Case report: composite pancreatic intraductal papillary mucinous neoplasm and neuroendocrine tumor: a new mixed neuroendocrine-non-neuroendocrine neoplasm?

Background Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) of the pancreas are extremely rare. Their pathogenesis and molecular landscape are largely unknown. Here, we report a case of mixed pancreatic intraductal papillary mucinous neoplasm (IPMN) and well-differentiated neuroendocrine tumor (NET) and identify its genetic alterations by next-generation sequencing (NGS). Case presentation A fifty-year-old male was admitted into the hospital for evaluation of a pancreatic lesion detected during a routine examination. Abdominal ultrasound indicated a hypoechoic mass of 2.6 cm at the head of the pancreas. Malignancy was suspected and partial pancreatectomy was performed. Thorough histopathological examination revealed a mixed IPMN-NET. In some areas, the two components were relatively separated, whereas in other areas IPMN and NET grew in a composite pattern: The papillae were lined with epithelial cells of IPMN, and there were clusters of NET nests in the stroma of papillary axis. NGS revealed shared somatic mutations (KRAS, PCK1, MLL3) in both components. The patient has been uneventful 21 months after the surgery. Conclusions Our case provides evidence of a common origin for mixed IPMN-NET with composite growth features. Our result and literature review indicate that KRAS mutation might be a driver event underlying the occurrence of MiNEN. We also recommend the inclusion of mixed non-invasive exocrine neoplasms and neuroendocrine neoplasms into MiNEN.

Endoscopic Ultrasound Guided Fine Needle Aspiration and Biopsy for Pancreatic Disease

The endoscopic ultrasound (EUS) is a device with an ultrasound probe on the tip of endoscope. We can observe the surrounding structures outside the alimentary tract by using EUS. It is also possible to get a tissue from the pancreatic lesion for histopathologic diagnosis by using EUS. The development of devices and techniques of EUS guided tissue acquisitions made it the choice of non-operative pathologic test for pancreatic diseases. This paper describes the clinical applications of this procedure in pancreatic lesions from the recent European and Korean guidelines, including how to choose the needle, role of rapid on site evaluation, usage of stylet, suction and fanning technique, how to process acquired specimen, procedure-related complications and educations of this method.

THROMBOELASTOGRAPHY IN ASSESSING THE HEMOSTATIC SYSTEM IN INTENSIVE CARE PATIENTS

The results of thromboelastography and standard coagulogram were analyzed in 35 patients aged from 18 to 86 who were treated in the resuscitation and intensive care unit. The majority of patients (34%) were hospitalized in the department with multisystem and concomitant injuries. The remaining patients were taken to the medical institution with different diagnoses (urolithiasis, liver cirrhosis, pancreatic lesion of various types, poisoning, peptic ulcer, sepsis). The data of coagulogram and thromboelastography at different stages of treatment were compared. In patients with the development of traumatic shock, the coagulogram parameters were changed to varying degrees depending on the stage of shock. At the first stage of shock, only an increase in soluble fibrin-monomer complexes by almost 2 times and a slight increase in fibrinogen dynamics were noted in the analysis. In a patient with stage 3 traumatic shock, the coagulogram parameters were within the normal range, but according to thromboelastography (EXTEM and FIBTEM tests), hypocoagulation due to the platelet link was noted. Only the coagulogram was evaluated in dynamics, hypocoagulation was noted in the indicators of internal and external hemostasis pathways: lengthening of the activated partial thromboplastin time, a decrease in the prothrombin index and an increase in the international normalized ratio, an increase in fibrinogen A and soluble fibrin-monomer complexes. In the group of male patients with closed craniocerebral trauma, an increase in soluble fibrin-monomer complexes in the coagulogram was always combined with changes in the FIBTEM test during thromboelastography. In most patients, no changes in the classical coagulogram tests immediately after the injury are noted. At this, thromboelastography makes it possible to make up for this deficiency at an earlier time, which indicates a high sensitivity of the method.

PBF-Guided Biopsy with a Novel Puncture Biopsy Forceps Needle—Feasibility Study

Endoscopic ultrasound (EUS) with fine needle aspiration (FNA) or biopsy (FNB) to diagnose lesions in the gastrointestinal tract is common. Demand for histology sampling to identify treatment-specific targets is increasing. Various core biopsy FNB needles to obtain tissue for histology are currently available, however, with variable (37–97%) histology yields. In this multicenter study, we evaluated performance, safety, and user experience of a novel device (the puncture biopsy forceps (PBF) needle). Twenty-four procedures with the PBF needle were performed in 24 patients with a suspected pancreatic lesion (n = 10), subepithelial lesion (n = 10), lymph node (n = 3), or pararectal mass (n = 1). In 20/24 (83%) procedures, the PBF needle yielded sufficient material for interpretation (sample adequacy). In 17/24 (71%), a correct diagnosis was made with the material from the PBF needle (diagnostic accuracy). All participating endoscopists experienced a learning curve. (Per)procedural technical issues occurred in four cases (17%), but there were no adverse events. The PBF needle is a safe and potentially useful device to obtain an EUS-guided biopsy specimen. As the design of the PBF needle is different to core biopsy FNB needles, specific training will likely further improve the performance of the PBF needle. Furthermore, the design of the needle needs further improvement to make it more robust in clinical practice.

Utility of CT to Differentiate Pancreatic Parenchymal Metastasis from Pancreatic Ductal Adenocarcinoma

Purpose: To report the computed tomography (CT) features of pancreatic parenchymal metastasis (PPM) and identify CT features that may help discriminate between PPM and pancreatic ductal adenocarcinoma (PDAC). Materials and methods: Thirty-four patients (24 men, 12 women; mean age, 63.3 ± 10.2 [SD] years) with CT and histopathologically proven PPM were analyzed by two independent readers and compared to 34 patients with PDAC. Diagnosis performances of each variable for the diagnosis of PPM against PDAC were calculated. Univariable and multivariable analyses were performed. A nomogram was developed to diagnose PPM against PDAC. Results: PPM mostly presented as single (34/34; 100%), enhancing (34/34; 100%), solid (27/34; 79%) pancreatic lesion without visible associated lymph nodes (24/34; 71%) and no Wirsung duct enlargement (29/34; 85%). At multivariable analysis, well-defined margins (OR, 6.64; 95% CI: 1.47–29.93; p = 0.014), maximal enhancement during arterial phase (OR, 6.15; 95% CI: 1.13–33.51; p = 0.036), no vessel involvement (OR, 7.19; 95% CI: 1.512–34.14) and no Wirsung duct dilatation (OR, 10.63; 95% CI: 2.27–49.91) were independently associated with PPM. The nomogram yielded an AUC of 0.92 (95% CI: 0.85–0.98) for the diagnosis of PPM vs. PDAC. Conclusion: CT findings may help discriminate between PPM and PDAC.

Primary Localization of a Hydatid Cyst in the Pancreas via Laparoscopic Treatment: A Case Report

Hydatid disease is mainly because of the Echinococcus granulosus at the larval stage. The liver and lung are its most consequences. The pancreatic hydatid cyst (PHC) incidence is very low (0.14%-2%). A 55- year-old female patient presented with epigastric pain for the last one year that the pain did not continue but during one months ago suffered continually. In physical examination, there was not any abdominal bulb, tenderness and rebound tenderness. A 54×59 mm cystic structure was observed by ultrasonography (USG) and Contrast-enhanced Computed Tomography (CT) in the pancreatic body with stone in the gallbladder. Amylase, lipase, and LFT levels were normal. The Anti-hydatid antibody was positive. During laparoscopic exploration, a hydatid cyst was found. Partial cystectomy with external drainage and cholecystectomy was performed once irrigation with scolicidal agent and evacuation of cystic contents was conducted. Histopathological biopsy reported Hydatid cyst. A pancreatic, hepatic cyst is a rare event. Hematogenous is the most common spread way. Cysts in the pancreatic head could be found with obstructive jaundice. Usually, cysts in the body and tail are known to be asymptomatic. USG, CT, and Hydatid serology are useful with the clinical diagnosis as well as monitoring the recurrence. An exploration via surgery is an option that includes pericystectomy, partial cystectomy with/without external drainage or omentopexy, marsupialization, or cysto-enterostomy, which is done. What makes this case unique is the laparoscopic method that we used instead of open surgery, which is a treatment of choice. The recommendation is pre-operative and postoperative antihelminthic (Albendazole). PHC could be present as pseudocyst or cystic neoplasm of the pancreas. For patients with endemic regions and laparoscopic surgery, differential diagnosis of the cystic pancreatic lesion should be noticed. Common surgery approach could be considered for such patients.

Metastatic Non-Functional Pancreatic Neuroendocrine Tumor in a Patient With a Pathogenic TSC1 Mutation

Introduction: Tuberous sclerosis complex (TSC) is an autosomal dominant multisystem disorder caused by mutations in two tumor suppressor genes (TSC1 and TSC2) encoding proteins critical in cell growth and proliferation and is associated with an increased risk for benign and malignant tumors. Pancreatic neuroendocrine tumors (PNET) occur in 1.5-1.8% of patients with TSC. They have been primarily reported in TSC2, with only a few cases in the context of TSC1. We describe a non-functional metastatic PNET in a patient with a known pathogenic TSC1 mutation. Case Description: A 49-year old female with a past medical history of bipolar disease, a family history of TSC1 in a daughter, a known pathologic TSC1 mutation (c.2356C>T), and previous history of renal cell carcinoma (RCC) was evaluated for a pancreatic lesion with likely liver metastases. Two years prior to the presentation, a 10 mm pancreatic lesion was initially noted on the CT scan during a renal mass evaluation. Nephrectomy confirmed the RCC. Two years later, surveillance imaging revealed two new lesions in the liver, and the pancreatic lesion had enlarged to 1.3 cm. She denied symptoms suggestive of a functional PNET, and her physical exam was unremarkable. Gallium-68 dotatate (GA-68) PET/CT scan demonstrated avid lesions of both the pancreatic tail and left hepatic lobe. Biopsy of the 3 cm liver mass revealed a metastatic high-grade neuroendocrine carcinoma (mib-1 proliferation index of 15%) favoring a primary PNET. Immunohistochemical stains were positive for chromogranin A, synaptophysin, EMA, PAX 8, CK 20, and villin. Stains for CA 19-9 and CK 7 were negative. Biochemical testing suggested that the PNET was non-functional. Specifically, serum concentrations of gastrin, somatostatin, glucagon, glucose, C-peptide, proinsulin, pancreatic polypeptide, chromogranin A, and vascular endothelial growth factor were within normal limits. The patient underwent distal pancreatectomy and resection of the liver mass. Pathological specimens confirmed the diagnosis of a PNET. The patient remained asymptomatic and was monitored regularly with MRI and GA-68 PET/CT scans without further evidence of the disease for 1.5 years. Conclusions: We conclude that this patient harboring a c.2356C>T TSC1 mutation developed a non-functional metastatic PNET. PNET is a likely component of TSC1. References: 1.Lodish, M. B., & Stratakis, C. A. (2010). Endocrine tumors in neurofibromatosis type 1, tuberous sclerosis, and related syndromes. Best practice & research Clinical endocrinology & metabolism, 24(3), 439-449.2.Dworakowska, D., & Grossman, A. B. (2009). Are neuroendocrine tumors a feature of tuberous sclerosis? A systematic review. Endocrine-Related Cancer, 16(1), 45.