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Biomedical subjects

U Willnow

Publications and source records attributed to U Willnow.

At least 19 recordsLinked to original sources

Therapeutic strategy of papillary cystic and solid neoplasm (PCSN): a rare non-endocrine tumor of the pancreas in children.

Papillary-cystic and solid neoplasm (PCSN) are rare tumors. Two personal observations and a review of the literature are presented with a total of 44 pediatric patients in addition to a total of 67 published cases in the review of Cohen (Pediatr. Surg. Int. 6 (1991) 128) and Snadjauf (Eur. J. Pediatr. Surg. 9 (1999) 416). Overall, PCSN shows a clear predominance in females and only occasionally occurs in males. Typically they grow to a large tumor mass with minimal symptoms. Their histologic and immunocytologic characteristics cause diagnostic difficulties, especially on frozen sections of small biopsies. The tumors are assumed to origin from pluripotent stem cells and present as tumors of low malignancy with a favorable prognosis. Nevertheless 10 children have been reported to develop metastases, 5 have demonstrated an invasive growth pattern and 4 local recurrence. But only two of the 111 pediatric cases have died from their tumor burden. Treatment of choice is a complete surgical resection, which is true for the primary tumor and for metastases as well as local recurrences. In our 2 patients one had spleen-conserving left pancreatic resection and one mesopancreatectomy with roux-en-y-reconstruction leading to long-term cure. Adjuvant therapy in curative resected patients is unnecessary and does not appear to improve prognosis.

Adolescent↗

Neurogenic appendicopathy in children.

BACKGROUND: In a prospective multicenter study, we could show that neurogenic appendicopathy is a histological entity. This study compares the general and the pediatric population with respect to clinical presentation and incidence of neurogenic appendicopathy (NA). METHODS: Included were patients that underwent appendectomy for suspected appendicitis, excluded were patients younger than 6 years and patients with missing data. Neurogenic appendicopathy was diagnosed by S-100 immunochemistry and/or haematoxylineosin (H.E.) staining. Two age groups (< or = 14 y and > 14 y) were compared with respect to the frequency of NA. RESULTS: In only four cases out of 84 children (4.8%) did we find neurogenic appendicopathy compared to 48 patients (24.2 %) out of 198 adolescents and adults. In the subgroup with negative appendectomy, the frequency of NA was 16.7% (< or = 14 years) and 56.6% (> 14 years). A clinical differentiation between neurogenic appendicopathy and acute appendicitis was not possible because of the small sample size. CONCLUSION: Neurogenic appendicopathy is a very rare histopathological entity in children. History and clinical examination do not make it possible for us to differentiate preoperatively between acute appendicitis and neurogenic appendicopathy.

Adolescent↗

Fibrosarcoma in infants and children: a retrospective analysis - overdiagnosis in earlier years.

During a 30-year period, 22 patients considered to have a fibrosarcoma (FS) were treated. In a retrospective study the clinicopathologic findings were summarized. With histologic and immunohistochemical re-evaluation, the diagnosis was confirmed in 8 cases. For 6 further patients FS was very probable but specimens were not available. In 8 cases the diagnosis was revised and benign lesions were found in 7. Two patients with irresectable tumors died (infantile FS, FS of mesentery and retroperitoneum). After repeated local recurrences and spread on the affected extremity, an amputation was life-saving in 1 boy. In earlier years many tumors were classified as FSs. Today, immunohistochemistry and molecular-biological methods are valuable tools to clearly identify these tumors. Wide local excision or en-bloc resection without sacrificing any significant function of the part should be the primary form of treatment in infants. Primary re-excision after incomplete excision should have priority over any adjuvant treatment. Preoperative chemotherapy may avoid incomplete resection or mutilation in cases with extended congenital FS.

Child↗

[Implantable catheter systems].

Central venous access devices represent a definitive improvement in childhood cancer therapy. The silicone elastomer catheters offer a both save and easy access, low related morbidity and acceptance by patient. Tunnelled right atrial catheter (Broviac, Hickman-Crawford or Quinton) for long-term use are firstline choice for venous access. Although ports reduce the number of complications and manipulations associated with percutaneous catheters, they are not devoid of problems. Related complications are catheter infection and occlusion or damage. The different techniques of venous vascular access and management of complications are reported.

Antineoplastic Agents↗

Treatment of children with relapsed soft tissue sarcoma: report of the German CESS/CWS REZ 91 trial.

BACKGROUND: The present study was performed to evaluate the possibilities of relapse treatment in patients heavily pretreated for a soft tissue sarcoma. PATIENTS AND METHODS: Prospective, multicenter study in 44 soft tissue sarcoma (STS) patients with first relapse. Primary diagnosis was embryonal rhabdomyosarcoma (RME) in 17 patients, alveolar rhabdomyosarcoma (RMA) in 13, primitive neuroectodermal tumor (PNET) in 6, and miscellaneous soft tissue sarcomas in 8 patients. Initial chemotherapy consisted of carboplatin/etoposide combination (150 mg/m2 each, days 1 to 4) followed by local therapy including surgical treatment and, whenever possible, radiotherapy. RESULTS: In 11/17 patients without primary tumor resection, CR or PR was achieved following the initial two cycles of chemotherapy (61%). The probability of event-free survival (pEFS) for RME patients was 0.41 +/- 0.12 at 5 years, and 0.25 +/- 0.12 for RMA patients. But, in contrast no PNET patient or patient with another soft-tissue sarcoma achieved long-term remission. Additional local radiotherapy significantly (P = 0.002) improved pEFS (3-year estimates of 0.23 +/- 0.2 vs. 0.1 +/- 0.1 in patients without radiotherapy). CONCLUSIONS: In patients with RME, relapse treatment employing a carboplatin/etoposide combination may induce a second remission in approximately 40% of patients. Surgical excision and additional local radiotherapy seem to be essential to maintain a stable remission. In patients with RMA or PNET, however, this treatment strategy is of no long-term benefit.

Antineoplastic Agents, Alkylating↗

Therapeutic management of rare malignant pancreatic tumors in children.

Malignant pancreatic tumors in children are rare. The major problem for the clinician is a lack of experience and of accepted therapeutic strategies. Malignant pancreatic tumors in children show a pattern different from that in adults. In infants, especially pancreatoblastomas, solid cystic tumors of females, and endocrine carcinomas of the pancreas must be expected. We report our experience in three patients with malignant pancreatic tumors (one pancreatoblastoma and two malignant endocrine pancreatic carcinomas) and review the present literature with a focus on the typical clinical and biologic features and the presently recommended therapeutic strategies. Pancreatoblastomas and solid cystic tumors are mainly found in the head of the pancreas. Fibrotic capsules with rare, late metastases are characteristics of these tumors, indicating total resection to be an important therapeutic procedure. Pancreatoblastomas should additionally be treated with chemotherapy (ADM, IFO, cis-PL, VP16). Endocrine carcinomas of the pancreas (malignant gastrinomas and malignant insulinomas) should also primarily be treated with radical surgery, including extensive lymph node dissection. In case of distant metastases, local resection (liver) or somatostatin in combination with chemotherapy (streptozocin in the case of malignant insulinomas) may be used.

Adolescent↗

Pancreatoblastoma in children. Case report and review of the literature.

Pancreatolblastomas are rare embryonal malignancies in childhood. We report a 3-year-old girl with a tumor of the head of pancreas. Staging by bone scintigraphy and CT scans of abdomen and chest did not show evidence of metastatic disease. Tumor markers showed elevated levels of alpha-1 fetoprotein (64 ng/ml; normal 0-10 ng/ml) and lactate dehydrogenase (423 U/l; normal range below 300 U/l). The tumor was macroscopically completely removed by local resection. Postoperative tumor grading was pT1, NO, MO. The child recovered very soon after surgery without severe complications. Tumor markers dropped to normal values, indicating complete remission (follow-up time 12 months). According to the biological growth characteristics of pancreatoblastomas and to the literature, localized and non-metastatic tumors should be completely resected without radical pancreatoduodenectomy and without adjuvant chemotherapy. This is the most conservative therapy with a good prognosis. However, metastatic disease, primarily inoperable conditions or local relapses are indications for chemotherapy combined with radiotherapy and followed by resection of the tumor. At present, the prognosis of such cases is rather poor.

Child, Preschool↗

[In vitro studies of DNA synthesis inhibition by hyperthermia (42.5 degrees C, 120 min) and cytostatic drugs in embryonal tumors and sarcomas in childhood].

The effect of hyperthermia (HT), (42.5 degrees C, 120 min), cytostatic agents and the simultaneous application of HT and cytostatics on embryonal tumors and sarcomas in children was studied using an autoradiographic in vitro method. The 3H-thymidine and 3H-uridine incorporation inhibition was measured. HT inhibits DNA and RNA synthesis in a range of 42.0%-51.0% and 45.6%-58.3%. HT enhances the effect of the cytostatic agents ADM, CPM, cis-PL, BLM and BCNU highly significantly. In each case and without exception the simultaneous application of HT and cytostatics overcomes primary resistance to HT or the cytostatic concerned.

Antineoplastic Agents↗

Total body hyperthermia in combination with etoposide and melphalan in a child with acute myelomonocytic leukemia.

In vitro and clinical studies have shown antineoplastic effects of hyperthermia alone and in combination with other treatment modalities. Synergistic cytotoxic effects of chemotherapy and hyperthermia have been demonstrated on leukemic cell clones in vitro. It seems that hyperthermia is effective in overcoming chemotherapy resistance. Several groups treated solid tumors by using total body hyperthermia (TBHT). However, only a few studies have been reported investigating the clinical effects of TBHT in myeloproliferative disorders. We report the case of a 7-year-old boy with myelomonocytic leukemia treated with TBHT (2 hours, 42 degrees C) combined with etoposide (600 mg/m2), melphalan (30 mg/m2) and hyperglycemia (200-300 mg/dl). Within 24 hours after TBHT, the leukemic cells decreased after TBHT from 53,000/microliters to zero. Skin leukemic infiltrates, resistant to conventional treatment, also responded well. Although our patient relapsed 34 days after TBHT, these results indicate that TBHT in combination with cytotoxic treatment may be a useful treatment modality in refractory leukemia.

Antineoplastic Combined Chemotherapy Protocols↗

Pre-emptive analgesia: comparison of preoperative with postoperative caudal block on postoperative pain in children.

We have compared in 25 children the effect of preoperative with postoperative caudal block on pain after circumcision in a double-blind, randomized study. After induction of anaesthesia, patients were allocated randomly to receive a caudal block either before (n = 14) or immediately after (n = 11) surgery. Postoperative pain was rated on a paediatric pain scale. If pain occurred, children received paracetamol in a dose related to body weight. Using the Mann-Whitney U test (significance < or = 0.05) there was no significant difference in cumulative postoperative analgesic requirements within the first 48 h and in times to first analgesic administration between the groups. Cumulative pain score, assessed every 30 min for the first 8 h after operation, was significantly lower for those patients who received caudal anaesthesia after operation. Thus we could not demonstrate any advantage in performing caudal block before compared with after surgery.

Acetaminophen↗

[Effect by proliferation kinetics of malignant tumors of children of hyperthermia].

Embryonal tumors (21 Wilms' tumors, 11 neuroblastomas, 9 rhabdomyosarcomas) and 16 sarcomas of the skeleton of childhood were studied with an autoradiographic in vitro method according to the responsibility to hyperthermia 42.5 degrees C/120 min, to Cyclophosphamide, to Doxorubicin, and to Actinomycin D, and also to the combined application of hyperthermia and cytostatic drugs. Hyperthermia alone reduced the median 3H-thymidine, respect. 3H-uridine incorporation between 38.1 and 56.8%, respect, 45.9 and 63.9%. Hyperthermia inhibited the RNA synthesis stronger than the DNA synthesis. The combined application of hyperthermia and the different cytostatic agents increased the inhibition of incorporation of the labeled precursors extraordinarily (by Cyclophosphamide to 81.6-90.8%, by Doxorubicin to 60.8-80.0, by Actinomycin D to 79.4-93.2%). In case of the inhibition of 3H-thymidine or 3H-uridine incorporation lower than 40% (resistance), in each case the combination of hyperthermia and cytostatic drug overcomes the resistance. The consequences of hyperthermia sensitivity testing of solid tumors of human beings are discussed.

Autoradiography↗

[Treatment of otherwise incurable tumor diseases in childhood using whole-body hyperthermia and chemotherapy].

Conventional methods of treatment having failed in 17 children (aged 9/12 to 16 5/12 years) with incurable solid malignant tumours underwent whole-body hyperthermia (41.8-42.0 degrees C, for 2-3 h), hyperglycaemia (20-25 mmol/l) and polychemotherapy. Five children had neuroblastoma (stage 4), three Wilm's tumour (stage 4 or 5, unfavourable histology), five skeletal sarcoma with metastases, three inoperable malignant liver tumour, and one brainstem tumour of unknown histology. Whole-body hyperthermia was induced by extracorporeal blood warming in an haemodialysis apparatus under neuroleptic analgesia, thermistors measuring the temperature in the oesophagus, rectum, trachea and skin. There were on average four treatment sessions (between one and ten, total 58), a week apart. The result could be assessed in 12 children: one persisting complete remission (19 months-metastasising renal rhabdoid tumour), eight partial or incomplete remissions, and three nonresponders (osteogenic sarcoma; Ewing sarcoma; brainstem tumour). If the risk can be satisfactorily judged the method is useful and of bearable toxicity. The results point to a high antitumour effectiveness of combined hyperthermia and chemotherapy.

Adolescent↗

[Malignant fibrous histiocytoma of the mandible in a 1 6/12-year-old boy].

A report on malignant fibrous histiocytoma of the mandible in a 1 6/12-year-old boy, a condition rarely seen in children. Because of inoperability radiotherapy was used resulting in complete tumour remission. The clinical course was complicated by a tracheo-oesophageal fistula and aspiration pneumonia. The spontaneous closure of the fistula occurred 5 months after tracheostomy and catheter jejunostomy. After 2 10/12 years there is no evidence of tumour disease. Possibilities and problems of therapy are discussed.

Child↗

[Simultaneous occurrence of Wilm's tumor and multiple lung hamartomas in a 15-year-old girl].

The observation of multiple adenofibroleiomyomatous hamartomas of the lung in a 15-year-old girl 7 years after treatment of a Wilms' tumour caused diagnostic difficulties that are discussed. The diagnosis was finally established by thoracotomy and tumour resection. The simultaneous occurrence of Wilms' tumours and benign or malignant tumours points to genetic factors in the development of both tumours.

Adolescent↗