A case of synchronous quintuple gastric cancer with large-cell endocrine carcinoma.

The patient was a 73-year-old man who visited our department with black stools as the chief complaint. Upper digestive tract endoscopy revealed three type 2 lesions in the lesser curvature of the gastric antrum and the gastric angle and the posterior wall of the upper gastric body, which were diagnosed by biopsy as tub2, por, and sig, respectively. Total gastrectomy was performed. The final pathological diagnosis was quintuple gastric cancer with a main lesion of large-cell endocrine carcinoma and four adenocarcinoma sublesions. We report this extremely rare case of gastric endocrine cell carcinoma complicated by adenocarcinoma.

Revisiting robotic approaches to endocrine neoplasia: do the data support their continued use?

PURPOSE OF REVIEW: Although the advent of the robot has revolutionized the modern treatment of endocrine neoplasia, substantial controversies exist on its applicability, safety and benefits over the conventional laparoscopic operations. The present review aims to review the recent literature on various robotic approaches in treating thyroid, parathyroid, adrenal and pancreatic endocrine neoplasia and see whether its continued use should be supported. RECENT FINDINGS: In summary, the role of robotic thyroidectomy has been clearly established and should be continued by experienced surgeons on selected patients. Because of the limited availability of evidence, the feasibility of robotic parathyroidectomy has yet to be elucidated. With proven favorable perioperative outcomes, robotic adrenalectomy and pancreatectomy should be continued as potential alternatives to conventional surgery. SUMMARY: Robotic endocrine procedures still play a pivotal role in minimally invasive endocrine surgery with demonstrable safety and effectiveness. Future research should embark on prospective randomized-controlled trials on robotic endocrine procedures to collect higher level of evidence and long-term survival data.

Complete metastasectomy for renal cell carcinoma: Comparison of five solid organ sites.

BACKGROUND AND OBJECTIVES: Patients with metastatic RCC can undergo metastasectomy to improve survival time. Our goal was to provide and compare characteristics and oncological outcomes of RCC patients who underwent complete metastasectomy at a single organ site. METHODS: A total of 138 RCC patients were identified as undergoing complete metastasectomy at a single organ site including adrenal, lung, liver, pancreas, or thyroid. Competing risk regression analysis was used to assess RFS and CSS adjusting for several covariates. RESULTS: In this highly selected cohort, RFS and CSS was 27% and 84% at 5 years following metastasectomy, respectively. Univariate analysis revealed that removal of multiple tumors, younger age, and a shorter interval between nephrectomy and metastasis was associated with worse RFS. Larger tumors and sarcomatoid histology at nephrectomy was associated with worse CSS. We found no evidence that metastases at the time of RCC diagnosis influenced recurrence or survival. Tumor size, number of metastases resected, and time from nephrectomy to first recurrence was significantly different, but recurrence rates were not found to be significantly different, when compared across all organ sites. CONCLUSIONS: These findings inform clinical and surgical management of select RCC patients with isolated metastasis to one of several organ sites. J. Surg. Oncol. 2016;114:375-379. © 2016 Wiley Periodicals, Inc.

[A case of endocrine cell carcinoma of the ascending colon with liver metastasis treated with hepatectomy after excision of the primary lesion and systemic chemotherapy].

A 77-year-old man who complained of melena was admitted to our department. Colonoscopy revealed a type 2 tumor in the hepatic flexure of the ascending colon. Biopsy examination revealed a poorly differentiated adenocarcinoma. Abdominal computed tomography(CT)revealed 3 tumors within the posterior segment of the right hepatic lobe. Initially, a right hemicolectomy was performed. Immunohistochemically, the tumor was diagnosed as an endocrine cell carcinoma. After surgery, a capecitabine, oxaliplatin, and bevacizumab(CapeOX/BEV)regimen was administered. However, after 5 chemotherapy courses, abdominal CT revealed enlargement of the 3 tumors in the posterior segment of the right hepatic lobe. There was no metastasis besides the liver metastasis. The patient underwent a radical hepatectomy of the posterior segment. At 8 months post-surgery, the patient remains alive and well. Endocrine cell carcinoma of the colon and rectum is usually malignant and is associated with a very poor prognosis because of rapid hematogenous or lymphogenous metastasis. Effective multimodal treatment regimens, including surgery and new chemotherapies such as molecular targeted therapies, should be established to improve the prognosis of patients with endocrine cell carcinomas of the colon and rectum.

Lymph node metastases are more frequent in paediatric appendiceal NET ≥1.5 cm but without impact on outcome - Data from the German MET studies.

BACKGROUND: Paediatric appendiceal neuroendocrine tumours (appNET) are very rare tumours, mostly detected incidentally by histopathological evaluation after appendectomy. Treatment recommendations are based on adult data considering high-risk NET as defined by European Neuroendocrine Tumour Society (ENETS) guidelines for completion right-sided hemicolectomy (RHC). Recent data suggest that less aggressive therapy may be justified. PROCEDURE: Analysis of children and adolescents with appNET prospectively registered with the German Malignant Endocrine Tumour (MET) studies between 1997 and 2022. RESULTS: By December 2022, 662 patients (64.7% females, 35.3% male) had been reported. Median age was 13.3 years [4.5-17.9], median duration of follow-up 2.2 years [0-10.9]. No distant metastases were reported. Tumour size was <1 cm in 63.5%, 1-2 cm in 33.2%, and >2 cm in 3.2% of patients. WHO grade 1 and 2 tumours were diagnosed in 76.9% and 23.1% of patients, respectively. Lymphovascular invasion and lymph node metastases were associated with tumour size ≥1.5 cm. 27.0% of patients presented with high-risk NET according to ENETS criteria. Of those, only 55.9% underwent secondary oncological right hemicolectomy. Neither distant metastases, nor recurrences or disease-related deaths occurred in patients with appendectomy only as well as in patients with completion RHC. Overall and event-free survival were both 100%. CONCLUSIONS: Internationally harmonized consensus recommendations on treatment of children and adolescents with appendiceal NET are urgently needed to avoid completion RHC in high-risk patients.

[Endocrine oncology : An update on surgical treatments]. / Endokrine Onkologie : Neues zu chirurgischen Therapien.

BACKGROUND: In the management of solid tumours, routine concepts are increasingly being transformed into individualized patient treatment. Endocrine surgery is traditionally characterized by resection strategies that are adapted to phenotype and genotype of the underlying disease. As complication rates in surgery correlate with the extent of resection, continuous efforts are made to identify selection criteria in order to limit the extent of surgery without compromising the oncological outcome. The aim is to design risk-stratified precision endocrine surgery. MATERIALS AND METHODS: A search was carried out in PubMed for new and modern strategies and approaches for oncological endocrine surgery. RESULTS: Several developments in surgical technique and technology, molecular pathology, medical therapy, and study data identify the potential to adapt the surgical strategy in all areas of endocrine surgery. CONCLUSION: According to prevalent data, limited extent of resection in thyroid cancer surgery shows a reduction in complication rates while preserving oncological outcome when adequate selection criteria are implemented. New insights and innovative technologies also influence additional areas in oncological endocrine surgery for parathyroid, adrenal, and neuroendocrine neoplasia. However, the broad practice of these new concepts needs to be evaluated with regard to long-term oncological outcome.

Concomitant pancreatic endocrine neoplasm and intraductal papillary mucinous neoplasm: a case report and literature review.

We report a case of concomitant pancreatic endocrine neoplasm (PEN) and intraductal papillary mucinous neoplasm (IPMN). A 74-year-old man had been followed-up for mixed-type IPMN for 10 years. Recent magnetic resonance images revealed an increase in size of the branch duct IPMN in the pancreas head, while the dilation of the main pancreatic duct showed minimal change. Although contrast-enhanced computed tomography and magnetic resonance imaging did not reveal any nodules in the branch duct IPMN, endoscopic ultrasound indicated a suspected nodule in the IPMN. A malignancy in the branch duct IPMN was suspected and we performed pylorus-preserving pancreatoduodenectomy with lymphadenectomy. The resected specimen contained a cystic lesion, 10 x 10 mm in diameter, in the head of the pancreas. Histological examination revealed that the dilated main pancreatic duct and the branch ducts were composed of intraductal papillary mucinous adenoma with mild atypia. No evidence of carcinoma was detected in the specimen. Incidentally, a 3-mm nodule consisting of small neuroendocrine cells was found in the main pancreatic duct. The cells demonstrated positive staining for chromogranin A, synaptophysin, and glucagon but negative staining for insulin and somatostatin. Therefore, the 3-mm nodule was diagnosed as a PEN. Since the mitotic count per 10 high-power fields was less than 2 and the Ki-67 index was less than 2%, the PEN was pathologically classified as low-grade (G1) according to the 2010 World Health Organization (WHO) criteria. Herein, we review the case and relevant studies in the literature and discuss issues related to the synchronous occurrence of the relatively rare tumors, PEN and IPMN.

[A case of gastric endocrine cell carcinoma that responded to chemotherapy with CPT-11+CDDP].

A 57-year-old male patient was referred to our department with a diagnosis of #3 lymph node recurrence of early gastric cancer after treatment of endoscopic submucosal dissection (ESD). The pathological diagnosis of the ESD specimen was neuroendocrine cell carcinoma of the stomach with positive immunohistochemical staining of chromogranin A. The diameter of the tumor was 10 mm and the depth of invasion was pSM2. Distal partial gastrectomy with standard lymph node dissection (D2) was performed. The pathological findings were negative for malignancy in the resected stomach and positive in 2 of the #3 lymph nodes. Adjuvant chemotherapy of S-1 was administered, but a recurrence in the paraaortic lymph nodes was revealed by follow up X-ray computed tomography (X-CT) 3 months later. The case was considered as a S-1 failure, and the chemotherapy was changed to the irinotecan(CPT-11) +cisplatin(CDDP). A clinical complete response (CR) was obtained after two courses and maintained for up to twenty months.

A rare combination of an endocrine tumour of the common bile duct and a follicular lymphoma of the ampulla of Vater: a case report and review of the literature.

Carcinoid tumours of the common bile duct represent an extremely rare entity. Similarly, primary follicular lymphomas of the ampulla of Vater constitute an infrequent neoplasia. Herein, we report the first case of a synchronous development of a carcinoid tumour of the common bile duct and an ampullary follicular lymphoma that was treated surgically with a Whipple's procedure, due to inability to establish definitive preoperative diagnosis despite the extensive diagnostic investigation.

A case of mixed ductal-endocrine carcinoma of the pancreas.

A 66-year-old male patient underwent a stomach-preserving pancreatoduodenectomy procedure because of a tumor located around the lower bile duct under the diagnosis of carcinoma of the lower bile duct. The tumor (3.5 × 2.5 cm) was found at the head of the pancreas and had invaded the papillae of Vater at the duodenum. Histology findings indicated both ductal adenocarcinoma and endocrine tumor. The ductal adenocarcinoma component expressed carcinoembryonic antigen, cytokeratin (CK)-19, CK-20, carbohydrate 19-9, and amylase, whereas the endocrine component, which occupied about one-third of the tumor, expressed glucagon, neuron-specific enolase, and chromogranin A. The Ki-67 labeling indices of the two components were 49.7% and 5.3%, respectively. Herein, we present this case of mixed ductal-endocrine carcinoma of the pancreas. Our findings indicate that its aggressive mass may be ascribable to the adenocarcinoma component with a high proliferative potential.

Benefits and adverse effects of endocrine therapy.

Endocrine-responsive tumors that are small and without nodal involvement (i.e. tumors classified as pT1 pN0) are a heterogeneous group of tumors that are associated with a low risk of relapse in the majority of the cases. Therefore, the costs and benefits of adjuvant endocrine therapy should be carefully considered within this subgroup of patients. Treatment decisions should take into consideration co-morbidities as well as the presence of other classical risk factors such as HER2 overexpression or extensive peritumoral vascular invasion. Tamoxifen or tamoxifen plus ovarian function suppression should be considered as proper endocrine therapies in premenopausal patients. Ovarian function suppression alone or ovarian ablation might also be considered adequate in selected patients (e.g. very low-risk patients, in the presence of co-morbidities or patient preference). An aromatase inhibitor should form part of standard endocrine therapy for most postmenopausal women with receptor-positive breast cancer, although patients at low risk or with co-morbid musculoskeletal or cardiovascular risk factors may be considered suitable for tamoxifen alone. Tailored endocrine treatments should be considered in patients with endocrine-responsive tumors classified as pT1 pN0. Issues focusing on safety, quality of life and subjective side effects should be routinely discussed.

Liver transplantation for primary or secondary endocrine tumors.

Endocrine tumors comprise a heterogeneous group of rare neoplasms. Liver metastatic endocrine tumors (MET) are amenable to various therapeutic modalities including liver transplantation (LT). However, LT for MET remains controversial because of the lack of clear selection criteria. The purpose of this study based on thorough perusal of English and French literature since 1989 was to identify prognostic factors and propose recommendations for selecting patients most likely to benefit LT for primary and secondary endocrine tumors.

Paraaortic paraganglioma arising in an ovarian carcinoma patient mimicking lymph node metastasis.

Paragangliomas are rare tumors arising from the chromaffin cells in the autonomic nervous system. While they both occur most frequently along the paraaortic chain, paraganglioma and ovarian carcinoma very rarely occur together. A 61-year-old, post-menopausal woman visited our hospital, with complaints of abdominal pain and genital bleeding. Image analysis showed a 21 x 18 x 10 cm ovarian mass, and a 38 mm tumor at the paraaortic lesion. First, she underwent bilateral salpingo-oophorectomy. Serous papillary cystadenocarcinoma of the left ovary was found, and so a second surgery was performed. The paraaortic tumor was completely eliminated in spite of fluctuating blood pressure intraoperatively. Microscopic examination revealed that the paraaortic tumor was paraganglioma. She was ultimately diagnosed as having ovarian carcinoma stage Ia (FIGO) with coincident paraganglioma. If blood pressure fluctuation is observed during dissection of the paraaortic lymph node, paraganglioma should be suspected and blood pressure must be carefully controlled.

Well-differentiated endocrine cell carcinoma of ileum treated by laparoscopy-assisted surgery--a case report.

A 72-year-old woman presented at our hospital with a 1-year history of intermittent right lower abdominal pain. Colonoscopic examination revealed a submucosal tumor with a pitted surface in the terminal ileum. Histopathological diagnosis of the carcinoid tumor was made following biopsy. Blood serotonin and urine 5-hydroxy-indoleacetic acid levels were normal, and carcinoid syndrome was not detected. Enhanced abdominal computed tomography scan and 18F-fluorodeoxyglucose positron emission tomography failed to detect multiple lesions, lymph node swelling or distant metastasis. Laparoscopy-assisted ileocecal resection with lymph node dissection was performed. The resected specimen showed a submucosal tumor with a pitted surface 11 x 11 mm in size, located at the terminal ileum. Histopathological examination revealed a well-differentiated endocrine cell carcinoma with an invasion depth to the muscularis propria. Immunohistochemical analysis showed the tumor cells to be chromogranin A and CD56-positive. The patient had no sign of recurrence for 16 months.

Surgical decision-making and prioritization for cancer patients at the onset of the COVID-19 pandemic: A multidisciplinary approach.

In the midst of the coronavirus disease 2019 (COVID-19) pandemic, governmental agencies, state medical boards, and healthcare organizations have called for restricting "elective" operations to mitigate the risk of transmission of the virus amongst patients and healthcare providers and to preserve essential resources for potential regional surges of COVID patients. While the fear of delaying surgical care for many of our patients is deeply challenging for us as cancer care providers, we must balance our personal commitment to providing timely and appropriate oncologic care to our cancer patients with our societal responsibility to protect our patients (including those on whom we are operating), co-workers, trainees, families, and community, from undue risks of contracting and propagating COVID-19. Herein, we present guidelines for surgical decision-making and case prioritization developed among all adult disease specialties in the MD Anderson Cancer Center Departments of Surgical Oncology and Breast Surgical Oncology in Houston, Texas.

Prognostic factor of recurrence for resected digestive endocrine tumors.

BACKGROUND/AIMS: The aim of the present study was to identify the factors of recurrence for digestive endocrine tumours resected with curative intent. METHODOLOGY: 170 endocrine digestive tumours were reviewed from January 1997 to January 1997, Twenty eight patients were selected in this study. Localization of tumours were as follows: 14 duodenopancreatic (DP) and 14 Digestive (DT: 9 small bowel and 4 appendix). The following factors were investigated: primary site, hormonal clinical symptom, and differentiation. RESULTS: Twenty eight patients (12 men) were selected. Median age was 48 (range, 23-79) yrs. All resection of metastasis were performed during the same procedure of primary tumour resection. There were 14 DT and 14 non functional DP tumours. For 28 patients, the only factor of recurrence was endocrine pancreatic tumour (p = 0.02). For non functional DP, the rate of recurrence was significantly dependent on histology and the expression of ki67 antigen and presence of metastasis. Survival free of disease for DT were: 100%,80% at 1, 5 yrs, and for DP they were 93%, 50%, 33% at 1, 3, 5 yrs, respectively. CONCLUSION: The expression of the Ki67 antigen and differentiation seem to be good indicators for DP recurrence and may need an adjuvant treatment despite the R0 resection.

Main pancreatic duct obstruction due to a small nonfunctioning endocrine tumor of the pancreas.

A 65-year-old Japanese male was referred to our hospital for evaluation of a main pancreatic duct obstruction. Two months previously, he had suffered an attack of acute pancreatitis that was resolved with conservative treatment. Dynamic contrast-enhanced study by multidetector row computed tomography revealed a well-enhanced 5 x 5 mm mass in the head of the pancreas with dilatation of the main pancreatic duct in the body and tail. On endoscopic retrograde pancreatography, obstruction of the main pancreatic duct in the head of the pancreas was noted. Pancreatic juice cytology was nondiagnostic. Endoscopic ultrasonography demonstrated a well-defined hypoechoic mass, about 5 mm in size, with distal main pancreatic duct dilatation. The patient underwent elective pylorus-preserving pancreaticoduodenectomy. Pathological examination revealed a well-differentiated endocrine tumor of the pancreas of uncertain behavior, 5 mm in size. Immunohistochemically, the tumor was diffusely positive for chromogranin A and synaptophysin, and focally it was positive for insulin, glucagon, and CA19-9; it was negative for gastrin. The final diagnosis was main pancreatic duct obstruction secondary to a nonfunctioning endocrine tumor of the pancreas of uncertain behavior. Of note, a small nonfunctioning endocrine tumor of the pancreas is important in the differential diagnosis of main pancreatic duct obstruction demonstrated by radiographic examinations.

Combined carcinoid and mixed (composite) glandular - endocrine cell carcinoma of the stomach in atrophic gastritis.

We describe a case of gastric carcinoid and inflammatory fibroid polyp concomitant with a composite tumor of the gastric antrum composed of poorly differentiated adenocarcinoma - endocrine carcinoma with immunohistochemical documentation of endocrine and non endocrine differentiation in a 67-year-old man with atrophic gastritis and intestinal metaplasia. When gastrectomy was carried out, two lymph nodes along the greater curvature harbored metastasis from carcinoid. The same occurrence is reported in several cases in the literature, which suggests that the association of gastric carcinoid to adenocarcinoma could point to the malignant nature of carcinoid. Furthermore, the findings in this patient reinforce the concept that the epithelial and neuroendocrine cells of the gastrointestinal tract both result from multidirectional differentiation of a primitive cell.