A Rare Case of Sclerosing Variant of Pancreatic Neuroendocrine Tumor-Expressing Glucagon

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S63-S63
Author(s):  
Rong Xia ◽  
Maryam Noori Koloori ◽  
Mohamed Alshal ◽  
Amir Dehghani ◽  
Qiang Xie ◽  
...  

Abstract Introduction Sclerosing variant of pancreatic neuroendocrine tumor (PanNET) is a rare variant of PanNET characterized by decreased tumor cellularity and prominent stromal fibrosis, and it is considered associated with serotonin expression. We herein present an unusual case of a sclerosing PanNET expressing glucagon. Methods The patient, a 75-year-old woman with refractory diabetes, scaly erythematous skin plaques in the eyelid, bilateral elbows and scalp, and episodes of nausea and poor appetite, presented to emergency department for acute abdominal pain. Abdominal computed tomography showed a 17-mm pancreatic head mass. Endoscopic ultrasound with fine-needle biopsy was performed for histopathological examination, followed by pancreaticoduodenectomy. Results Biopsy showed a background of predominantly blood and benign strips of pancreatic parenchyma and scanty fragments of fibrotic hyalinized stroma containing medium-sized atypical monotonous cells with round-to-ovoid nuclei, smooth borders, and inconspicuous nucleoli. These cells were difficult to characterize morphologically due to the scanty sampling. Immunohistochemical stains revealed the atypical cells were positive for AE1/AE2, synaptophysin, chromogranin, and glucagon and negative for insulin, serotonin, and pancreatic polypeptide. The lack of heterogeneous peptide cell composition supported a neoplastic proliferation of neuroendocrine cells. The pancreaticoduodenectomy specimen showed a 1.2-cm white firm mass in the pancreatic head. Histopathological study showed that, in a sclerotic background, the tumor cells grew mostly in trabecular patterns and occasionally in single cells, infiltrating into adjacent pancreatic parenchyma, and showed the same immunoprofile features as in the biopsy specimen. The proliferative index was <3%, supporting the diagnosis of low-grade sclerosing PanNET. The patient recovered well postsurgery. Moreover, the episodes of nausea and loss of appetite improved postsurgery. Patient did not show up for follow-up appointments in the dermatology clinic. Conclusion Sclerosing PanNET is challenging for diagnosis, especially on small biopsy specimens. Immunohistochemistry helps establish the diagnosis. Our case demonstrates a rare presentation of sclerosing PanNET expressing glucagon.

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Randhir Sagar Yadav ◽  
Ashik Pokharel ◽  
Shumneva Shrestha ◽  
Ashbita Pokharel ◽  
Deepshikha Gaire ◽  
...  

Mixed serous-neuroendocrine neoplasm constitutes pancreatic serous cystic neoplasms and pancreatic neuroendocrine tumor, two tumor components with different underlying pathologies. The differentiation of these tumors is important as the management and prognosis depend on the pancreatic neuroendocrine tumor component. We report a case of mixed serous-neuroendocrine neoplasm in a 47-year-old female who presented with epigastric pain abdomen for two years. Imaging studies, tumor markers, thorough systemic evaluation, surgical resection, histopathological examination, and timely follow-up constituted our management approach. A 4 cm × 4 cm mass in the distal pancreas with multiple cysts in the pancreatic parenchyma containing serous fluid on distal pancreatectomy and splenectomy was found. The histopathological examination revealed combined benign serous cystadenoma and neuroendocrine tumor. She did not have any recurrence or metastasis by four years of follow-up.


2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A999-A1000
Author(s):  
Monique Maher ◽  
Dimpi Desai ◽  
Mandeep Bajaj

Abstract Background: Patients with an insulinoma, a type of pancreatic neuroendocrine tumor, typically present with fasting hypoglycemia (1). Occurrence of exclusively postprandial hypoglycemia as a result of a predominantly proinsulin-secreting metastatic neuroendocrine tumor is rare (2). Clinical Case: A 69-year-old man presented with episodes of postprandial blurry vision, sweating and confusion for the last two years that were becoming more frequent over the last several weeks. Self-monitoring of blood glucose at home revealed postprandial hypoglycemia (45-70mg/dl) and symptoms were consistent with Whipple’s triad. Continuous glucose monitoring over 14-days via Dexcom G6 showed no nocturnal or fasting hypoglycemia and revealed only postprandial hypoglycemia within one-two hours after meals. Laboratory measurements were performed at 8am in fasting state which revealed a blood glucose of 97mg/dl, insulin level 7.8 µIU/ml (2-21 µIU/ml), c-peptide 1.67 ng/ml (1.1-4.4 ng/ml) and elevated proinsulin level of 39 pmol/l (&lt;8.0 pmol/l). An outpatient fast was conducted in the clinic, and when serum blood glucose dropped to 47mg/dL (21 hours after the initiation of the fast), insulin (6 µIU/ml) and c-peptide (2.0ng/ml) levels were detectable with an elevated proinsulin (20.8pmol/L) level. CT abdomen and pelvis showed a 1.6cm hyperenhancing lesion in the distal body of the pancreas. He underwent endoscopic ultrasonography with fine-needle aspiration, confirming the diagnosis of a pancreatic neuroendocrine tumor. Distal pancreatectomy and splenectomy were performed to resect the tumor which led to resolution of his symptoms. Pathology revealed a low grade well-differentiated neuroendocrine tumor with lymphovascular invasion and regional lymph node metastases. Conclusion: Pancreatic neuroendocrine tumor should be considered in post-prandial hypoglycemia, even in the absence of fasting hypoglycemia. Measuring proinsulin is essential in the diagnostic workup of insulinoma causing hypoglycemia References: 1. Placzkowski KA, Vella A, Thompson GB, Grant CS, Reading CC, Charboneau JW, et al. Secular trends in the presentation and management of functioning insulinoma at the Mayo Clinic, 1987-2007. J Clin Endocrinol Metab. 2009;94(4):1069-73.2. Murtha TD, Lupsa BC, Majumdar S, Jain D, Salem RR. A Systematic Review of Proinsulin-Secreting Pancreatic Neuroendocrine Tumors. J Gastrointest Surg. 2017;21(8):1335-41.


2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Larissa Brussa Reis ◽  
Daniele Konzen ◽  
Cristina Brinckmann Oliveira Netto ◽  
Pedro Moacir Braghirolli Braghini ◽  
Gabriel Prolla ◽  
...  

Abstract Background Tuberous Sclerosis Complex (TSC) is a complex and heterogeneous genetic disease that has well-established clinical diagnostic criteria. These criteria do not include gastrointestinal tumors. Case presentation We report a 45-year-old patient with a clinical and molecular diagnosis of TSC and a family history of cancer, presenting two rare associated findings: gastrointestinal polyposis and pancreatic neuroendocrine tumor. This patient was subjected to a genetic test with 80 cancer predisposing genes. The genetic panel revealed the presence of a large pathogenic deletion in the TSC2 gene, covering exons 2 to 16 and including the initiation codon. No changes were identified in the colorectal cancer and colorectal polyposis genes. Discussion and conclusions We describe a case of TSC that presented tumors of the gastro intestinal tract that are commonly unrelated to the disease. The patient described here emphasizes the importance of considering polyposis of the gastrointestinal tract and low grade neuroendocrine tumor as part of the TSC syndromic phenotype.


2020 ◽  
pp. 115-115
Author(s):  
Dragan Eric ◽  
Vladimir Milosavljevic ◽  
Boris Tadic ◽  
Dragan Gunjic ◽  
Milos Bjelovic

Introduction Introduction. Neuroendocrine tumors of the pancreas are rare neoplasms. They are divided into two groups: functional and non-functional. Non-functional tumors represent a diagnostic challenge, given that they often remain asymptomatic and are diagnosed as an incidental finding. Case outline. We present a patient in whom the tumor was discovered at the junction of the body and the tail of the pancreas on the dorsal side. The patient had no specific symptomatology, with no loss in body weight. Considering the conducted diagnostics and the condition of the patient, we decided to perform a laparoscopic enucleation. This procedure has been applied in a safe and efficient manner, so the operative and postoperative course passed without complications. The definitive histopathological examination confirmed the finding that it was a non-functional pancreatic neuroendocrine tumor. Conclusion. Laparoscopic enucleation is an effective and safe treatment modality of these tumors with well-known advantages compared to open surgery, but there is always a tending to improve already existing results and thus to contribute not only to treatment but to greater and better comfort of the patient


2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Adam Purdy ◽  
Firas Ido ◽  
Deborah Stahlnecker

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is an atypical pulmonary disorder with limited understanding. Given the rare nature of this disease, it is essential to obtain adequate tissue pathology to confirm the diagnosis. This disease is mainly diagnosed in middle-aged, nonsmoking females, and it is now accepted as a precursor lesion to pulmonary carcinoid tumors. DIPNECH presents with characteristic radiographic and histologic findings, but its diagnosis, management, and prognosis are often underrecognized and poorly understood. Those with symptoms may present with shortness of breath, wheezing, and persistent cough and are often misdiagnosed with reactive airway disease. Pulmonary function testing may reveal airflow obstruction and air trapping. Imaging is characterized by multiple lung nodules, typically less than 5 mm in size, with a background mosaic attenuation on computed tomography imaging. Histologically, DIPNECH can be suspected based on the presence of hyperplastic neuroendocrine cells. DIPNECH is considered a precursor to invasive neuroendocrine tumor, and up to 50% of patients may have a well-differentiated neuroendocrine tumor at the time of presentation. Here, we present the case of a 46-year-old female with a history of ulcerative colitis on mesalamine who presented with a 6-month history of ongoing shortness of breath, chest tightness, wheezing, and cough. She was initially diagnosed with asthma before imaging later revealed as multiple pulmonary nodules with a diffuse mosaic pattern. Using robotic-assisted navigational bronchoscopy, she underwent sampling of a dominant 1.8 cm right middle lobe pulmonary nodule and pathology was consistent with low-grade neuroendocrine tumor.


2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Marcello Moro Queiroz ◽  
Carlos Diego Holanda Lopes ◽  
Alessandra Corte Real Salgues ◽  
Felipe de Galiza Barbosa ◽  
Emerson Shigueaki Abe ◽  
...  

Abstract Background Pancreatic neuroendocrine tumor (PNET) is a subgroup of neuroendocrine tumor (NET) that has unique biology and natural history. The histological classification has a major role in the management of this pathology, but in recent years Gallium 68 dotatate (68Ga-DOTA) scanning is at the center of a discussion about how these imaging technologies can modify clinical management of neuroendocrine tumors and how their results are correlated to Ki67 index. Method We hereby describe a case of a patient that investigated an unspecific stable pancreatic nodule suspected of high-grade NET after evaluation with 68Ga-DOTATOC positron emission tomography—computed tomography (PETCT) and 18F-Fluorodeoxyglucose (18F-FDG) PETCT. Results The images corroborate the hypothesis of high-grade NET based on the standard uptake value (SUV) described in both image exams (16.4 in 18FDG PETCT and 9.2 in 68Ga-DOTATOC PETCT). After surgery, the histopathological analyses revealed a localized grade 2 well-differentiated NET, Ki-67 of 4.7, glucose transport proteins 1 (GLUT1) negative by immunohistochemistry, evidencing a rare case of mismatch between the functional image and the in vivo characterization of the neoplasm. Conclusion Functional imaging of neuroendocrine tumors with different modalities of PETCT is a well-described strategy for evaluating PNET and can dictate conducts in some cases. However, histopathological analysis is crucial to confirm the grade and prognosis related to this disease.


2021 ◽  
Author(s):  
Marcello Moro Queiroz ◽  
Carlos Diego Holanda Lopes ◽  
Alessandra Corte Real Salgues ◽  
Felipe de Galiza Barbosa ◽  
Emerson Shigueaki Abe ◽  
...  

Abstract BackgroundPancreatic neuroendocrine tumor (PNET) is a subgroup of neuroendocrine tumor (NET) that has unique biology and natural history. The histological classification has a major role in the management of this pathology, but in recent years Gallium 68 dotatate (68Ga-DOTA) scanning is at the center of a discussion about how these imaging technologies can modify clinical management of neuroendocrine tumors and how their results are correlated to Ki67 index.MethodWe hereby describe a case of a patient that investigated an unspecific stable pancreatic nodule suspected of high-grade NET after evaluation with 68Ga-DOTATOC positron emission tomography - computed tomography (PETCT) and 18F-Fluorodeoxyglucose (18F-FDG) PETCT. ResultsThe images corroborate the hypothesis of high-grade NET based on the standard uptake value (SUV) described in both image exams (16.4 in 18FDG PETCT and 9.2 in 68Ga-DOTATOC PETCT). After surgery, the histopathological analyses revealed a localized grade 2 well differentiated NET, Ki-67 of 4.7, evidencing a rare case of mismatch between the functional image and the in vivo characterization of the neoplasm.ConclusionFunctional imaging of neuroendocrine tumors with different modalities of PETCT is a well-described strategy for evaluating PNET and can dictate conducts in some cases. However, histopathological analysis is crucial to confirm the grade and prognosis related to this disease. Besides the rarity of this case, our patient presented a mismatch between imaging and histopathological analysis, with the former one allowing active surveillance based on a low-grade neuroendocrine tumor.


Author(s):  
M. Ya. Belikova ◽  
T. N. Trofimova ◽  
M. Yu. Kabanov ◽  
K. V. Sementsov

Objective: optimization of the technique and study of the possibilities of MR-elastography in assessing the rigidity of the pancreatic parenchyma under normal conditions, with fibrotic changes and adenocarcinoma. Materials and methods. MR elastography was performed for 15 patients with no history and clinical laboratory data indicating the presence of pancreatic diseases, 20 patients with pancreatic adenocarcinoma at the stage of preoperative planning to assess the rigidity of the tumor and parenchyma, in order to predict the probability of pancreatic fistulas. With the application of special software, the quantitative values of stiffness in kPa were calculated, a qualitative analysis of the state of the parenchyma was carried out using color parametric maps. Results. The stiffness of the pancreatic parenchyma normally ranged from 1,54 to 2,87 kPa, averaging 2,5 kPa. The unchanged parenchyma was characterized by blue staining on color parametric maps in the hardness range from 0 to 8 kPa, violet in the range from 0 to 20 kPa. In patients with a tumor process in all cases there was a low-grade adenocarcinoma of the pancreatic head, verified by histological data. The rigidity of adenocarcinoma varied from 6 to 9 kPa, on the color parametric maps there was a red staining of the tumor in the range of hardness of 0– 8 kPa, yellow in the range of 0–20 kPa. In the postoperative period, the pancreatic fistula on the background of preoperative preparation was formed only in 3 patients with normal pancreatic parenchyma, according to elastography rigidity indicators. Conclusion. MR-elastography is an informative method for quantitative and qualitative analysis of the stiffness of pancreatic parenchyma, and effective instrument for predicting the development of pancreatic fistulas.


2019 ◽  
Vol 31 (1) ◽  
pp. 1-8
Author(s):  
Khadiza Khanam ◽  
Shah Md Badruddoza ◽  
SM Asafudullah ◽  
Md Khalilur Rahman ◽  
Arefa Sultana ◽  
...  

Breast cancer is the second common cause of cancer death in women of our country. Treatment of breast cancer depends on a number of parameters known as pathological and biological prognostic markers. Knowledge of the tumour grade would avoid under treatment of high grade carcinomas and over treatment of low grade carcinomas. Out study evaluated the feasibility of a grading system on FNA of duct cell carcinoma (NOS) and its ability to predict the histologic grade of the surgical specimens. The two widely practice grading system; Robinson’s cytological grading and Scraff Bloom Richardson histological grading system were utilized for this purpose. Various reports quote varied reproducibility, ranging from 50 to 90% correlation between histologic and cytologic grade. In this study, the correlation between histologic and cytologic grade is 86.3%. In this study association between grading by FNAC and histology was statistically highly significant (χ2=27.66, df=1, p<0.05) sensitivity 100% and specificity 58.33%. Maximum correlation was noted in grade-I. Out of 53 cases 46 showed correlation between histologic and cytologic grading, only 7 cases showed no correlation. Histologic grade were higher than cytologic grade and maximum were within one grade difference. The reasons for discrepancies between cytologic and histologic grades appear to be due to lack of histologic parameter of mitosis in cytology. In this study, a total 57 cases of breast lump were selected for FNAC; of them 56 were female and 1 male. Out of 57 cases, 44 cases had duct cell carcinoma (DCC) and 13 cases had atypical finding such as small cells, inconspicuous nucleoli. Fifty three cases were diagnosed as DCC and lobular carcinoma by histopathological study. After confirmation of the diagnosis of breast cancer by histopathological examination ER, PR determination by immunohistochemistry was done in all 53 cases of breast cancer. FNAC has good sensitivity (85.29%) and very high specificity (100%). It can replace the open biopsy in the majority cases of clinically malignant disease. TAJ 2018; 31(1): 1-8


2021 ◽  
Author(s):  
Sebnem Ece Eksi ◽  
Alex Chitsazan ◽  
Zeynep Sayar ◽  
Andrew Fields ◽  
Ryan Kopp ◽  
...  

Approximately, 30% of early-stage localized prostate cancer cases reoccur within 5 to 10 years [1, 2]. However, identifying precise molecular subtypes attributable to specific stages of prostate cancer has proven difficult due to high heterogeneity within localized tumors [3-5]. Bulk assays represent a population average, which is a result of the heterogeneity that exists at the individual prostate cancer cell level [6]. Here, we sequenced the accessible chromatin regions of 14,424 single-cells collected from 18 fresh-frozen prostate tumors using sci-ATAC-seq [7, 8]. We observed that shared chromatin features among low-grade prostate cancer epithelial cells were lost in high-grade tumors. Despite this loss, all high-grade tumors exhibited an enrichment for FOXA1, HOXB13 and CDX2 transcription factor binding sites within their accessible chromatin regions, indicating a shared trans-regulatory program. Single-cell analysis of the differentially accessible regions in high- versus low-grade prostate tumors identified two unique genes encoding neuronal adhesion molecules, NRXN1 and NLGN1. We found that NRXN1 and NLGN1 are expressed in the epithelial luminal, basal and neuroendocrine cells, as well as the immune, endothelial and neuronal cell types in all prostate tumors. Overall, these results provide a deeper understanding of the active gene regulatory networks in low- and high-grade prostate tumors at a striking resolution and provide critical insights for molecular stratification of the disease.


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