scholarly journals Metastatic Insulinoma Presenting After Bariatric Surgery in a Patient Subsequently Diagnosed With MEN 1

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A999-A999
Author(s):  
Caroline A Poku ◽  
Shanika Prajani Samarasinghe
Keyword(s):  
Bariatric Surgery ◽  
Genetic Testing ◽  
Serum Glucose ◽  
The Body ◽  
Pancreatic Islet Cell ◽  
Ki 67 ◽  
Men 1 ◽  
Heritable Disorder ◽  
Well Differentiated ◽  
Fasting Hypoglycemia

Abstract Background: Hypoglycemia is a recognized complication of bariatric procedures due to changes in the gut hormonal milieu. Possible causes of hypoglycemia include dumping syndrome, nesidioblastosis and rarely insulinoma. MEN1 is a heritable disorder characterized by the occurrence of parathyroid, anterior pituitary and pancreatic islet cell tumors. Clinical Case: A 48 yo female with HTN, depression, PCOS, primary hyperparathyroidism s/p 3.5 gland parathyroidectomy 2008, facial angiofibroma 2011, obesity s/p gastric sleeve 2018, and a history of DMT2 presented with frequent episodes of hypoglycemia. DMT2 was diagnosed in 2003, but all DM drugs were withdrawn shortly after sleeve gastrectomy. One year after surgery, she started to develop primarily fasting hypoglycemia in the 40-50 mg/dl range confirmed on CGM. Symptoms included perioral numbness, diaphoresis and confusion which resolved with glucose tablets. She reported a 7 kg weight regain due to eating every 2 hours to minimize episodes but symptoms progressed. Family history was significant for HTN and DM. A 72 hour fast confirmed hyperinsulinemia with symptomatic hypoglycemia at a serum glucose of 37 mg/dl, a suppressed ß-hydroxybutyrate of 0.6 mmol/L, elevated proinsulin 7.7 ρmol/L and inappropriate normal c-peptide 0.8 ng/mL. The oral hypoglycemic panel was negative. A pancreatic protocol CT revealed a 1.2 cm heterogeneous arterial enhancing lesion in the body of pancreas, a 0.6 cm focus in the tail of pancreas, a 1.6 cm enhancing lesion in the liver and a 1.4 cm abdominal wall mass. Genetic testing was positive for MEN-1 and additional biochemical evaluation including VIP, gastrin and glucagon was negative. A pituitary MRI was unremarkable. She underwent an ex-lap, partial hepatectomy, distal pancreatectomy, splenectomy, and resection of the duodenal mass. The pancreas had multiple NETs, largest was 1.6 cm and stained positive for insulin. The liver mass demonstrated a metastatic well differentiated NET. The 1.7 cm duodenal mass was consistent with a leiomyoma. All surgical margins were negative, but focal lymphovascular and perineural invasion was identified with negative lymph nodes (0/27). She was diagnosed with a metastatic insulinoma with histopathology revealing a well differentiated neuroendocrine tumor G1, <1mitosis/2mm2, Ki-67 less than 3%, stage pT1N0M1. Hypoglycemia resolved post-operatively. Conclusion: Hypoglycemia predominantly in the fasting state, worsening shortly after bariatric surgery, or refractory to dietary or medical management should be further evaluated to exclude insulinoma. Given her medical history, there was concern for MEN-1, thus prompting genetic testing. Unlike with sporadic cases, there is a higher rate of recurrence with MEN-1 associated insulinoma. Highly unusual is also the finding of metastatic disease, occurring only in 4- 14% of all insulinoma cases.

scholarly journals SUN-929 An Unusual Presentation of Insulinomas-Profound Hypophosphatemia

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
William West ◽  
Gabriel Ikponmosa Uwaifo
Keyword(s):  
Neuroendocrine Tumors ◽  
Early Morning ◽  
Needle Aspiration ◽  
The Body ◽  
Serum Phosphorus ◽  
Men 1 ◽  
Dietary Phosphorus ◽  
Multiple Imaging ◽  
Well Differentiated

Abstract BACKGROUND: Insulin induces intracellular shift of phosphorus, causing hypophosphatemia. This is mild, transient and quiescent in normal persons. Persistent hyperinsulinemia in patients with depleted phosphorus stores can cause profound symptomatic hypophosphatemia. Clinical Case: A 36yr old man with MEN 1 presented in follow-up. His original manifestation of MEN 1 was familial primary hyperparathyroidism (pHPT) for which he had parathyroidectomy in 2009. At one of his endocrine clinic visits, he was noted to have asymptomatic hypophosphatemia with serum phosphorus of 2.4 (2.7–4.5 mg/dL) and mildly elevated PTH of 89 (9–77 pg/mL). This was presumed to be due to mild persistent/recurrent pHPT, and he was advised to increase dietary phosphorus intake. About a year later, he presented to the emergency room (ER) with palpitations and marked asthenia. Serum phosphorus was 1.0. He was treated with intravenous phosphorus and discharged on oral phosphorus replacement with advice to increase his dietary phosphate intake. Despite this, he continued to have recurrent spells of dizziness, weakness, palpitations, and marked hypophosphatemia several requiring repeat ER visits. His degree of mild pHPT remained stable and imaging studies to identify a possible cause for tumor induced osteomalacia with phosphate wasting was unrevealing. He volunteered at a follow up visit that he had a long history of low blood sugar spells (confirmed by fingerstick) that he had learnt to manage with frequent carbohydrate rich snacks. This prompted the consideration of a possible insulinoma as the cause for his persistent hypophosphatemia. Despite significant hypoglycemia on an abbreviated supervised fast, an OGTT and mixed meal test showed profound severe insulin mediated hypoglycemia. CGMS also showed multiple extended early morning severe hypoglycemic spells <50mg/dl over a 2 wk period. Multiple imaging tests were negative but a Gallium Dotatate scan revealed multiple areas of uptake in his pancreas. Endoscopic ultrasound of the pancreas demonstrated multiple masses in the pancreas: one 7 x 5 cm mass in the body and one 4 x 4 cm mass near the neck of the pancreas. Fine needle aspiration biopsy revealed well-differentiated neuroendocrine tumors. He had distal pancreatectomy and tumor enucleation of 5 tumors identified on intraoperative sonography. Histopathology confirmed multiple neuroendocrine tumors, with immunostaining for insulin. He has had no further episodes of hypophosphatemia since despite being off oral phosphate supplements. Conclusion: Profound hypophosphatemia can be the dominant presentation of persistent hyperinsulinemia seen in patients with insulinoma. Insulinomas and other causes of persistent hyperinsulinemia need to be considered in the differential diagnosis of persistent/recurrent hypophosphatemia.

scholarly journals An Atypical Presentation of a Patient With Postprandial Hypoglycemic Induced Seizures

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A990-A991
Author(s):  
Jeremy S Harmson ◽  
Max Sosa-Pagan
Keyword(s):  
Insulin Therapy ◽  
Insulin Level ◽  
Needle Aspiration ◽  
Serum Glucose ◽  
Intermittent Fasting ◽  
Pancreatic Islet Cell ◽  
Left Pleural Effusion ◽  
Fasting Hypoglycemia ◽  
Dextrose Infusion ◽  
Carbohydrate Meal

Abstract Background: Insulinomas are exceptionally uncommon pancreatic islet cell neuroendocrine tumors. Typically, insulinoma induced hypoglycemia occurs exclusively in the fasting state in 73 percent, reported in a retrospective analysis of 237 patients, whereas 6 percent reported only postprandial symptoms. Clinical Case: A 53-year-old female with a history of rheumatoid arthritis, obesity, and prediabetes initially admitted for new onset seizures and recurrent spontaneous hypoglycemic episodes. She experienced recurrent, symptomatic, post-prandial, hypoglycemia daily for the past 8 years. Each episode was closely associated with a high carbohydrate meal, inducing a post-prandial hypoglycemia more consistently and more profoundly than intermittent fasting. Symptoms of lightheadedness, shakiness, and seizure were exacerbated by each carbohydrate meal. Initial labs revealed serum glucose of 35 mg/dl. After recovery with dextrose infusion, a brief fasting trial less than 24 hours was performed with no recurrence of hypoglycemia. However, a mixed meal study utilizing watermelon resulted in a postprandial serum hypoglycemia of 28 mg/dl, provoking a seizure within 30 minutes. During her hospitalization, recurrent hypoglycemia was found during the postprandial period rather than intermittent fasting periods requiring dextrose infusion and octreotide. A Hypoglycemia panel sent during the initial episode was consistent with endogenous hyperinsulinism (Serum glucose=35, Insulin level=24, Proinsulin=166, C-peptide=0.9, BHB=undetectable, Sulfonurea=negative). A subsequent CT of the abdomen/pelvis revealed a mass associated with the pancreatic tail, measuring 4.1 x 4.4 x 5.2 cm concerning for pancreatic malignancy. A fine needle aspiration followed by a distal pancreatectomy and splenectomy with histopathological and immuno-histochemical evaluation confirming a well differentiated (grade 1), 5.0 x 4.5 x 3.0 cm, neuroendocrine tumor (insulinoma). Her recovery, post-operatively, was complicated by an intraabdominal abscess, left pleural effusion, necessitating insulin therapy for hyperglycemia believed to be secondary to morbid obesity, weight gain, and insulin resistance. Hypoglycemic symptoms resolved, blood glucose normalized, and insulin therapy was weaned over the following 3 months and she remains on metformin to date with no evidence of recurrence. Conclusion: Although rare, an insulinoma should be considered in the differential diagnosis of any individual with recurrent episodes of frequent symptomatic hypoglycemia. The classical clinical manifestation of an insulinoma is a fasting hypoglycemia, with distinct episodes of autonomic symptoms. However, postprandial symptoms have been reported with increasing frequency. Here we present a case of surgically confirmed insulinoma with predominantly post-prandial hypoglycemia.

Immunotherapy and potential predictive biomarkers in the treatment of neuroendocrine neoplasia

2020 ◽  
Author(s):  
Guanghui Xu ◽  
Yuhao Wang ◽  
Hushan Zhang ◽  
Xueke She ◽  
Jianjun Yang
Keyword(s):  
Current Knowledge ◽  
Checkpoint Inhibitors ◽  
Treatment Options ◽  
Predictive Biomarkers ◽  
The Body ◽  
Low Grade ◽  
Ablative Therapies ◽  
Cancer Types ◽  
New Treatment ◽  
Well Differentiated

Neuroendocrine neoplasias (NENs) are a heterogeneous group of rare tumors scattered throughout the body. Surgery, locoregional or ablative therapies as well as maintenance treatments are applied in well-differentiated, low-grade NENs, whereas cytotoxic chemotherapy is usually applied in high-grade neuroendocrine carcinomas. However, treatment options for patients with advanced or metastatic NENs are limited. Immunotherapy has provided new treatment approaches for many cancer types, including neuroendocrine tumors, but predictive biomarkers of immune checkpoint inhibitors (ICIs) in the treatment of NENs have not been fully reported. By reviewing the literature and international congress abstracts, we summarize the current knowledge of ICIs, potential predicative biomarkers in the treatment of NENs, implications and efficacy of ICIs as well as biomarkers for NENs of gastroenteropancreatic system, lung NENs and Merkel cell carcinoma in clinical practice.

scholarly journals Molecular Pathology of Well-Differentiated Pulmonary and Thymic Neuroendocrine Tumors: What Do Pathologists Need to Know?

2021 ◽  
Vol 32 (1) ◽  
pp. 154-168 ◽  
Author(s):  
Marco Volante ◽  
Ozgur Mete ◽  
Giuseppe Pelosi ◽  
Anja C. Roden ◽  
Ernst Jan M. Speel ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Young Patients ◽  
Carcinoid Tumors ◽  
Neuroendocrine Neoplasms ◽  
Grey Zone ◽  
Cell Hyperplasia ◽  
Ki 67 ◽  
Familial Form ◽  
Well Differentiated ◽  
Neuroendocrine Carcinomas

AbstractThoracic (pulmonary and thymic) neuroendocrine tumors are well-differentiated epithelial neuroendocrine neoplasms that are classified into typical and atypical carcinoid tumors based on mitotic index cut offs and presence or absence of necrosis. This classification scheme is of great prognostic value but designed for surgical specimens, only. Deep molecular characterization of thoracic neuroendocrine tumors highlighted their difference with neuroendocrine carcinomas. Neuroendocrine tumors of the lung are characterized by a low mutational burden, and a high prevalence of mutations in chromatin remodeling and histone modification-related genes, whereas mutations in genes frequently altered in neuroendocrine carcinomas are rare. Molecular profiling divided thymic neuroendocrine tumors into three clusters with distinct clinical outcomes and characterized by a different average of copy number instability. Moreover, integrated histopathological, molecular and clinical evidence supports the existence of a grey zone category between neuroendocrine tumors (carcinoid tumors) and neuroendocrine carcinomas. Indeed, cases with well differentiated morphology but mitotic/Ki-67 indexes close to neuroendocrine carcinomas have been increasingly recognized. These are characterized by specific molecular profiles and have an aggressive clinical behavior. Finally, thoracic neuroendocrine tumors may arise in the background of genetic susceptibility, being MEN1 syndrome the well-defined familial form. However, pathologists should be aware of rarer germline variants that are associated with the concurrence of neuroendocrine tumors of the lung or their precursors (such as DIPNECH) with other neoplasms, including but not limited to breast carcinomas. Therefore, genetic counseling for all young patients with thoracic neuroendocrine neoplasia and/or any patient with pathological evidence of neuroendocrine cell hyperplasia-to-neoplasia progression sequence or multifocal disease should be considered.

scholarly journals Efficacy of Caffeic Acid on Diabetes and Its Complications in the Mouse

Molecules ◽  
2021 ◽  
Vol 26 (11) ◽  
pp. 3262
Author(s):  
Nada Oršolić ◽  
Damir Sirovina ◽  
Dyana Odeh ◽  
Goran Gajski ◽  
Vedran Balta ◽  
...  
Keyword(s):  
Lipid Profile ◽  
Caffeic Acid ◽  
Fasting Blood Glucose ◽  
Kidney Tissue ◽  
Serum Glucose ◽  
The Body ◽  
Diabetic Mice ◽  
Atherogenic Indices ◽  
Liver And Kidney ◽  
Hematological And Biochemical Parameters

Diabetic dyslipidemia and hyperglycemia contribute to excessive reactive oxygen species (ROS) production, leading to deleterious complications, such as nephropathy, atherosclerosis and cardiac dysfunction, and target major organs in the body. The aim of this study was to investigate the effect of caffeic acid (CA) on mouse weight and survival, serum level of fasting blood glucose (FBG), serum lipid parameters and atherogenic indices, oxidative damage in blood, liver and kidney tissue, pathophysiological changes and their function markers in healthy and alloxan-induced type 1 diabetic mice. Diabetes was induced in mice with a single intravenous injection of alloxan (75 mg kg−1). Two days later, CA (50 mg kg−1) was given intraperitoneally for seven days in diabetic mice. Diabetes affected glucose level, lipid profile, hematological and biochemical parameters, induced DNA damage and apoptotic/necrotic death in whole blood cells, liver and kidney, leading to weight loss and a decreased lifespan. CA treatment of diabetic mice revealed a protective effect on the liver and kidney, hypoglycemic and hypolipidemic properties and high protection against atherogenic outcomes. The obtained results suggest that CA is a safe and potent agent against diabetes that acts as an effective antioxidant in reducing serum glucose, lipid profile and atherogenic indices, leading to increased lifespan in mice.

Prognostic Signicance of Cellular Iron Metabolism in Breast Cancer

2018 ◽  
Vol 1 (1) ◽  
Author(s):  
Rizwan Ullah Khan ◽  
Amber Hassan ◽  
Imrana Tanvir ◽  
Kashifa Ehsan
Keyword(s):  
Breast Cancer ◽  
Breast Carcinoma ◽  
Ki 67 ◽  
Luminal B ◽  
Cellular Iron ◽  
Menopausal Women ◽  
New Strategy ◽  
Poorly Differentiated ◽  
Well Differentiated ◽  
Post Menopausal

Breast carcinoma is among the most common malignancy in women. Abstract:Original ArticleAim of the present study was to evaluate the prognostic signicance of iron expression in the biopsies of patients with breast cancer Objective:24 breast biopsies were studied. 19 cases were poorly differentiated, 5 cases were moderately differentiated and there was no well differentiated case. Iron, Estrogen receptor (ER), Progesterone receptor (PR), HER2 and Ki-67 immunohistochemical staining was performed for all these cases. Methods: Among the 5 moderately differentiated cases, 3 (60%) were positive for iron staining and among 19 poorly differentiated cases, 11 cases (57.89%) were positive. More iron positive cases (7 out of 14) were triple positive belonging to Luminal B class. Out of 14 iron positive cases, 11 were positive for HER2, 10 for ER, 9 for PR and all positive for Ki-67. Results: Iron deciency in premenopausal and overload in post-menopausal women can contribute to the development of breast carcinoma. So, iron can be considered as a cheap and effective marker for the prognosis of breast cancer. Association between a rise in iron levels and HER2 expression may provide new strategy for breast cancer treatment.

scholarly journals Poorly Differentiated Small-Cell-Type Neuroendocrine Carcinoma of the Prostate: A Case Report and Literature Review

2018 ◽  
Vol 11 (3) ◽  
pp. 676-681 ◽  
Author(s):  
Kishore Kumar ◽  
Rafeeq Ahmed ◽  
Chime Chukwunonso ◽  
Hassan Tariq ◽  
Masooma Niazi ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Tumor Grade ◽  
Small Cell ◽  
The Body ◽  
Neuroendocrine Cells ◽  
Cell Type ◽  
Variable Response ◽  
Ki 67 ◽  
Platinum Based Chemotherapy ◽  
Poorly Differentiated

Neuroendocrine cells are widespread throughout the body and can give rise of neuroendocrine tumors due to abnormal growth of the chromaffin cells. Neuroendocrine tumors divide into many subtypes based on tumor grade (Ki-67 index and mitotic count) and differentiation. These tumors can be further divided into secretory and nonsecretory types based on the production of peptide hormone by tumor cells. Poorly differentiated small-cell-type neuroendocrine tumors are one of the subtypes of neuroendocrine tumors. These tumors are less common; however, they tend to be locally invasive and aggressive in behavior with poor overall median survival. Treatment of the nonsecretory small-cell type is modeled to small-cell lung cancer with a regimen consisting of platinum-based chemotherapy and etoposide with variable response. Here, we present a case of poorly differentiated small-cell neuroendocrine tumor originating from the prostate.

Sign in / Sign up

Export Citation Format

Share Document