Purpose: Insulinoma and non-single-insulinoma pancreatogenic hypoglycemia syndrome (NIPHS) are rare causes of hypoglycemia. Treatment options and postoperative outcome are discussed to define future strategies.

Introduction: Ankylosing spondylitis, also known as Bekhterev's disease, is a chronic inflammatory, systemic rheumatic disease, mainly affecting the axial skeleton and leading to progressive stiffness of the spine.

Die chirurgische Therapie der Schilddrüsenkarzinome beruht auf einer histologie- und stadienadaptierten Vorgehensweise mit hoher interindividueller Variabilität. Sie kann von rein abwartendem Verhalten bis zu ausgedehnten multiviszeralen Eingriffen reichen. Die Zusammenfassung unterschiedlicher histologischer Malignomtypen führt zu Fehleinschätzungen bei der jeweils notwendigen Anpassung der chirurgischen Radikalität. Für die chirurgische Therapie sind die Kenntnis des biologischen Verhaltens der Tumoren, der Patientengefährdung durch den Tumor oder die Therapie, aber auch ein breites chirurgisches Spektrum limitierter und ausgedehnter Resektionsverfahren im Hals- und Thoraxbereich notwendig. Die hier vorgenommenen Empfehlungen richten sich primär nach den deutschen Leitlinien der chirurgischen Arbeitsgemeinschaft für Endokrinologie der Deutschen Gesellschaft für Allgemein- und Viszeralchirurgie und nach eigener Erfahrung der Autoren neben der Nennung von Leitlinien anderer Arbeitsgruppen.

Advanced preoperative imaging of parathyroid adenomas and intraoperative parathyroid hormone determination optimized the results in the surgical treatment of primary hyperparathyroidism patients. We asked, whether reasons for failure have changed during the last 25 years.We retrospectively analyzed operations for persistent primary hyperparathyroidism in our department between 2001 and 2011 (n=67), and compared these results to our experience between 1986 and 2001 (n=80).From 2001 to 2011, 765 primary hyperparathyroidism patients were operated on at our department. All but 4 patients were cured (761/765, 99.5%). 67 operations were performed for persistent primary hyperparathyroidism. Main reasons for failure were a misdiagnosed sporadic multiple gland disease in our own patients (18/29, 62.1%), and an undetected solitary adenoma in patients referred to us after -initial operation in another hospital (22/38, 57.9%) (statistically significant). From 1986 to 2001 (1 105 primary hyperparathyroidism patients), main indications for re-operation due to persistent disease were an undiagnosed sporadic multiple gland disease in our own patients (15/24, 62.5%), and a missed solitary adenoma in patients being operated on primarily somewhere else (38/56, 67.9%) (statistically significant).Comparing our experience in 147 patients with persistent primary hyperparathyroidism being operated on between 2001-2011 and 1986-2001, not much has changed with the modern armamentarium of improved preoperative imaging or intraoperative biochemical control. Whereas sporadic multiple gland disease was the most common reason for unsuccessful surgery in experienced hands, other units mainly failed due to an undetected solitary adenoma. Re-operations for persistent primary hyperparathyroidism performed by us were successful in 93.8% (2001-2011) and 96.0% (1986-2001), respectively.

s of the 3rd ITLT Essen 2013 / Digestive and Liver Disease 45S (2013) S233–S260 S245 nally metastases, into neoplasms with benign, uncertain, low-grade malignant and high-grade malignant behavior. A new TNM classification system was invented to get insights in the stage and also prognosis of these heterogeneous diseases.

Introduction: Spontaneous tendon ruptures are a rare event. The few cases described in the literature are mostly associated with chronic metabolic diseases, such as chronic haemodialysis in patients with renal failure and subsequent secondary hyperparathyroidism.

Introduction: In about 1 – 15% of thyroidectomies the goiter is located intrathoracic with higher rates of operative complications and a somewhat different management. In the literature sternotomy should only be performed in cases of previous cervical thyroidectomy, invasive carcinoma and truly intrathoracic goiter. We assessed, whether sternotomies are necessary in these patients, at all.

Surgical therapy for thyroid neoplasms is based on tumor histology and comprises stage-adapted procedures with a high degree of inter-individual variability. This can range from waiting and monitoring, to extensive multivisceral surgery. Grouping together histologically different types of malignancies leads to false assumptions when gauging the radicality of surgery necessary in each particular case. Surgical therapy requires not only an understanding of the biological behavior of the tumor and the risk that it or the therapy poses to the patient, but also knowledge of a wide surgical spectrum of limited and complex resection procedures in the neck and thorax region. The following recommendations are based primarily on the guidelines of the Surgical Working Group for Endocrinology of the German Society for General and Visceral Surgery as well as on the authors' own experience and, where indicated, the guidelines of other working groups.

Pancreatic neuroendocrine tumours (PNET) are rare entities with an annual incidence of < 100,000. About 1 - 2 % of pancreatic neoplasias are neuroendocrine tumours. About one third of these tumours secrete biologically active substances that lead to development of specific clinical syndromes. PNET may occur sporadically or in association with hereditary syndromes, such as multiple endocrine neoplasia type 1 (MEN1). Among the functional PNET, insulinomas and gastrinomas are the most common entities. In contrast, vasoactive intetinale peptide (VIP)-secreting tumours, glucagonomas, serotonin-secreting carcinoid tumors, and tumours with secretion of ectopic hormones, such as calcitonin, are extremely rare. Once diagnosis has been established on the basis of clinical and laboratory findings, localization of the source of pathologic hormone secretion is warranted. Imaging methods frequently used for localization of PNET comprise anatomical imaging modalities, computed tomography, and magnetic resonance imaging, endoscopic ultrasound, selective arterial catheterization with hepatic venous sampling, DTPA-octreotid scintigraphy and DOTA-D-Phe(1)-Tyr(3)-octreotid positron emission tomography. Therapy is based on the specific tumour entity and the extent of the disease. In the majority of patients, even in the case of malignant disease, a surgical approach is warranted, eventually combined with a medical treatment.

Eine Struma nodosa ist nach Ergebnissen von Reihenuntersuchungen in Deutschland bei etwa 20% aller Erwachsenen zu finden und die chirurgische Therapie einer Struma nodosa mit Euthyreose wird bei uns über 90000-mal pro Jahr durchgeführt. Sie stellt damit über 80% aller Schilddrüseneingriffe dar und wird weitaus häufiger vorgenommen, als dies für die Operationen einer Hyperthyreose mit Struma (etwa 15–20%) oder die eines Schilddrüsenkarzinoms (ca. 1%) gilt. Dies steht im Gegensatz zu den Verhältnissen in Ländern ohne Jodmangel, bei denen Operationen der Schilddrüse wegen malignem Tumorwachstum häufiger durchgeführt werden als Operationen an einer Struma nodosa mit Euthyreose. Somit könnte man die Operation der Struma nodosa mit Euthyreose für Deutschland auch als „Brot-und-Butter-Chirurgie“ eines jeden endokrinen Chirurgen bezeichnet, da zu erwarten ist, dass ohne sicheres Vorgehen bei dieser Operation, mit nachweislich guten Ergebnissen, weitere Patienten mit endokrin-chirurgischen Problemen ausbleiben werden. Um eine erfolgreiche Operation gewährleisten zu können, ist jedoch die Kenntnis der Pathophysiologie der Erkrankung fraglos notwendig, wie auch das Wissen um die häufigen und seltenen Komplikationen der Operation unabdingbar ist. Adäquate Schilddrüsenchirurgie kann damit auch nicht auf die alleinige Frage nach einer Thyreoidektomie oder Teilresektion der Schilddrüse reduziert werden. Nur auf der Basis des Verständnisses endokrinologischer Zusammenhänge und technischer Details der Therapie kann den Patienten eine realistische Einschätzung über Nutzen und Probleme der Operation vermittelt werden, die auch als Grundlage der Entscheidung für oder gegen eine Operation dienen sollte. Im Falle einer Entscheidung für die Operation wird der weitere Ablauf der Therapie danach relativ standardisiert erfolgen und gibt somit genügend Raum, sich auf die spezifischen und individuellen Aufgaben besonders konzentrieren zu können (Abb. 1).

HISTORY AND CLINICAL FINDINGS:A 42-year-old woman was found by her husband with unconsciousness and seizure at night three weeks after delivery of her fifth child. At a blood glucose level of 25 mg/dl, she received an intravenous infusion of glucose by the called emergency physician, leading to a rapid improvement of her symptoms.INVESTIGATION AND DIAGNOSIS:The following examination showed a low basal blood glucose level as well as pathological levels of insulin and C-peptide. These findings together with the Whipple trias (hypoglycaemia, neurological symptoms and rapid improvement after infusion of glucose) were highly suspicious of an insulinoma. Whereas CT, MRI and DOTATOC-PET were negative, endoscopic ultrasound showed a mass of 13 mm in the tail of the pancreas.TREATMENT AND COURSE:The tumour was resected from the tail of the pancreas by laparoscopic enucleation. Histological examination revealed an endocrine tumour (insulinoma) of the pancreas. Postoperative blood glucose levels were within the normal range. The patient and her healthy newborn child could be dismissed from hospital on the third day after surgery.CONCLUSION:Despite its rarity, an insulinoma represents an important differential diagnosis of hypoglycaemia during and right after pregnancy.

The molecular genetic changes from certain endocrine tumors are already understood, reflecting as they do the etiology of these sporadic familial disorders. This already has clinical consequences to the treatment of familial endocrine tumors, which often appear in the course of syndromatic disorders. These consequences consist in slight changes to surgical technique, the search for other active and usually endocrinal tumors, and examination of family members for other gene carriers (of disease-specific mutations) and the most suitable prophylactic tumor therapy. In contrast, for sporadic endocrine tumors there exists far less clinically relevant knowledge. Starting with anamnesis and clinical findings of active endocrine tumors, we discuss the current possibilities for molecular genetic determination of disease-specific mutations (germline and tumor DNA) and their effect on surgical procedure.

Benign adrenal gland tumors smaller than 6 cm are nowadays the indication for minimally invasive surgery. Until now there has been no significant difference between retroperitoneoscopic and transabdominal adrenalectomy. Intestinal adhesions could be a contraindication against transabdominal laparoscopic adrenalectomy, and therefore the retroperitoneoscopic approach could be an advantage in these cases. A prospective study concerning this question has not been published yet. Our clinical investigation here includes 114 adrenalectomies during the last 5 years. We show that in any case of abdominal preoperation, laparoscopic adrenalectomy can be performed by transabdominal approach and without conversion to open surgery. Discussed are the different indications for laparoscopic adrenalectomy, operating time, conversion rate to open surgery, and amount and type of abdominal preoperation. We compared patients with and without abdominal preoperations.

Die molekulargenetische Veränderungen einiger endokriner Tumoren sind zu Teilen aufgeklärt und spiegeln die ätiologische Grundlage der Entwicklung dieser sporadischen und familiären Erkrankungen wider. Für familiäre endokrine Tumoren, die oft im Rahmen von Syndromerkrankungen entstehen, hat dies schon jetzt klinisch praktische Auswirkungen. Sie liegen im teilweise veränderten chirurgischen Vorgehen, in der Suche nach weiteren, meist endokrin aktiven Tumoren und in der Untersuchung von Familienmitgliedern zur Entdeckung weiterer Genträger (Träger der spezifischen Mutation) und gegebenenfalls einer prophylaktischen Tumortherapie. Für sporadische endokrine Tumoren verfügen wir dagegen über weit weniger klinisch verwendbare Erkenntnisse.