Prenatal sonographic detection of a giant multiseptate hepatic cyst in the third trimester.
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Adult↗
Biomedical subjects
E Horcher
Publications and source records attributed to E Horcher.
Intratumoural heterogeneity of 1p deletions and MYCN amplification in neuroblastomas.
BACKGROUND: At least three genetic hallmarks identify aggressive tumour behaviour in neuroblastomas; amplification of the oncogene MYCN; deletion (loss of heterozygosity [LOH]) at the short arm of chromosome 1 (del1p36), seen in approximately 28% of the cases; and di-tetraploidy. The MYCN oncogene is amplified in approximately 23% of all neuroblastomas and becomes important for the stratification of therapy in localised and 4s tumours. Up to now, it has been believed that the genetic constellation of neuroblastic tumours is stable and does not alter during tumour evolution or during tumour progression. PROCEDURE: Using fluorescence in situ hybridisation techniques (FISH) to investigate different tumour areas on touch preparations and histological sections, we show that genetic heterogeneity can be detected in neuroblastomas, especially in tumours detected by urinary mass screening. CONCLUSION: The identification of such cell clones is important, because the MYCN amplification and/or the deletion at 1p36 appear to be responsible for aggressive local growth and development of metastases.
Chromosomes, Human, Pair 1↗
Solid-pseudopapillary tumor of the pancreas (Frantz tumor) in children: report of four cases and review of the literature.
BACKGROUND: Solid-pseudopapillary tumor of the pancreas (SPT) is an exceptionally rare neoplasm in children. Its origin remains enigmatic. It is of low malignant potential and occurs most frequently in young females. PATIENTS AND METHODS: A cumulative review of the tumor's clinicopathological characteristics from the world's literature is presented. The clinical course, pathohistologic data and outcome of surgery of four Austrian children treated at the general hospital of Vienna are analyzed. RESULTS: Between 1987 and 1999, four girls (age: 12--16 years) with SPT were diagnosed at our institution. All patients presented with an abdominal mass and uncharacteristic abdominal pain. Two tumors were located in the tail, one in the body and tail and one in the head of the pancreas (diameter: 7--15 cm). Surgical procedures included three distal pancreatectomies and one partial duodenopancreatectomy (Whipple procedure). One patient had two recurrences with metastases that could only be partially resected. Chemotherapy was initiated for this patient. In the follow-up period (range: 6 months to 12 years) all patients are alive with no evidence of recurrence. CONCLUSIONS: SPT is a rare differential diagnosis of a pancreatic mass in children. It is mandatory to establish this diagnosis since complete surgical removal of the tumor even in case of metastases or local invasion offers an excellent prognosis.
Adolescent↗
Expression of cytokeratin-18-related tissue polypeptide-specific (TPS) antigen in Wilms tumor.
BACKGROUND: So far, there is no approved tumour marker for diagnosis or follow-up in Wilms tumour (WT). Tissue polypeptide-specific antigen (TPS), a cytokeratin 18 proteolytic fragment, has been suggested to be of value in the clinical management of WT patients. Cytokeratin 18 fragments are an early indicator of apoptosis and cytokeratin 18 might influence tumour cell behaviour. We investigated TPS expression in specimens of WT and other paediatric renal malignancies PROCEDURE: Immunoreactivity of WT sections (n = 9), clear cell sarcomas (CCSK, n = 3), and a renal cell carcinoma (RCC), and two pediatric kidney tumour cell lines (WT: SK-NEP-1 and rhabdoid tumour of the kidney: G-401) were investigated using the monoclonal antibody M3. Additionally, immunoblotting and RT-PCR analysis were performed. Cell culture supernatants were evaluated for TPS release. Serum TPS was measured in five patients at diagnosis, during chemotherapy and after surgical resection. RESULTS: Moderate to strong immunoreactivity for TPS was found in tubular and blastemal components of nearly all (8/9) WT specimens. This was confirmed by Western-blotting. Cystic and epithelial-like portions of CCSKs and RCC showed distinct reactivity (3/3). The supernatant of G-401 but not of SK-NEP-1 showed a time- and cell number-dependent increase of TPS release. Interestingly, TPS synthesis was demonstrated in SK-NEP-1 cells. Median preoperative serum TPS was elevated (293 U/l) compared to healthy children and lowest after surgical resection (49.5 U/l). CONCLUSIONS: This is the first study demonstrating the synthesis and release of TPS by WTs and other paediatric renal malignancies. Considering the elevated levels of TPS in serum of these patients, a further investigation of this marker by larger clinical trials seems to be justified.
Adolescent↗
Bilateral Wilms' tumor--surgical aspects.
Four to eight percent of all Wilms' tumors are bilateral. Achieving curative resection of such tumors by partial nephrectomy or tumor enucleation while maintaining sufficient renal function represents a surgical challenge. Effective preoperative chemotherapy facilitates this aim considerably. Seven patients who were diagnosed to have bilateral synchronous Wilms' tumors between 1990 and 1994 are being reviewed. At the time of initial diagnosis, their mean age was 24.4 months, range 7 to 45 months. In 4 cases, pre-operative imaging did not reveal the full extent of the lesions, and in one of these, involvement of the second kidney was only detected by surgical exploration. Five of the children received at least 4 weeks of neo-adjuvant chemotherapy without primary biopsy, followed by bilateral tumor resection. One child had to be operated on as an emergency without any preliminary treatment, and in one, chemotherapy was interrupted after 3 weeks because of veno-occlusive disease. After a follow-up period of 66 months on the average (range 50 to 81 months), five of the patients are free of recurrence and clinically well--one with a kidney graft. The remaining two patients have died. Discussion is focussed on different management strategies of this rather rare pathology considering SIOP und NWTS protocols.
Antineoplastic Combined Chemotherapy Protocols↗
[Persistent hyperinsulinemic hypoglycemia (PHHI)--surgical management of neonatal hypoglycemia].
Sporadic persistent hyperinsulinemic hypoglycaemia in a newborn is a rare disease which requires early surgical treatment if conservative therapy fails. We report on a newborn female in whom PHHI was diagnosed at the second day of life. She presented with severe hypoglycemia and high levels of plasma insulin. Conservative treatment failed and a near-total pancreatectomy was performed on the 24th day of life. The postoperative outcome and the follow-up studies showed the patient to be euglycaemic. If hypoglycaemia is resistant to conservative therapy, surgical treatment is recommended. A loss of pancreatic exocrine function is not necessarily to be expected.
Child, Preschool↗
[Isolated rupture of the jejunum after trauma in a child. A complex mechanism of injury with difficult diagnosis].
Isolated rupture of the lower jejunum after blunt abdominal trauma is rare. The pediatric population, especially school-aged children, sustains these isolated injuries due to blunt trauma more frequently than adults. The lack of severe clinical symptoms in the beginning and therefore a delay in diagnosis are the main problems of these traumatic injuries. We report on a 7-year-old girl who sustained isolated rupture of the lower part of the jejunum secondary to blunt trauma. The purpose of this report is to illustrate the complex mechanism of injury. Additionally, we discuss the different ways of diagnosis with a review of the literature.
Abdominal Injuries↗
Perforating appendicitis. An indication for laparoscopy?
BACKGROUND: This pilot study was performed to reassess the widespread postulate that laparoscopic surgery is contraindicated in cases of perforating appendicitis. METHODS: A total of 75 children (appendiceal perforation: n = 67; perityphlic abscesses and peritonitis: n = 8) were analyzed in a prospective nonrandomized trial. Ten of them were treated by laparoscopic appendectomy. RESULTS: In the laparoscopy group, both pain and hospitalization were less time-consuming (i.e., by 50% and 19%, respectively). Antibiotics use was down from 2.6 over 6 days to 2.2. over 5.5 days, while the duration of surgery was up by 52%. Wound healing disturbances occurred in 10% (n = 1) and postoperative fever in 50% (n = 5) of patients, compared to 14% (n = 9) and 15% (n = 10) in the group treated by open surgery. All severe complications requiring reintervention (10%; massive subcutaneous abscess, n = 3; retrocolic abscess, n = 2; adhesion-related ileus, n = 3; appendicular stump, n = 1) were associated with open surgery. CONCLUSIONS: There was not a single major complication in the laparoscopy group. These unexpected results are in contrast to previous reports and have prompted us to initiate a prospective randomized trial.
Appendicitis↗
CT and MR imaging of generalized cystic lymphangiomatosis in pediatric patients.
OBJECTIVE: The aim of this study was to describe the spectrum of abnormalities seen in generalized cystic lymphangiomatosis as shown by CT and MR imaging and to correlate these findings to gross pathology. CONCLUSION: MR imaging and CT may substantially broaden visualization of the spectrum of abnormalities seen in generalized cystic lymphangiomatosis by revealing the complete extent of disease and, thus, may contribute to clinical management of the disease by preventing initial misdiagnosis.
Child↗
Pulmonary echinococcosis (hydatidosis) in children: results of surgical treatment.
From 1986-1996, 33 children with 49 pulmonary hydatid cysts underwent surgical treatment in Vienna and Istanbul. Cysts were unilateral in 28 and bilateral in 5 cases; unruptured cysts (URC) were diagnosed in 19 patients, and 14 children presented with ruptured cysts (RC). Ten patients had cysts in other organs (liver, spleen, central nervous system) in addition to pulmonary cysts. Diagnosis was primarily based on chest X-ray and computed tomography scan. In Austrian children, a new combination of serological tests was used successfully (71% positive). The standard surgical procedure was cystotomy followed by capitonnage. The main postoperative complications were fever and wound infection. There were two recurrences after a mean follow-up of 4.8 years, and one patient died because of multiple organ involvement. We conclude that the therapy of choice in pediatric pulmonary hydatidosis is complete surgical elimination of the cyst by cystotomy and capitonnage, whereas more extended resections should be avoided. Ideally, benzimidazole treatment should be combined with surgery. New serological tests can improve diagnostic accuracy.
Adolescent↗
[Congenital oesophageal stenosis due to tracheobronchial remnants].
This paper presents the authors' experience with congenital oesophageal stenosis (= CES) in three patients who had CES due to tracheobronchial remnants. Difficulties with feeding started at 6-9 months of age, which corresponded with the introduction of solids. Contrast studies showed stenosis at the junction of the mid and distal thirds of the oesophagus. Limited resection of the oesophageal stenosis and primary anastomosis was performed in all three patients with good results. CES should be suspected if all other causes of oesophageal stenosis have been excluded by upper gastrointestinal examinations, endoscopy, oesophageal pH monitoring and oesophageal manometry.
Adolescent↗
Successful conservative treatment of severe renal candidosis with fungus balls.
Partial fungal obstruction of the renal collecting system is an unusual finding among infants that poses specific management problems. We report a patient with sepsis and fungal infection of the kidneys post surgery who presented with bilateral fungus balls and was successfully managed by conservative measures. Sonography is the imaging technique of choice in the diagnosis and follow-up of such patients. The need for prompt diagnosis in high-risk patients and the role of sonography are discussed.
Candidiasis↗
Short-bowel syndrome associated with subtotal necrosis of small intestine after rectal trauma.
We report on a 4-year-old girl who experienced rectal trauma during swimming, sitting on an uncovered draining valve in the swimming pool. This resulted in a powerful suction effect on her rectum, followed by rupture of the sigmoid colon and evisceration of the small intestine. Laparotomy showed a near complete necrosis of the small bowel because of thrombotic lesions and wall lacerations of the superior mesenteric artery (SMA). A subtotal bowel removal associated with a jejuno-ileostoma was carried out, a total length of about 35 cm of the small intestine could be left in situ. Parenteral nutrition was stopped after eight months. At the moment defecation takes place 2-3 times a day, growth and weight gain are quite normal.
Accidents↗
Biliary diversion by use of the appendix (cholecystoappendicostomy) in progressive familial intrahepatic cholestasis.
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Abdominal Muscles↗
Necrotizing sarcoid granulomatosis in a 14-yr-old female.
A case of a 14-yr-old female with necrotizing sarcoid granulomatosis (NSG) is presented. She was referred because of chest pain and malaise, and radiography revealed multiple pulmonary nodules. Her history showed seasonal sensitization to aeroallergens and hay fever. Infectious agents or malignancies did not characterize these nodules. However, she was treated with macrolide antibiotics because of suspected infection with Chlamydia pneumoniae. Open lung biopsy showed histological findings of NSG, with epithelioid granulomatous inflammation, including giant cells, and vasculitis. No further treatment was performed, and symptoms disappeared within a few weeks. The chest radiograph showed gradual improvement. The aetiology of NSG is poorly understood, and is postulated to represent either sarcoidosis or rare forms of pulmonary vasculitis such as Wegener's granulomatosis or the Churg-Strauss syndrome. In the case presented, a coincidence of infection with Chlamydia pneumoniae suggests an involvement of infectious agents in the pattern of formation of immune complexes in the aetiology of NSG.
Adolescent↗
Retroperitoneal necrotizing fasciitis in a 4-year-old girl.
Necrotizing fasciitis is a rare but serious condition with a poor prognosis both in adults and in children. Retroperitoneal localization is mostly associated with fatal outcome. Early diagnosis, extensive and repeated surgical debridement, and use of antibiotics are necessary. Herein the authors report on a 4-year-old girl in whom retroperitoneal necrotizing fasciitis developed after she suffered from pyelonephritis. In this case, the outcome was favorable because of early surgical intervention, confirming the diagnosis.
Anti-Bacterial Agents↗
Neurenteric cyst mimicking pleurodynia: an unusual case of thoracic pain in a child.
This study reports an 8-year-old boy with chronic recurring thoracic pain. Magnetic resonance imaging of the spine revealed an intradural cyst at T1-T2. Despite severe compression of the spinal cord, there was no neurologic deficit. After minimally invasive cystectomy, the patient recovered completely. Histological examination established the diagnosis of a neurenteric cyst. The clinical, pathohistologic, and radiologic aspects are discussed, and the decisive role of magnetic resonance imaging in the diagnosis of intraspinal cyst is emphasized.
Child↗
Diagnostic value of tissue polypeptide-specific antigen (TPS) in neuroblastoma and Wilms' tumour.
Although tissue polypeptide-specific antigen (TPS) has been described as a potentially useful serum marker of tumour activity in adult epithelial tumours, few data are available for childhood malignancies. Neuroblastomas and Wilms' tumours are the commonest types of solid malignancies found in the retroperitoneum of children. At this time, a widely used marker for Wilms' tumour is not available. Using an enzyme-linked immunosorbent assay (ELISA) kit, serum TPS levels in 23 children with neuroblastomas, nine with Wilms' tumours and 22 with benign tumours were evaluated to test the usefulness of the marker in identifying malignancies. Compared with healthy children (n = 110), the preoperative least-square means (LSM) of serum TPS were considerably elevated in both neuroblastoma (LSM = 209 U l(-1)) and Wilms' tumour (LSM = 235 U l(-1)), whereas values in benign tumours were only slightly elevated. Although the Wilms' tumours were associated with higher preoperative serum TPS levels, there was no statistically significant difference compared with neuroblastomas. Receiver operating characteristic analysis (ROC curves) showed a high sensitivity and specificity for both malignancies. Successful treatment resulted in decrease in TPS serum values. Serum TPS measurements in children presenting with abdominal masses can help in diagnosing the two commonest extracranial solid malignancies of childhood. Furthermore, TPS could acquire a pivotal role in monitoring therapy.
Analysis of Variance↗