and seven had additional radiotherapy. Fourteen patients had radical cystoprostatectomy, with continent cutaneous urinary diversion with an ileocaecal pouch in seven, in one each a transverse colonic pouch, orthotopic ileocaecal bladder substitution, a rectal reservoir and rectosigmoid pouch and a colonic conduit diversion in two patients. RESULTS: After a mean (range) follow-up of 8.6 (1.0-26) years, 17 patients had no evidence of disease. Five patients presenting initially with advanced tumour stages died from progressive RMS. Two patients with a continent urinary diversion required ureteric reimplantation for stenosis. In two patients severe bladder contraction after radiotherapy required bladder augmentation. CONCLUSION: Primary chemotherapy followed by radical surgery of RMS of the prostate and/or bladder allows complete tumour resection in most cases, and yields excellent cure rates.

Adolescent↗

Clinical aspects and prognosis of ependymoma in infants and children. A single institution experience.

Thirty-two patients (22 boys and 10 girls) with a histologically confirmed diagnosis of ependymoma were treated between 1972 and 1999. A total macroscopic resection was achieved in 16 of these patients, whereas 15 resections were classified by the surgeon as subtotal. In 1 patient a ventriculostomy was created as part of a palliative strategy. All children over 3 years old were treated with postoperative radiotherapy. Chemotherapy consisted of procarbazine, ifosfamide, etoposide, methotrexate, cisplatin and cytosine arabinoside. There was 1 perioperative death. Twenty children developed a relapse of disease within 2 months to 13 years and 1 month after the initial therapy. A maximal number of five recurrences were seen in 1 patient. The value of adjuvant chemotherapy on the prognosis of children with ependymoma seems to be limited. With regard to the poor outcome, the advisability of further treatment after multiple recurrences is debatable.

Adolescent↗

[Head circumference in relation to sonographic morphometry of the cerebral ventricles in neonates and infants].

AIM: To determine normal values of sonographic measurements of the brain in neonates and infants. METHOD: Cerebral ultrasonographic examinations were performed in 143 healthy newborns and infants. The size of the ventricles was determined by ascertaining distances as well as performing planimetric measurements of the ventricular area and circumference in standardized planes. RESULTS: Throughout the course of the study we observed a continous increase in size and a strong correlation with head circumference. The middle coronary plane used for the measurements allows the accurate representation of the ventricles and a more objective assessment of size. CONCLUSION: Based on the morphometric data, charts for the determination of ventricular size were developed.

Body Height↗

Survivors of childhood cancer for more than twenty years.

Present health status, complications, and development of long-term survivors of childhood cancer followed for more than 20 years in a single institution were reviewed. The departmental database was searched to identify patients diagnosed with childhood cancer and consequently treated between 1965 and 1978. A total of 124 (77%) long-term survivors participated on a voluntary basis in the study. A semi-standardized interview consisted of measures evaluating the present health condition, sequelae of treatment, second malignancies, intellectual development and presence of offspring of the former patients. The majority of patients were treated with chemotherapy (82%). 67% received radiotherapy and 67% underwent surgery. A relapse of the primary tumor was diagnosed in four patients as well as a second malignancy in four other patients. In 33% of the long-term survivors one or more serious therapy-related health problems were noted. Adequate mental and intellectual development was achieved in 65%. Children treated in the early years of pediatric oncology seem to have a satisfactory outcome as viewed over the long term. Consequent ongoing follow-up is still necessary to detect health problems and enhance quality of life for subsequent generations of children with cancer.

Central Nervous System Neoplasms↗

Primary mediastinal germ cell tumors in children and adolescents: results of the German cooperative protocols MAKEI 83/86, 89, and 96.

PURPOSE: To evaluate children and adolescents with primary mediastinal teratoma and malignant germ cell tumors (GCTs). PATIENTS AND METHODS: Forty-seven patients from the German nontesticular GCT studies were analyzed (median age, 2.5 years; range, neonate to 17 years). Teratoma (n = 21) were resected, and no adjuvant treatment was given. Malignant GCTs (n = 26) were treated with cisplatin-based chemotherapy and resection. Three of 26 patients underwent radiotherapy. RESULTS: In all patients with teratoma, tumor markers were normal. Surgery of teratoma was complete in 17 of 21 patients and microscopically incomplete in four of 21 patients, and we observed no relapse after a median follow-up of 29 months. In 23 of 26 patients with malignant GCTs, alpha-fetoprotein and/or beta-human chorionic gonadotropin were elevated. Twelve of 26 patients received adjuvant chemotherapy after initial resection, which was complete in six of 12 patients, whereas delayed resection after preoperative chemotherapy was complete in 10 of 11 patients (P =.03). Four of six patients underwent second-look thoracotomy after incomplete primary surgery. Three of 26 patients did not undergo tumor resection. The final completeness of resection was the strongest prognostic indicator (event-free survival ¿EFS, 0.94 +/- 0.06 v 0.42 +/- 0.33; P <.002). Local stage and distant metastases were not prognostically significant at the.05 level. For all malignant GCTs, the 5-year survival rate was 0.87 +/- 0.05 (median follow-up, 51 months), with an EFS of 0.83 +/- 0.05. CONCLUSION: The prognosis of mediastinal teratoma is excellent after complete or microscopically incomplete resection. In children with malignant GCT, the prognosis is favorable with a therapeutic strategy of delayed resection after preoperative chemotherapy. In most children, the diagnosis can be based on elevated tumor markers and imaging. Biopsy is indicated in nonsecreting GCT.

Adolescent↗

Secondary thyroid carcinoma after treatment for childhood cancer.

BACKGROUND: Second malignant neoplasms (SMNs) have become a primary concern in evaluating long-term effects of treatment in pediatric oncology. Thyroid carcinoma has proven to be a common SMN. METHODS: In a multicenter study involving 58 hospitals in Germany, Austria and Switzerland, 18 of 239 (7.5%) SMNs documented following first malignant neoplasm (FMN) in childhood were thyroid carcinoma. RESULTS: The age at diagnosis of FMN ranged from 1 to 15 years. Eleven patients were female. Six children had survived Hodgkin disease, seven acute leukemia, two Ewing sarcoma and three various other tumors. Fifteen of the 18 patients had been treated with radiotherapy to the head and neck region. The time interval between treatment and diagnosis of thyroid carcinoma ranged from 4 to 19 years (median 8 years). The carcinoma was papillary in 17 cases and follicular in one. Eleven patients had metastases in the regional lymph nodes at presentation. DISCUSSION: Radiotherapy appears to be an important risk factor in secondary thyroid carcinoma, but it does not explain all cases. The current data are remarkable for the large proportion of patients who received only prophylactic cranial irradiation for ALL and for the three patients who received no irradiation to the head and neck region. Genetic determinants and chemotherapy must also be considered. CONCLUSIONS: Regular thyroid examination should be included in the long-term follow-up of survivors of childhood malignancy.

Adolescent↗

Physical performance in long-term survivors of acute leukaemia in childhood.

UNLABELLED: The aim of this study was to assess the physical performance in long-term survivors of acute leukaemia in childhood and to evaluate the effects of anthracycline therapy. Electrocardiography, echocardiography and spiroergometry were carried out on 56 patients aged 9-28 years, of whom 44 patients had been treated with 15-483 mg/m2 doxorubicin (or equivalent). Acute leukaemia had been diagnosed 1.5-16 years earlier. Of the patients 75% reached normal maximal oxygen uptake, 69% normal oxygen uptake at the anaerobic threshold and 95% normal maximal work rate. Of the patients 75% achieved adequate values for maximal heart rate and 78% normal blood lactate concentration. No difference was seen between patients treated with and without anthracyclines. CONCLUSION: The results of this study provide little evidence for cardiopulmonary impairment in long-term survivors of ALL. Both the cardiac function, as evaluated by ECG and echocardiography, and the physical performance in spiroergometry are normal in a large number of these patients. Anthracycline treatment does not appear to have a negative effect on these parameters.

Adolescent↗

Paediatric oncology.

Recent research into paediatric urologic oncology has identified a number of certain subgroups of neuroblastoma and Wilms' tumour, which allows a more tailored treatment of the individual child with respect to treatment sequelae and finance issues. The importance of surgery, radiotherapy and cytostatic treatment has to be individualized. The controversies regarding the management of rhabdomyosarcoma of the bladder and the prostate remain unresolved.

Journal Article↗

[Rhabdomyosarcoma of the orbits in childhood].

BACKGROUND: Rhabdomyosarcoma is the most common malignant orbital tumor in children. Treatment modalities (individualized therapy or study protocol) have been changed radically. Surgery was supplemented by radiation therapy and chemotherapy. The objective of our retrospective analysis was to define the prognosis in correlation to changes of treatment in an unselected patient group of a single institution. PATIENTS AND METHODS: Between 1954 and 1995, 18 patients (age at presentation 1 month to 17 years, 11 male and 7 females) with orbital rhabdomyosarcoma were diagnosed and treated at our institution. RESULTS: The 5-year survival rate was 76%. Primary exenteration was replaced by tumor resection (microsurgery), radiation therapy and chemotherapy. Favorable outcome in the precytostatic era (with permanent loss of function) was not significantly improved by polychemotherapy. Microsurgery seems to increase the risk of recurrence. CONCLUSION: Considering the high risk of recurrence, primary exenteration in selected patients with expanded primary tumor is still a therapeutic option.

Adolescent↗

Cerebral haemorrhage in long-term survivors of childhood acute lymphoblastic leukaemia.

Modern treatment of childhood acute lymphoblastic leukaemia (ALL) has dramatically improved the prognosis for children with this disease. Therapeutic approaches consist of multimodal chemotherapy and radiotherapy with significant long-term side-effects. We report on 4 children out of a group of 120 newly diagnosed patients with ALL, who survived the disease for more than 2 years and developed a cerebral haemorrhage after chemotherapy and fractionated cranial irradiation. Following a period of 2-12 years the four children presented with acute neurological signs and symptoms. i.e. seizures, ataxia and hemiparesis. CT and MRI revealed intracerebral mass lesions, interpreted as haemorrhage. After neurosurgery the patients neurological state improved. Histological examination confirmed the suspected diagnosis of bleeding cavernous haemangioma or capillary telangiectases. There are two possibilities to explain these rare alterations: they may be pre-existent to the disease and therapy or they may be caused by irradiation. CONCLUSION Acute neurological symptoms in patients treated for ALL may be caused by spontaneous cerebral haemorrhaging of cavernous haemangiomas or capillary telangiectases induced by chemotherapy and/or radiotherapy.

Adolescent↗

[Rosai-Dorfman lymphadenopathy, a rare reactive lymphoproliferative disease. How valid are ultrasound morphologic criteria in lymph node involvement?].

AIM: The established criteria for benignity or malignancy of lymph nodes when evaluated with B-mode ultrasound can be divided into quantitative measures (size, quantity, contour and density) and qualitative parameters (configuration and structure). The validity of these criteria is controversial. METHODS: Two cases of sinus histiocytosis with massive lymphadenopathy (SHML) are presented; this is a rare benign reactive disorder in which massive enlargement of cervical lymph nodes often occurs. These enlarged nodes offer a chance to demonstrate and discuss the validity of the sonomorphological criteria. RESULTS: The enlarged lymph nodes in SHML fulfill most of the criteria for malignancy. The same results are meet in other disorders with prominent but benign lymphadenopathy. CONCLUSION: The current ultrasonic criteria to determine benignity/malignancy in lymph nodes most likely simply indicate a large number of enlarged nodes. Pathologic diagnosis remains the gold standard.

Child↗