Background and aimsPrevious guidelines addressing surgery of adrenal tumors required actualization in adaption of developments in the area. The present guideline aims to provide practical and qualified recommendations on an evidence-based level reviewing the prevalent literature for the surgical therapy of adrenal tumors referring to patients of all age groups in operative medicine who require adrenal surgery. It primarily addresses general and visceral surgeons but offers information for all medical doctors related to conservative, ambulatory or inpatient care, rehabilitation, and general practice as well as pediatrics. It extends to interested patients to improve the knowledge and participation in the decision-making process regarding indications and methods of management of adrenal tumors. Furthermore, it provides effective medical options for the surgical treatment of adrenal lesions and balances positive and negative effects. Specific clinical questions addressed refer to indication, diagnostic procedures, effective therapeutic alternatives to surgery, type and extent of surgery, and postoperative management and follow-up regime.MethodsA PubMed research using specific key words identified literature to be considered and was evaluated for evidence previous to a formal Delphi decision process that finalized consented recommendations in a multidisciplinary setting.ResultsOverall, 12 general and 52 specific recommendations regarding surgery for adrenal tumors were generated and complementary comments provided.ConclusionEffective and balanced medical options for the surgical treatment of adrenal tumors are provided on evidence-base. Specific clinical questions regarding indication, diagnostic procedures, alternatives to and type as well as extent of surgery for adrenal tumors including postoperative management are addressed.

The diagnosis and assessment of severity of acute colonic diverticulitis may be difficult. A case is presented, in which the delayed diagnosis of diverticulitis resulted in the development of a diverticular mesocolic abscess complicated by hepatic-portal venous gas (HPVG). The utility of ultrasound as a rapid, noninvasive tool to diagnose this distinctly rare condition is outlined. The literature on HPVG associated with acute colonic diverticulitis is reviewed, and the therapeutic options are discussed.

The value of preoperative intravenous urography (IVU) to prevent iatrogenic damage to the urogenital tract was investigated in a series of 236 females and 188 males (mean age 54 [5-87] years) who underwent an operation on the colon or rectum between April 1988 and March 1992. The operations were: 101 right and 49 left hemicolectomies, 125 sigmoid resections, 74 anterior rectum resections, 58 abdomino-perineal rectum amputations and 17 total colectomies. Preoperative IVU was performed in 279 patients (65.8%), while in 145 (34.2%) urgency of the operation or intolerance to contrast medium prevented the procedure being done. The results were abnormal in 75 of the 279 IVUs (26.9%). Of the latter, only 19 (6.8%) of the abnormalities were related to the colorectal disease (renal obstruction due to tumour: 9, malignant invasion of the bladder: 5, ureter displacement: 5). Other diagnostic procedures had given abnormal results in six patients, although the preoperative IVU had been unremarkable. Iatrogenic damage to a ureter occurred in only one patient: the IVU had been normal. No ureteral damage occurred in any of the patients who had not had an IVU. These data indicate that the decisive factor in preventing intraoperative damage to a ureter is not a preoperative IVU but careful intraoperative dissection and visualization of the ureter. There is, therefore, no need for routine preoperative IVU in these cases.

AimsThe LAPSG is a multidisciplinary group dedicated to the study of the biology, pathophysiology and disease of the endocrine and exocrine pancreas. In particular the fields of gastroenterology, endocrinology, oncology, surgery, pharmacology, cell and molecular biology, nutrition, immunology and epidemiology will receive attention.It is also the study group’s aim to enhance the communication among scientists from different areas (especially the fields of gastroenterology and endocrinology) who otherwise rarely meet. The means to achieve these goals will be meetings of the members, postgraduate courses, clinical-research workshops and symposia. Pancreatologists and those working in related areas that live and work in Latin America, and who have published at least once during the last 5 years, are warmly invited to join the LAPSG. Members of the LAPSG will automatically become members of the …

Chen RF, Li ZH, Lou SQ and Chen _] S (luargqzlatvu, Cr'1:'m1 Effects of antisense oligonucleotides of PKC-(1 on proliferation and apoptosis of I-lepG, _, in vitro 75 7.1111 l-SH, Yao ZX, I. uo S], _] ia11g IM, Xiao] W, Liu SC, Liu JB, Sun _] M and Pei'. 7, Y CI1ungq:'n_q_ China

BACKGROUND:The death rate from human diaphragmatic hernia (CDH) ranges from 50 to 80%, mainly due to the associated lung hypoplasia. To prevent these irreversible pathological and physical defects, the question of intrauterine surgical intervention arises. The histological changes of the lung tissue after inducement of a diaphragmatic hernia were examined. Of special interest was the time elapsing until the development of lung hypoplasia.METHODS:A model of intrauterine inducement of diaphragmatic hernia was established using five fetal lambs to study consecutive pulmonary hypoplasia. Inducement of a diaphragmatic hernia was undertaken between 105 and 108 days' gestation. Lung tissue was examined histologically on postoperative days 8, 17, 21, 22, and 25 after inducement of the defect.RESULTS:On postoperative days 8, 17, and 21, no signs of pulmonary hypoplasia were found on histological examination. A pulmonary hypoplasia was found in two fetuses (on the 22nd and 25th postoperative day). The pathological and anatomical examination of a unilateral pulmonary hypoplasia after a short period of time shows that the artificially created diaphragmatic defect is a good model for producing a congenital diaphragmatic hernia.DISCUSSION:The severity of the pulmonary hypoplasia is related to the duration of lung compression by the herniated organs. The time elapsing until the development of lung hypoplasia is shorter than expected. Tracheal occlusion seems to be an effective strategy for treatment of the defect CDH, but the best technique for achieving occlusion, and particularly the ideal point in time to carry out "Fetendo," are unknown. Further research into this congenital illness is required in order to treat it.

The solid-pseudopapillary tumor (SPT) is a very rare pancreatic neoplasm that predominantly affects young females. About 450 cases have been described in the world literature and approximately 20% of the reported patients were children. The occurrence of SPT with distant metastases in children is extremely rare with only two previously reported cases. We now report a 16-year-old Asian girl with a large SPT and synchronous multiple liver metastases who was successfully treated in a 2-step strategy, including initial pylorus-preserving partial duodenopancreatectomy, right hemicolectomy, resection and allografting of the portal vein and secondary resection of 12 liver metastases. The patient is disease free after a follow-up of 18 months after resection of the primary tumor, suggesting that an aggressive surgical treatment might also be justified for metastasized SPT.

Der primäre Hyperparathyreoidismus (pHPT) tritt bei etwa 90% der Patienten mit multipler endokriner Neoplasie Typ 1 (MEN1) auf. Im Gegensatz zum sporadischen pHPT sind üblicherweise mehrerer Drüsen betroffen. Das geeignete Operationsverfahren ist Gegenstand kontroverser Diskussionen. Ziel der vorliegenden Studie war, die Ergebnisse der chirurgischen Therapie des pHPT bei Patienten mit genetisch gesicherter MEN1 zu analysieren. Hierzu wurden präoperative Befunde, operatives Vorgehen und Therapieergebnisse mit Langzeitverlauf des pHPT sowie mögliche Genotyp-Phänotyp-Korrelationen bei 34 Patienten mit genetisch gesicherter MEN1 im Rahmen einer Beobachtungsstudie analysiert. Nach diesen Ergebnisse scheint tPTX+T (total parathyroidectomy+thymectomy) mit Autotransplantation gegenüber der sDE (selektive Drüsenexstirpation) und der stPTX (subtotal parathyroidectromy) ohne zervikale Thymektomie bei Patienten mit MEN1-pHPT das überlegene Operationsverfahren zu sein, da hierbei Rezidive oder eine Persistenz der Erkrankung selten sind. Zur definitiven Beurteilung ist jedoch ein Vergleich der stPTX mit zervikaler Thymektomie und der tPTX mit zervikaler Thymektomie und Autotransplantation im Rahmen einer prospektiven randomisierten Multicenterstudie erforderlich. Eine Genotyp-Phänotyp-Korrelation wurde nicht gefunden.

Summary Background : Minimally invasive adrenalectomy is the standard procedure for the treatment of small benign adrenal neoplasms. The question of whether this technique is also suitable for treating adrenal malignancies has recently been discussed with controversy. Methods : A literature search was performed to gather published experience and opinions concerning the role of endoscopic adrenalectomy in the treatment of primary adrenal malignancies and adrenal metastases. Results : Adrenocortical carcinomas (ACCs) have been approached laparoscopically, and this technique has even been advocated for large tumours. Several small ACCs have been successfully removed laparoscopically. Oncological catastrophes with peritoneal carcinomatosis have been reported. Reoperation after laparoscopic adrenalectomy for unsuspected ACC was usually not performed. There are no data demonstrating a survival benefit of systematic lymphadenectomy. Adrenal metastases are firm and rarely penetrate the capsule of the adrenal gland. Several reports on endoscopic adrenalectomy for metastases have been published. Conclusions : Laparoscopic adrenalectomy for ACC should be considered with great reluctance. If ACC is diagnosed postoperatively upon histological examination after laparoscopic adrenalectomy for a presumably benign tumour, reoperation is not mandatory provided oncological principles were respected during the primary operation. Adrenal metastases confined to the adrenal gland can be removed laparoscopically.

Zusammenfassung Hintergrund. Der primäre Hyperaldosteronismus ist in der Mehrzahl der Fälle durch ein Aldosteron produzierendes Adenom verursacht. Die Entfernung des Adenoms ist die Therapie der Wahl.Im Gegensatz zu bilateralen Erkrankungen der Nebenniere (z.B.das Phäochromozytom beim MEN-II-Syndrom) besteht beim Conn-Syndrom keine Einigkeit über den Stellenwert der subtotalen Adrenalektomie. Fallbericht. Bei einem 44-jährigen Patienten mit primärem Hyperaldosteronismus wurde nach diagnostiziertem NN-Adenom rechts eine subtotale Adrenalektomie durchgeführt und dabei ein Adenom entfernt.Bei postoperativ fortbestehender arterieller Hypertonie und Hypokaliämie musste der Patient bei persistierendem Hyperaldosteronismus nochmals operiert und restadrenalektomiert werden. Diskussion. Die subtotale Adrenalektomie ist beim Conn-Syndrom ein interessanter Therapieansatz, dessen Bedeutung bei dieser Erkrankung im Vergleich zu hereditären Tumorerkrankungen der Nebenniere eher gering ist.Eine mögliche Persistenz der Erkrankung muss gegen den möglichen Vorteil von erhaltenem Nebennierengewebe bei intakter Nebenniere der Gegenseite abgewogen werden.

Incidentally detected adrenal tumors are a common finding during abdominal ultrasonography, computed tomography, and magnetic resonance imaging. Although most of these lesions are benign adenomas, adrenocortical carcinomas and metastases constitute 5% to 10% of all tumors. Adrenal biopsy may be helpful, but its diagnostic value is controversial and disputed, and prospective studies have not yet been performed. Therefore, the diagnostic accuracy of adrenal core biopsy was evaluated in a prospective multicenter study involving 8 surgical centers in Germany and Austria. A total of 220 biopsies from surgical specimens of the adrenal gland were punctured in an ex vivo approach and processed for pathohistologic diagnosis using paraffin sections, routine staining, and immunohistochemistry (keratin KL1, vimentin, S100 protein, chromogranin A, synaptophysin, neuron-specific enolase, D11, MiB-1, and p53 protein). The evaluating pathologist was blinded for clinical data from the patients. A total of 89 adrenal adenomas (40.5%), 22 adrenal carcinomas (10.0%), 55 pheochromocytomas (25.0%), 15 metastases (6.8%), 16 adrenal hyperplasias (7.2%), and 23 other tumors (10.5%) were studied. Nine cases were excluded due to incomplete data (n = 2) or insufficient biopsy specimen (n = 7). In the remaining 211 tumors, compared with the final diagnoses of the surgical specimen, bioptic diagnoses were absolutely correct in 76.8% of the cases, nearly correct in 13.2% of the cases, and incorrect in 10% of the cases. Pheochromocytomas were correctly diagnosed in 96% of the cases, cortical adenomas were correctly or nearly correctly reported in 91% of the cases, cortical carcinomas were correctly or nearly correctly reported in 76% of the cases, and metastases were correctly or nearly correctly reported in 77% of the cases. Of the 39 malignant lesions, only 4 were misclassified, 2 as benign and 2 as possibly malignant. This resulted in an overall sensitivity for malignancy of 94.6% and specificity of 95.3%. Our findings suggest that adrenal core biopsy is a useful method for identifying and classifying adrenal tumorous lesions if sufficient biopsy specimens can be obtained. However, in clinical practice it remains to be shown whether the benefits of biopsy outweigh the risks of the procedure. HUM PATHOL 34:180-186. Copyright 2003, Elsevier Science (USA). All rights reserved.

Einleitung: Der primäre Hyperparathyreoidismus (pHPT) tritt bei 80 – 100% der Patienten mit Multipler Endokriner Neoplasie Typ 1 (MEN 1) auf. Die operative Therapie wird kontrovers diskutiert. Ziel der Studie war, die eigenen Ergebnisse bei MEN 1-Patienten zu überprüfen. Methoden: Bei unseren Patienten mit nachgewiesener MEN1 -Keimbahnmutation und pHPT wurden Daten zum Verlauf prospektiv bei regelmäßigen Verlaufs- und Vorsorgeuntersuchungen erfasst (klinischer Befund, Serum-Calcium, Parathormon). Ergebnisse: Von 34 Patienten waren 30 an einem pHPT erkrankt (88%). Das mediane Follow-Up betrug 60 (2 -228) Monate. Insgesamt wurden bei 28 Patienten 40 Operationen durchgeführt, 2 Patienten lehnten bisher eine Operation ab. Als Ersteingriff wurden 12 selektive Drüsenextirpationen (sDE), 3 subtotale (3,5 Drüsen) Parathyreoidektomien (sPTX) und 13 totale Parathyreoidektomien mit cervicaler Thymektomie und Autotransplantation von Nebenschilddrüsengewebe in den Unterarm (PTX + T) vorgenommen.

Primary adrenal hyperplasia, which may occur as a familial disorder, is a rare cause of ACTH-independent Cushing's syndrome. In most of these cases the underlying pathology is primary adrenocortical micronodular dysplasia. Very few cases of familial Cushing's syndrome due to primary macronodular adrenal hyperplasia have been described. We report a family with seven affected family members. The pedigree indicates an autosomal dominantly inherited disorder. Interestingly only female family members developed the clinically apparent syndrome. The only available obligatory male gene carrier failed to adequately suppress his plasma cortisol level on overnight dexamethasone suppression test. His adrenal glands showed nodular enlargement on abdominal computed tomographic imaging. Screening of the MEN 1 gene and genetic analysis of the hot spot regions of the GNAS 1 (codons 201 and 227) and GNAI 2 (codons 179 and 205) genes did not show any mutations in the constitutional DNA or the adrenal tissue DNA of the index patient. In conclusion, this family is the largest kindred reported in the literature with ACTH-independent Cushing's syndrome due to autosomal dominant inherited macronodular adrenocortical hyperplasia. Four currently alive and affected family members in two generations and further careful observation of the yet unaffected members of the third available generation might offer the opportunity to identify the still unknown gene defect in the future.

INTRODUCTION:Mechanistic study endpoints, evaluated exclusively by the physician, are mostly used in clinical studies evaluating new treatment modalities (e.g. laparoscopic cholecystectomy). Those endpoints often lack clinical relevance. The patient's opinion concerning the importance of a study endpoint is particularly important in the evaluation of minimally invasive procedures, which place special emphasis on patient comfort.METHODS:In a first step it was evaluated by meta-analysis, which clinical endpoints have been used for comparison of laparoscopic and conventional cholecystectomy. Furthermore, using a qualitative analysis it was investigated how important the individual study endpoints are for patients and physicians. Ten patients and five surgeons were questioned in a structured interview.RESULTS:Of all outcome variables used world-wide, approximately one third were hermeneutic study endpoints, depending on the quality of the study, but often the method of evaluation was insufficient. Only three of 215 endpoints (< 2%) were quality of life scores, an integrated concept of outcome was missing completely. The qualitative analysis confirms the claimed difference between isolated and integrated evaluation of treatment goals. The importance of postoperative death is underestimated by patients and physicians; postoperative pain is overestimated. Patients ranked the outcome variable "restoration of full physical fitness" as the most important study endpoint after avoidance of complications and death. It is underestimated in isolated evaluation and has not been used in the world literature at all.CONCLUSION:The analysis of clinical relevance of study endpoints should be the first and not the last step of studies to evaluate surgical technology. It cannot be based purely on intuition; it must make use of scientifically accepted techniques (e.g. qualitative analysis).

Introduction: To choose one or more appropriate study endpoints is one of the most important steps in planning randomized clinical trials. The choice is made by physicians and statisticians for various reasons, one being feasibility. The evaluation by the patients themselves is rarely and only indirectly taken into consideration. Therefore, the clinical relevance of many study endpoints must be considered as questionable. Using the example of the comparison of laparoscopic and conventional cholecystectomy, the degree of importance attached to the different goals of medical treatment by patients and physicians was systematically evaluated. Methods: In total, 18 randomized trials comparing laparoscopic and conventional cholecystectomy were identified in a meta-analysis. The study endpoints of these trials were quantitatively ascertained and used in a subsequent qualitative analysis (Dey 1993). On the day prior to elective cholecystectomy ten patients and five surgeons performing the operations were asked in a standardized interview about the relevance which they attached to these endpoints: initially as single variables using a Likert scale, and then in a competitive sequence (rank list with cards). Results: The classical mechanistic outcomes such as mortality, complication rate and length of in-hospital stay were almost exclusively identified in the meta-analysis. Hermeneutic endpoints (reported by the patient from his subjective experience) were rarely used except for postoperative pain, the latter often with invalid methods. Quality-of-life scores were only used as two of a total of 72 endpoints (< 3%), an integrated concept of outcome (patient and physician) was missing completely. Rating the different outcomes (qualitative analysis) surgeons as well as patients considered death and intra- and postoperative complications as most important. Return to full physical fitness was the most important hermeneutic endpoint for the patients, while this endpoint was much less important for the surgeons. Most patients considered postoperative pain as much less important than physicians. Length of hospital stay was often given the lowest rank. Conclusion: An integrated outcome concept is recommended which includes the rating of study endpoints by patients and physicians. Analysis of the clinical relevance of outcome variables needs to be one of the first and not the last steps in surgical technology assessment. It should not originate exclusively from the intuition of the doctor, but needs to be done with new scientifically accepted methods (e.g. qualitative analysis).