: Pancreatic neuroendocrine tumors (PanNETs) are rare neoplasms that include even rarer variants that may pose different diagnostic problems, especially in fine needle aspiration cytology (FNAC). We describe the diagnostic clues of the amyloid-rich variant of PanNETs in endoscopic ultrasound (EUS)-guided fine needle aspiration cytology (EUS-FNAC). Three cases of PanNETs with an amyloid-rich stromal component were retrieved and retrospectively reviewed. For every case, the pancreatic lesion was investigated by a EUS-FNAC procedure. The final diagnosis was supported by immunocytochemistry and Congo red staining. All cases had similar EUS-FNAC features: neoplastic cells were entrapped in an eosinophilic, homogeneous dense and amorphous matrix. The neuroendocrine nature was confirmed by immunoexpression of synaptophysin and chromogranin A, while the amorphous stroma was characterized as amyloid based on positive Congo red staining. Regarding the hormonal profile, no insulin or proinsulin reactivity was observed, but all cases were diffusely positive for amylin. The diagnosis of uncommon variants of PanNETs, such as the amyloid-rich, is challenging especially in EUS-FNAC procedures because of a unique and misleading morphology, potentially mimicking fibrotic conditions and amyloid deposition within systemic amyloidosis. In cytology specimens, the presence of amorphous material requires amyloid deposition to be considered in the differential diagnosis of pancreatic neoplasms with neuroendocrine phenotype.
Insulin-producing pancreatic neuroendocrine tumors (PanNETs)/insulinomas are generally considered to be indolent tumors with an excellent prognosis after complete resection. However, some insulinomas have a poor prognosis due to relapses and metastatic disease. Recently, studies in non-functional PanNETs indicated that behavior can be stratified according to alpha- and beta-cell differentiation, as defined by expression of the transcription factors ARX and PDX1, respectively. It is unknown wheth...
#1Wei Ling(GLMU: Guilin Medical University)H-Index: 9
#2Yan-Mei Huang(GLMU: Guilin Medical University)H-Index: 8
Last. Hai-Lu Zhao(GLMU: Guilin Medical University)H-Index: 20
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: The histopathological hallmark of type 2 diabetes is islet amyloid implicated in the developing treatment options. The major component of human islet amyloid is 37 amino acid peptide known as amylin or islet amyloid polypeptide (IAPP). Amylin is an important hormone that is co-localized, copackaged, and co-secreted with insulin from islet β cells. Physiologically, amylin regulates glucose homeostasis by inhibiting insulin and glucagon secretion. Furthermore, amylin modulates satiety and inhibi...
Last. Shereen Ezzat(Princess Margaret Cancer Centre)H-Index: 40
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The management of pancreatic neuroendocrine tumors (PanNETs) involves classification into non-functional or functional PanNET, and as localized or metastatic PanNET. In addition, while most PanNETs are sporadic, these endocrine neoplasms can also be manifestations of genetic syndromes. All these factors may assist in forming a risk stratification system permitting a tailored management approach. Most PanNETs are classified as non-functional because they are not associated with clinical sequelae ...
Kleine (<2 cm) sporadische neuroendokrine Pankreasneoplasien (pNENs) werden in funktionell aktive Tumoren und nichtaktive Tumoren unterteilt. Funktionell aktive pNENs setzen unterschiedliche Hormone wie Insulin, Gastrin, Glukagon oder vasoaktives intestinales Hormon frei und fuhren so, unabhangig von ihrer Grose, zu schweren Symptomen wie z. B. zu Hypoglykamie, Koma (Insulinom), Durchfallen (Gastrinom, Vipoma), Magen-Darm-Ulzera (Gastrinom) und Hyperglykamien (Glukagonom). Die asymptomatischen n...
Background and study aim Standard endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) procedures involve use of no-suction or suction aspiration techniques. A new aspiration method, the stylet slow-pull technique, involves slow withdrawal of the needle stylet to create minimum negative pressure. The aim of this study was to compare the sensitivity of EUS-FNA using stylet slow-pull or suction techniques for malignant solid pancreatic lesions using a standard 22-gauge needle. Patients an...
#1Sönke Detlefsen(OUH: Odense University Hospital)H-Index: 21
#2Günter Klöppel(TUM: Technische Universität München)H-Index: 38
In 2011, chronic fibroinflammatory processes occurring simultaneously or metachronously in various organs and associated with elevated IgG4 serum levels and/or tissue infiltration with IgG4-positive plasma cells have been recognized as manifestations of a systemic disorder called IgG4-related disease (IgG4-RD). The histologic key findings are lymphoplasmacytic infiltration rich in IgG4-positive plasma cells combined with storiform fibrosis and obliterative phlebitis. Among the organs mainly affe...
Last. Mauro Papotti(UNITO: University of Turin)H-Index: 98
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Neuroendocrine neoplasms (NENs) are a heterogeneous group of epithelial neoplastic proliferations that irrespective of their primary site share features of neural and endocrine differentiation including the presence of secretory granules, synaptic-like vesicles, and the ability to produce amine and/or peptide hormones. NENs encompass a wide spectrum of neoplasms ranging from well-differentiated indolent tumors to highly aggressive poorly differentiated neuroendocrine carcinomas. Most cases arise...
#1Yun Wang(HUST: Huazhong University of Science and Technology)H-Index: 7
#2Qian Chen(HUST: Huazhong University of Science and Technology)H-Index: 4
Last. Bin Cheng(HUST: Huazhong University of Science and Technology)H-Index: 9
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Several suction techniques have been developed recently to enhance tissue acquisition when sampling solid lesions using endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA). The aim of this study is to determine whether a new modified wet suction technique (MWST) compared with the conventional dry suction technique (DRST) shall present better outcomes with respect to diagnostic yield and specimen quality of solid lesions in the intra-abdomen and mediastinum. This is a single-blind, rand...
#2Ashley Vogel(Thomas Jefferson University Hospital)H-Index: 1
Last. Haroon Shahid(Thomas Jefferson University Hospital)H-Index: 5
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We report a rare case of diffuse replacement of the pancreas with neuroendocrine tumour mimicking chronic pancreatitis. A 55-year-old female with no significant past medical history initially presented with abdominal pain in 2006. A CT of the abdomen and pelvis was performed, revealing diffuse pancreatic parenchymal calcifications with mild pancreatic ductal dilatation and no discrete mass. She was diagnosed with chronic pancreatitis and followed clinically until 2015, where she presented with r...