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American Journal of Surgery Feb 2021How malignant insulinomas present relative to benign insulinomas is unknown. (Comparative Study)
Comparative Study
BACKGROUND
How malignant insulinomas present relative to benign insulinomas is unknown.
METHODS
A single-institution retrospective study identified patients with insulinoma. Malignancy was defined by distant metastases, positive lymph node(s), T stage of 4, direct invasion into surrounding peripancreatic tissue, or presence of lymphovascular invasion. Wilcoxon Rank Sum tests and Kaplan-Meier analysis were used.
RESULTS
A total of 311 patients were identified: 51 malignant and 260 benign. Patients with malignant insulinoma presented with higher levels of insulin, proinsulin, and c-peptide. Malignant lesions were larger: 4.2 ± 3.2 vs 1.8 ± 0.8 cm in benign lesions, p < 0.01. Overall survival at 5 years was 66.8% vs 95.4% for malignant and benign insulinoma respectively, p < 0.01.
CONCLUSIONS
Larger size of insulinoma and increased serum β-cell polypeptide concentrations were associated with malignancy. Malignant insulinoma has poorer survival. Further work-up to rule out malignancy may be indicated for larger pancreatic lesions and for patients with higher pre-operative insulin and pro-insulin.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; C-Peptide; Child; Diagnosis, Differential; Female; Humans; Insulin; Insulinoma; Kaplan-Meier Estimate; Lymphatic Metastasis; Male; Middle Aged; Neoplasm Invasiveness; Pancreas; Pancreatic Neoplasms; Retrospective Studies; Young Adult
PubMed: 32873371
DOI: 10.1016/j.amjsurg.2020.08.003 -
Advances in Clinical and Experimental... Feb 2020Neuroendocrine tumors (NET) of the gastrointestinal tract and pancreas are extremely rare in the pediatric population and limited data is available. In most cases, NET... (Review)
Review
Neuroendocrine tumors (NET) of the gastrointestinal tract and pancreas are extremely rare in the pediatric population and limited data is available. In most cases, NET of the gastrointestinal tract in children are located in the appendix. Pancreatic NET are a small but partially distinct group of the gastrointestinal neuroendocrine neoplasms. The most common in this group are insulinomas; however, in some research, the gastrinoma type neoplasms are perceived to be most common in children. This study reviews the typical clinical presentation, appropriate diagnostics, staging, and treatment of these uncommon neoplasms. It is important to know the epidemiology and symptomatology in this age group despite the fact that the majority of physicians treating the youngest patients will never have to deal with it. This will facilitate an early diagnosis in case of symptoms that may suggest neuroendocrine cancer. It appears necessary to create harmonized recommendations regarding the diagnosis, treatment and post-treatment follow-up for pediatric patients.
Topics: Child; Gastrointestinal Neoplasms; Humans; Neoplasm Staging; Neuroendocrine Tumors; Pancreatic Neoplasms; Pediatricians
PubMed: 32091671
DOI: 10.17219/acem/111806 -
Revista de Gastroenterologia Del Peru :... 2022Gastrinomas are neuroendocrine tumors usually located in the duodenum and pancreas, in the context of a Multiple Endocrine Neoplasm and forming a Zollinger-Ellison...
Gastrinomas are neuroendocrine tumors usually located in the duodenum and pancreas, in the context of a Multiple Endocrine Neoplasm and forming a Zollinger-Ellison syndrome. The location of this type of lymph node tumor is extremely unusual and its early diagnosis constitutes a real challenge to be able to establish an adequate treatment and manage the complications that these entail. We present the case of a 64-year-old male patient with a lymph node gastrinoma and whose surgical removal resulted in the immediate remission of the patient's symptoms.
Topics: Male; Humans; Middle Aged; Gastrinoma; Pancreatic Neoplasms; Zollinger-Ellison Syndrome; Multiple Endocrine Neoplasia; Lymph Nodes
PubMed: 36513358
DOI: No ID Found -
Journal of Surgical Oncology May 2021Metastatic pancreatic ductal adenocarcinoma (PDAC) is a major cause of cancer-related mortality in 2021. Cytotoxic therapies are the therapeutic mainstay for PDAC. The... (Review)
Review
Metastatic pancreatic ductal adenocarcinoma (PDAC) is a major cause of cancer-related mortality in 2021. Cytotoxic therapies are the therapeutic mainstay for PDAC. The recent approval of olaparib as maintenance therapy for germline BRCA1/2-mutated PDAC and pembrolizumab for mismatch repair deficient PDAC represent molecularly targeted approaches for this disease. Investigational therapeutic strategies include targeting the stroma, metabolism, tumor microenvironment, and the immune system, and selected approaches are reviewed herein.
Topics: Antineoplastic Combined Chemotherapy Protocols; Carcinoma, Pancreatic Ductal; Clinical Trials as Topic; Humans; Immunotherapy; Neoplasm Metastasis; Pancreatic Neoplasms; Randomized Controlled Trials as Topic
PubMed: 33831245
DOI: 10.1002/jso.26359 -
Pancreatology : Official Journal of the... Jan 2021Mixed neuroendocrine non-neuroendocrine neoplasms (MiNEN) of the pancreas and periampullary region are extremely rare and heterogeneous malignancies. Literature is...
BACKGROUND/OBJECTIVES
Mixed neuroendocrine non-neuroendocrine neoplasms (MiNEN) of the pancreas and periampullary region are extremely rare and heterogeneous malignancies. Literature is sparse, clinical management is not standardized and little is known about survival outcomes. The aim of this study was to identify pathological and radiological features of MiNEN and assess the outcome of surgical management.
METHODS
Patients undergoing surgery for pancreatic and periampullary MiNEN between 2001 and 2019 were retrospectively analysed based on a prospective database. Histological, radiological and clinical features were assessed. Survival was analysed in a nested case-control study and matched-pair analyses with pure neuroendocrine neoplasms (pNEN) and ductal adeno- or acinar cell carcinomas of the pancreas. A literature review with focus on survival after surgical resection was additionally performed.
RESULTS
Of 13 patients with MiNEN, 5 had acinar-MiNEN and 8 adeno-MiNEN. Two of 5 (40%) acinar-MiNEN and one adeno-MiNEN patients had liver metastases. All but one adeno-MiNEN (88%) showed preoperative radiological features of pancreatic adenocarcinoma, 3 of 5 (60%) acinar-MiNEN exhibited mainly neuroendocrine features. No surgical mortality was observed. The 5-year overall survival rate in all MiNEN was 40%. Five-year survival rate was 58% in adeno-MiNEN and comparable to that of matched ductal adenocarcinomas (36%) and pNEN (48%). Five-year overall survival rate was 20% in acinar-MiNEN, compared to 39% in acinar carcinoma patients and 59% in matched pNEN patients.
CONCLUSIONS
MiNEN are rare and difficult to distinguish from pure adenocarcinoma or neuroendocrine neoplasm preoperatively. Surgical resection would therefore be the treatment of choice in localized tumors.
Topics: Adenocarcinoma; Adult; Aged; Aged, 80 and over; Carcinoma, Acinar Cell; Carcinoma, Pancreatic Ductal; Case-Control Studies; Female; Humans; Kaplan-Meier Estimate; Male; Middle Aged; Neoplasm Metastasis; Neoplasms, Complex and Mixed; Neuroendocrine Tumors; Pancreatectomy; Pancreatic Neoplasms; Retrospective Studies; Survival Analysis; Treatment Outcome
PubMed: 33309225
DOI: 10.1016/j.pan.2020.11.020 -
Journal of the American Society of... 2023The World Health Organization (WHO), the International Academy of Cytology, and the International Agency for Research on Cancer have developed an approach to... (Review)
Review
The World Health Organization (WHO), the International Academy of Cytology, and the International Agency for Research on Cancer have developed an approach to standardized reporting of pancreaticobiliary cytopathology. The WHO Reporting System for Pancreaticobiliary Cytopathology (WHO System) revises the Papanicolaou Society of Cytopathology (PSC) System for Reporting Pancreaticobiliary Cytology published in 2015 and replaces the 6 PSC categories with 7 categories: "Insufficient/Inadequate/Nondiagnostic"; "Benign/Negative for malignancy"; "Atypical"; "Pancreaticobiliary neoplasm, low risk/grade (PaN-low)"; "Pancreatic neoplasm, high risk/grade (PaN-High)"; "Suspicious for malignancy"; and "Malignant". In the PSC system, there is a single category for "Neoplastic" lesions that includes 2 groups, 1 for benign neoplasms and 1 named "Neoplastic-other", dominated by premalignant intraductal neoplasms primarily intraductal papillary mucinous neoplasms and low-grade malignant neoplasms (pancreatic neuroendocrine tumors (PanNET) and solid pseudopapillary neoplasms (SPN). In the WHO System, benign neoplasms with virtually no risk of malignancy are included in the "Benign" category and low-grade malignancies (PanNET and SPN) are included in the "Malignant" category, as per the 5th edition of the WHO Classification of Digestive System Tumors, while the non-invasive pre-malignant lesions of the ducts are divided by the cytomorphological grade of the epithelium into PaN-low and PaN-high with distinctly different risks of malignancy. Within each category, key diagnostic cytopathologic features and the ancillary studies for diagnostic and prognostic evaluation, as well as the implications of diagnosis for patient care and management, are outlined. Reporting and diagnostic management options recognize the variations in the availability of diagnostic and prognostic ancillary testing modalities in low- and middle-income countries.
Topics: Humans; Pancreatic Neoplasms; Cytodiagnosis; Societies, Medical
PubMed: 37003924
DOI: 10.1016/j.jasc.2023.03.002 -
Journal of Gastrointestinal Surgery :... Jan 2021
Topics: Carcinoma, Pancreatic Ductal; Humans; Pancreas; Pancreatic Neoplasms
PubMed: 32394127
DOI: 10.1007/s11605-020-04638-y -
Expert Review of Anticancer Therapy Dec 2019: Neuroendocrine tumors of the pancreas (pNETs) represent only 1% to 2% of all pancreatic neoplasms. These tumors can be classified as functional or nonfunctional... (Review)
Review
: Neuroendocrine tumors of the pancreas (pNETs) represent only 1% to 2% of all pancreatic neoplasms. These tumors can be classified as functional or nonfunctional tumors; as sporadic or from a genetic origin; as neuroendocrine neoplasms or carcinoma. Over the last decade, diagnosis of pNETs has increased significantly mainly due to the widespread use of cross-sectional imaging. Those tumors are usually associated with a good prognosis. Surgery, the only curative option for those patients, should always be discussed, ideally in a multidisciplinary team setting.: We discuss ), the preoperative management of pNETs and the importance of accurate diagnosis, localization, grading and staging with computed tomography, magnetic resonance imaging, endoscopic ultrasound, and nuclear medicine imaging; ), surgical indications and ), the surgical approach (standard pancreatectomy pancreatic-sparing surgery).: The treatment option of all patients presenting with pNETs should be discussed in a multidisciplinary team setting with surgeon's experienced in both pancreatic surgery and neuroendocrine tumor management. A complete preoperative imaging assessment - morphological and functional - must be performed. Surgery is usually recommended for functional pNETs, nonfunctional pNETs >2 cm (nf-pNETs) or for symptomatic nf-pNETs.
Topics: Humans; Neoplasm Grading; Neoplasm Staging; Neuroendocrine Tumors; Pancreatectomy; Pancreatic Neoplasms; Patient Care Team; Preoperative Care
PubMed: 31825691
DOI: 10.1080/14737140.2019.1703677 -
Der Pathologe Dec 2019Cytology has a key role in the step-wise diagnostic approach to pancreatic mass lesions. Brush cytology and ultrasound-guided endoscopic fine-needle aspiration provide... (Review)
Review
BACKGROUND
Cytology has a key role in the step-wise diagnostic approach to pancreatic mass lesions. Brush cytology and ultrasound-guided endoscopic fine-needle aspiration provide specimens for diagnosis prior to surgical or conservative therapy. The diagnostic system of the Papanicolaou Society of Cytopathology provides a conceptual framework for reporting these specimens. Cystic lesions represent a particular challenge in pancreatic cytology, as in many instances a purely morphological approach will not result in an adequate diagnostic interpretation. Noteworthy from a conceptual point of view is how the Papanicolaou Society System incorporates non-morphological methods: laboratory chemical (CEA >192 ng/ml) and molecular (KRAS and/or GNAS mutations) findings are part of the formal diagnostic criteria for neoplastic cysts.
RESULTS
The Bern experience shows that such an integrated approach results in a significantly increased diagnostic yield. Among 83 samples analyzed, adequate DNA could be extracted in 79 samples (95%). Next generation sequencing identified pathogenic mutations in 46 cases (58%). Of these, in 35 (76%) a neoplastic cyst could not have been diagnosed by morphology alone.
CONCLUSION
These findings illustrate a new perspective for diagnostic situations, where morphology alone does allow for a sufficient diagnostic work-up. Along this line of thinking, liquid biopsy should not be regarded as a replacement, but rather an extension of the cytology's diagnostic armamentarium, according to the principle of "doing more with less."
Topics: DNA Mutational Analysis; DNA, Neoplasm; Endoscopic Ultrasound-Guided Fine Needle Aspiration; Humans; Mutation; Pancreas; Pancreatic Cyst; Pancreatic Neoplasms
PubMed: 31705231
DOI: 10.1007/s00292-019-00697-4 -
Folia Medica Dec 2021Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is a relatively new entity that has gained increased attention because of its unique features - presence... (Review)
Review
Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is a relatively new entity that has gained increased attention because of its unique features - presence of different subtypes with different malignant potential, biological behavior, and prognosis, higher rates of recurrences and concomitant or metachronous pancreatic duct cancer. It is rare with an incidence of 4 to 5 cases per 100 000. The relative lack of experience significantly hampers decision making for surgery (pancreatic head resection, distal pancreatectomy or enucleation) or follow-up.Herein we present two cases managed by diametrically different tactic according to the risk stratification - distal pancreatectomy with splenectomy and observation, respectively. An up-to-date literature review on the key points in diagnostics, indications for surgery, the extent of surgery, follow-up, and prognosis is provided.The tailored approach based on risk stratification is the cornerstone of management. Absolute indications for surgery are the lesions with high-risk stigmata, whereas the worrisome features should be evaluated by endoscopic ultrasound and fine-needle aspiration. Main duct and mixed type are usually referred to surgery, whereas the management of a branch type is more conservative due to the lower rate of invasive cancer. Strict postoperative follow-up is mandatory even in negative resection margins due to a high risk for recurrences and metachronous lesions.Despite the guidelines, the intraductal papillary mucinous neoplasm remains a major challenge for clinicians and surgeons in the balance the risk/benefit of observation versus resection. Risk stratification plays a key role in decision-making. Future trials need to determine the optimal period of surveillance and the most reliable predictive factors for concomitant pancreatic duct cancer.
Topics: Adenocarcinoma, Mucinous; Carcinoma, Pancreatic Ductal; Humans; Pancreas; Pancreatectomy; Pancreatic Neoplasms; Recurrence; Retrospective Studies
PubMed: 35851243
DOI: 10.3897/folmed.63.e63071