Josef Hager

Comparison of low-molecular-weight heparin and antithrombin versus antithrombin alone for the prevention of symptomatic venous thromboembolism in children with acute lymphoblastic leukemia.

Children with acute lymphoblastic leukemia (ALL) have a substantial risk for thromboembolism (TE) that is related to L‐asparaginase‐induced antithrombin (AT) deficiency and placement of central venous lines. Recent in vitro studies showed that the anticoagulant effects of low‐molecular‐weight heparin were profoundly affected by endogenous AT levels in children undergoing ALL therapy.

Congenital Fibrosarcoma as Cause for Fetal Anemia: Prenatal Diagnosis and in utero Treatment

Objectives: To discuss diagnosis and management of a case of a rare fetal tumor complicated by fetal anemia due to intratumoral hemorrhage. Case Report: We report on a 29-week-old fetus with a tumor in the posterior left shoulder region. The morphologic aspect of the tumor, lack of fetal movements and an increased middle cerebral artery (MCA) peak systolic velocity (PSV) were indicative of fetal anemia caused by intratumoral bleeding. Following intravascular blood transfusion the pregnancy was safely prolonged for 15 days, during which lung maturity was induced. After delivery the neonate underwent surgical excision. Histological examination revealed an infantile congenital fibrosarcoma. Conclusion: Anemia must be ruled out in cases with fetal tumors. MCA PSV is useful in diagnosis and surveillance in these fetuses.

Solid-pseudopapillary tumor of the pancreas in a 12-year-old girl – 7 years follow-up and histopathological reevaluation: Case report and subject review

ZusammenfassungGRUNDLAGEN: Ein solid-pseudopapillärer Tumor des Pankreas (sog. Frantz-Tumor) ist ein seltenes, typischerweise sich benigne verhaltendes Neoplasma, das vorwiegend bei jungen Frauen, aber kaum einmal bei jungen Mädchen vorkommt. METHODIK: Es wird die Krankengeschichte einer 12jährigen Patientin, die wegen eines solchen Tumors operiert worden war, vorgestellt und in Konsequenz auf die diagnostischen und therapeutischen Vorgehensmöglichkeiten eingegangen. Zudem wird die Bedeutung der histologischen Tumorklassifikation bzw. der aktuellen immunhistochemischen Untersuchungen, auch in Bezug auf ein neues, eventuell besseres Tumorverständnis, unter Berücksichtigung der einschlägigen Literatur diskutiert. Das eben erwähnte Mädchen wurde vor 7 Jahren wegen akut aufgetretener und in der Folge persistierender Oberbauchschmerzen an unserer Abteilung vorgestellt. Bei normalem klinischen Befund und unauffälligen Routinelaborparameter zeitigte die Ultraschalluntersuchung des Abdomen einen kugeligen, im Durchmesser etwa 8 cm haltenden Tumor mit soliden und zystischen Arealen, ohne Anhalt für Metastasen. Die Kombination der zwei Fakten – junges Mädchen und solid-zystische Läsion des Pankreas – ließ an einen Frantz-Tumor denken. Mittels CT und MRI des Abdomens konnte die Verdachtsdiagnose erhärtet werden. Das Mädchen wurde einer Kausch–Whipple Operation (modifiziert nach Traverso–Longmire) unterzogen, um eine radikale Tumorentfernung zu realisieren. ERGEBNISSE: Die klinische und radiologische Verdachtsdiagnose wurde histologisch bestätigt, d. h. die Läsion wurde als solid-pseudopapillärer Tumor des Pankreas bewertet. Der Verlauf nach diesem Eingriff war bislang komplikationslos, sowohl postoperativ wie auch im Langzeitverlauf (Follow-up von 7 Jahren). Anlässlich der letzten Kontrolluntersuchung (April 2009) wurde eine immunohistochemische Reevaluierung des Tumormaterials mit den derzeit zur Verfügung stehenden Möglichkeiten durchgeführt, neue Aspekte in Bezug auf die Tumorentstehung waren aber nicht zu realisieren. SCHLUSSFOLGERUNGEN: Solid-pseudopapilläre Tumoren des Pankreas kommen selten vor. Das Gros dieser Tumoren verhält sich, obwohl eigentlich maligne, benigne; maligne Verläufe sind aber bekannt. Eine klinische Verdachtsdiagnose ist trotz ihres seltenen Vorkommens möglich, basierend auf den Fakten, dass diese Tumoren typischerweise junge Frauen betreffen und bildgebend solide und zystische Areale zeigen. Eine radikale Tumorresektion gilt als Methode der Wahl. Eine Tumorenukleation ist wegen des Risikos einer Tumoreröffnung bzw. der Entstehung von Pankreasfisteln umstritten. Trotz spezieller immunhistochemischer und genetischer Untersuchungen ist die Herkunft dieser Tumoren nach wie vor unbekannt.SummaryBACKGROUND: Solid-pseudopapillary tumor of the pancreas (so-called Frantz tumor) is a rare neoplasm of typically benign behavior, predominantly occurring in young females, but very rare in girls. METHODS: The clinical course of a 12-year-old girl suffering from such a tumor is presented and diagnostic and therapeutic methods are pointed out. Furthermore the relevance of the histological tumor classification respectively of recent immunohistochemical investigations with consideration of the actual literature will be discussed. The mentioned girl was admitted to our department of pediatric surgery because of upper abdominal pain, persistent for 12 hours. Initial examinations (physical examination, routine laboratory parameters, and abdominal ultrasound) showed a well-defined, spheric tumor (8 cm in diameter) with solid and cystic areas, without hints for metastases. The combination of the two facts – young girl and solid-cystic lesion of the pancreas – made us think about a Frantz tumor. By performing CT and MRT of the abdomen this diagnosis was hardened. In fact the patient underwent a Kausch–Whipple operation (modified by Traverso–Longmire) to perform a radical tumor removal. RESULTS: The clinical and radiological diagnosis was confirmed by histological examination of the tumor, i.e., the lesion was valued as solid-pseudo papillary neoplasm of the pancreas. Neither short-term or long-term surgical complications, nor metastases have been recorded in the follow-up (7 years up to now). An immunohistochemical reevaluation of the tumor material was carried out on occasion of the recent follow-up (April 2009); new aspects concerning tumor genesis could not be realized. CONCLUSIONS: Solid-pseudo papillary tumors of the pancreas are rare tumors. The majority of these tumors show benign behavior; very few malignant clinical courses have been reported. Despite rare occurrence of the tumor clinical presumption diagnosis possible, based on the fact that these tumors particularly affect young females and radiological findings show a tumor with solid and cystic parts. Radical tumor resection is the therapeutic method of choice. Because of the risk of injuring of the tumor an enucleation is discussed controversial. Despite special immunohistochemical and genetic examinations (influences of estrogenic hormones?) the origin of the tumor remains unknown.

Editorial: Pediatric surgery – topics of interest for general surgeons

In Europe, pediatric surgery was part of general surgery for a long time. Not till the sixties of the last century, three years of specialized training in pediatric surgery in addition to the complete training for general surgery were mandatory to become a certified pediatric surgeon in Austria. At the beginning of the nineties, pediatric surgery became an autonomous discipline. This led to an improved quality of care for our young patients, such as a dramatic improvement of the survival rate of premature infants from 10 to 90 percent, all this together with an improved quality of life. A deep understanding of the embryologic development and the changes of the different organ systems during growth and maturation is needed in order to conform to the needs of these patients. This calls for sub-specialization without any doubt. Therefore, pediatric surgery can be subdivided into four sub-specialities: (1) surgery of the newborn (2) general pediatric surgery (3) pediatric traumatology (4) pediatric urology. Nevertheless, the pediatric patients grow up and – by the time, as adults – they might need surgery. In order to comply with some particular clinical situations, general surgeons need to concern themselves with the medical/surgical history of these patients. Therefore, general surgeons need a certain degree of knowledge about pediatric surgery. Sometimes, they will have to consider a pediatric surgeon to be able to manage a patient’s problem appropriately. The aim of this main topic of “European Surgery” is to spark interest in pediatric surgery to all “adults’ surgeons” and to build a bridge between general and pediatric surgeons. Finally, I want to thank Dr. HeinzWykypiel, Medical University Innsbruck and also the staff of Springer Medizin for their kind assistance in the realization of this project.