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Background: Neuroendocrine tumors of the small bowel (small intestine neuroendocrine neoplasms, SI-NEN) are the most frequent tumors of the small intestine and approximately 30-40% are still surgically treatable with curative intent at the time of diagnosis. Certain surgical principles must be followed for optimal oncological outcomes and good postoperative quality of life., Methods: Based on international guidelines and own experiences, the locoregional surgical treatment of SI-NENs is presented., Results: Locoregional SI-NENs should always be resected if technically feasible, as only this approach can achieve a long-term cure and even small primary tumors (< 10 mm) often already show lymphatic metastasis. The resectability of SI-NENs and their difficulty depend on the extent of lymphatic metastasis, which should be assessed based on preoperative imaging of the extent around the superior mesenteric artery. Currently, the surgical gold standard for SI-NENs is open surgery with bidigital palpation of the entire small intestine followed by primary tumor resection via small bowel segment resection, right hemicolectomy or ileocecal resection and vessel-sparing, and therefore organ-preserving lymphadenectomy (≥ 8 lymph nodes). The guidelines consider that laparoscopic or robotic approaches are justified only for early stages of SI-NENs., Conclusion: Guideline-compliant surgical treatment of locoregional SI-NEN enables recurrence-free long-term survival with good quality of life., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Gastroenteropancreatic neuroendocrine neoplasms (GEP-NEN) are mainly found in the small intestine and pancreas. The course of the disease in patients is highly variable and depends on the degree of differentiation (G1-G3) of the neoplasm. The potential for metastasis formation of GEP-NEN is high even with good differentiation (G1). Lymph node metastases and, in many cases, liver metastases are also often found. Less common are bone metastases or peritoneal carcinomas. The treatment of these GEP-NENs is surgical, whenever possible. If an R0 resection with removal of all lymph node and liver metastases is successful, the prognosis of the patients is excellent. Patients with diffuse liver or bone metastases can no longer be cured by surgery alone. The long-term survival of these patients is nowadays possible due to the availability of drugs (e.g., somatostatin analogues, tyrosine kinase inhibitors), peptide receptor radionuclide therapy (PRRT) and liver-directed procedures, with a good quality of life., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Background: Anaplastic thyroid carcinoma (ATC) represents the rarest but most aggressive tumor entity of the thyroid gland. In this respect, the treatment of advanced ATC has rapidly evolved in recent years. Recently, new personalized forms of treatment that address the somatic mutational status of the tumor have been increasingly used. The aim of this article is to provide an overview of current molecular-based and personalized treatment options for ATC., Methods: A current literature search was performed with a focus on personalized molecular-based treatment options for ATC., Results: The majority of patients suffering from ATC have an advanced tumor disease at the time of initial diagnosis. Despite multimodal treatment approaches consisting of surgery, external beam radiation therapy (EBRT) and chemotherapy (CTX), the prognosis of ATC is still poor. Accordingly, the focus of innovative treatment approaches is on molecular-based, individualized tumor therapy, including in particular BRAFV600E and multikinase inhibitors. The potential of the latter seems to lie particularly in combination therapy with immune checkpoint inhibitors. These treatment options can be used in both adjuvant and neoadjuvant settings. Neoadjuvant treatment of advanced ATC can achieve a potentially resectable treatment setting and improve the poor prognosis of affected patients; however, larger prospective and randomized studies on these combination therapies are currently pending., Conclusion: The focus of future treatment approaches for ATC will be on individualized, molecular-based tumor therapy. In particular, the neoadjuvant use of these therapies may change the paradigm of ATC surgery as locally advanced as well as metastatic carcinomas can be converted to a potentially resectable status and made amenable to surgery., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Background: Multiple endocrine neoplasia type 1 (MEN1)-associated duodenopancreatic neuroendocrine neoplasms (dpNEN) represent the most frequent syndrome-associated cause of death, but the adequate treatment is sometimes considered controversial., Objective: Presentation of possible diagnostic and therapeutic options for MEN1-associated dpNENs., Material and Methods: In this review article retrospective case studies, expert recommendations, national and international guidelines as well as personal experiences were analyzed and evaluated., Results: Due to early detection programs and the use of the most modern imaging techniques, dpNEN are nowadays diagnosed much earlier. Nonfunctional pNENs currently represent the most frequent dpNENs with about 70%, followed by gastrinomas and insulinomas. Regardless of their functional activity, dpNENs with a size of > 2 cm are generally an indication for surgery. The choice of the optimal treatment strategy, however, in most cases remains the subject of controversial discussions, although nowadays surgery should always be performed in an organ-preserving and minimally invasive way when feasible. Recurrences or new dpNENs are expected in more than 60% of cases, necessitating a reoperation in up to 40% of these cases. Duodenopancreatic resections and reoperations can be carried out safely by experienced practitioners and with an acceptable level of risk., Conclusion: The planning of treatment requires careful consideration of the suitable timing, the extent of the operation, the risk of recurrence and potential morbidities. Furthermore, preserving pancreatic function and the quality of life is of utmost importance. In view of the complexity of the disease, MEN1 patients should be treated in specialized centers., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Background: In 2019 approximately 7500 procedures were carried out for parathyroid diseases in Germany (Statistisches Bundesamt 2020, https://www.destatis.de/DE/ ). All operations were performed as inpatient procedures. The catalogue of outpatient procedures for 2023 does not include operations on the parathyroid glands., Objective: Which conditions are prerequisites for parathyroid surgery on an outpatient basis?, Material and Methods: Published data on outpatient parathyroid surgery were analyzed with respect to the underlying disease, procedures performed and patient-specific circumstances., Results: Initial operations for localized sporadic primary hyperparathyroidism (pHPT) seem to be suitable for outpatient surgery, provided that affected patients fulfil the general prerequisites for an outpatient operation. The procedures focused parathyroidectomy and unilateral exploration can be carried out using local or general anesthesia and have a very low risk for postoperative complications. The organization of the day of the operation and the postoperative treatment of the patient should be organized within a detailed standard of procedure. The remuneration for an outpatient parathyroidectomy is not included in the German outpatient surgery catalogue and is therefore currently not adequately financially reimbursed., Conclusion: In selected patients a limited initial intervention for primary hyperparathyroidism can be safely performed on an outpatient basis; however, the present German reimbursement modalities have to be revised so that the cost of these outpatient operations can be adequately covered., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Despite the triumph of minimally invasive techniques in adrenal surgery, the indications for open adrenalectomy are indispensable in the canon of treatment options and must remain part of the repertoire of visceral surgery. Open adrenalectomy is indicated for advanced adrenal carcinoma (ENSAT stage III). In addition to the frequent local infiltration of these carcinomas which makes the en bloc resection of adjacent organs necessary, thromboses in the renal vein or the vena cava or multiple lymph node metastases can also necessitate an open procedure; however, open adrenalectomy is justified and must also be discussed for adrenocortical carcinoma ENSAT stages I-II (tumor size ≤ 5 cm or > 5 cm, NO). Furthermore, highly suspicious large adrenal tumors (6-8 cm, Hounsfield units > 20) without preoperative evidence of malignancy and other adrenal pathologies, such as neuroblastomas, large pheochromocytomas and also schwannomas can be an indication for open adrenalectomy., (© 2022. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Background: For the increasingly diagnosed entity of small asymptomatic, sporadic, nonfunctional, pancreatic neuroendocrine neoplasms (pNEN), a negligible or at least unclear prognostic relevance of the disease for patient survival has often been observed., Objective: Safety and acceptance of a watch-and-wait strategy versus surgical resection for small, asymptomatic nonfunctional (NF) pNEN., Methods: Presentation and evaluation of the relevant literature as well as the corresponding national and European guidelines., Results: Surgery of small NF-pNEN shows complication rates of 15-32% (Clavien-Dindo ≥ 3) and a mortality of 3.6%. Even for pNEN < 2 cm the presence of lymph node metastases has been observed in 11% of cases, while their prognostic relevance in G1-pNEN compared with active surveillance remains unclear. On average 14% of patients under active surveillance for small NF-pNEN, underwent a resection. Relevant tumor growth during surveillance was found in < 20% of cases. In all well-selected surveillance cohorts no metachronous lymphatic or distant metastases occurred during active surveillance and especially no cases of a metachronous no longer curable disease., Conclusion: Even small asymptomatic NF-pNEN have a certain metastatic potential but the clinical relevance has prospectively not yet been clearly determined. Controlled surveillance of these tumors is at least an alternative to immediate tumor resection. Especially patients above 70 years old do not seem to benefit from resection. The pros and cons of a resection should therefore be individually evaluated with the patient., (© 2022. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)