0
selected
-
1.
Case Report: Unmasking Hypercalcemia in Patients With Neuroendocrine Neoplasms. Experience From Six Italian Referral Centers.
Giannetta, E, Sesti, F, Modica, R, Grossrubatscher, EM, Guarnotta, V, Ragni, A, Zanata, I, Colao, A, Faggiano, A
Frontiers in endocrinology. 2021;:665698
Abstract
BACKGROUND Hypercalcemia is a common paraneoplastic syndrome which can occur in up to 10% of patients with advanced neoplasms. Paraneoplastic parathyroid hormone-related protein (PTHrP) represents the most frequent cause of this syndrome. In neuroendocrine neoplasms (NENs) paraneoplastic hypercalcemia is rare. CASE SERIES The present series includes all patients with NENs and paraneoplastic hypercalcemia from four Italian centres: (I) A 40-year-old man was hospitalized for repeated episodes of falls, hyposthenia and drowsiness. Severe hypercalcemia was found. Metastatic pancreatic G2 NEN and PTHrP-related hypercalcemia were diagnosed. The patient started therapy with somatostatin analogs (SSA) and Denosumab. After disease progression peptide receptor radionuclide therapy (PRRT) was started with an objective response associated with PTHrP reduction and normocalcemia. (II) A 45-year-old man was referred for pancreatic G2 NEN. SSA and subsequently everolimus were administered for metastases occurrence. Hypercalcemia occurred and PRRT and Denosumab were started for disease progression with the onset of bone metastases. Despite disease stability after four cycles of PRRT the patient's performance status worsened until death. (III) A 49-year-old woman was hospitalized for psychic slowdown, confusional state, sensory dullness. A severe hypercalcemia, associated with a pancreatic G1 NEN was diagnosed and treated with haemodialysis, bisphosphonates injections and continuous infusion of calcitonin. 1,25-dihydroxyvitamin D was high, PTHrP was undetectable. After surgery serum calcium levels and 1,25-dihydroxyvitamin D were normalized. (IV) A 69-year-old man was hospitalized after the onset of shortness of breath and dyspnea, asthenia and weight loss. Computed Tomography (CT) and 68Ga DOTATOC Positron Emission Tomography (PET)-CT revealed a left pulmonary nodule. Hypercalcemia and markedly elevated PTHrP levels were detected. The histological examination revealed an atypical carcinoid. After surgery, calcium levels were normalized, PTHrP was significantly reduced with an improvement of general conditions. CONCLUSION In our series, paraneoplastic PTHrP-related hypercalcemia occurred in pancreatic NEN and in one bronchial carcinoid representing the third case in the literature. Our case associated with 1,25-dihydroxyvitamin D secretion represents the fourth case in the literature. PTHrP secretion should be considered in NENs' patients with hypercalcemia. Acute treatment should be focused on lowering calcium levels, and long-term control can be achieved by tumor cytoreduction inhibiting PTHrP release.
-
2.
Upfront molecular profiling of pancreatic cancer patients - An idea whose time has come.
Bhutani, MS, Cazacu, IM, Roy-Chowdhuri, S, Maitra, A, Pishvaian, MJ
Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]. 2020;(3):391-393
Abstract
Pancreatic cancer patients have not benefited from survival improvements brought about by personalized medicine in other malignancies. Even though this is the era of precision medicine, unfortunately most clinicians in practice either do not embrace or are unaware of the concept of molecular profiling for pancreatic cancer patients. Improving the grim prognosis of this challenging disease requires a paradigm shift. To exploit all potential therapeutic options in this lethal disease, molecular profiling should be performed ideally at the moment of diagnosis to identify potentially trial-eligible tumoral mutations or support off label use of an agent approved for another indication. This perspective article aims to increase awareness of the importance of upfront molecular profiling for pancreatic cancer patients.
-
3.
Perivascular Epithelioid Cell Tumor (PEComa) of Pancreas Diagnosed Preoperatively by Endoscopic Ultrasound-Guided Fine-Needle Aspiration: A Case Report and Review of Literature.
Collins, K, Buckley, T, Anderson, K, Karasik, M, Ligato, S
Diagnostic cytopathology. 2017;(1):59-65
Abstract
Perivascular epithelioid cell tumors (PEComas) of the pancreas are extremely rare mesenchymal tumors and to our knowledge, only 17 cases have been reported in the English literature to date. We report our experience with a new case of primary pancreatic PEComa diagnosed preoperatively by endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) complemented by tissue cell block and immunohistochemistry. The patient was a 54-year-old female whose chief complaint was intermittent severe right upper quadrant abdominal pain. Computed-tomography (CT) imaging revealed a mass between the head and the body of the pancreas. EUS-FNA smear preparation was obtained but was nondiagnostic. However, examination of the tissue cell block showed sheets of epithelioid cells with abundant eosinophilic cytoplasm and immunohistochemistry studies revealed positivity for both melanocytic (HMB-45 and Melan-A) and smooth muscle markers (actin and desmin). A diagnosis of PEComa was made and an uncomplicated middle pancreatectomy was performed. Our case and review of the literature demonstrates that EUS-FNA complemented with tissue cell block increases cellular yield, improved preoperative diagnostic accuracy, and may assist the surgeon in planning conservative surgical management. Diagn. Cytopathol. 2017;45:59-65. © 2016 Wiley Periodicals, Inc.
-
4.
Non-insulinoma Pancreatogeneous Hypoglycemia Syndrome with False-Positive Somatostatin Receptor Scintigraphy: A Case Report and Review of Literature.
Preechasuk, L, Pongpaibul, A, Kunavisarut, T
Journal of the Medical Association of Thailand = Chotmaihet thangphaet. 2016;(3):354-9
Abstract
Non-insulinoma pancreatogeneous hypoglycemia syndrome (NIPHS) is a rare cause of hypoglycemia in adults. The cause of NIPHS is diffuse hyperinsulinism. As a result, computed tomography (CT) of pancreas, endoscopic pancreatic ultrasonography (EUS), and somatostatin receptor scintigraphy (SSRS), which are usually performed to locate an insulinoma, are not able to diagnose NIPHS. Moreover, SSRS can give a false-positive result. In this case report, we introduce a 22-year-old Thai woman who presented with fasting and postprandial hyperinsulinemic hypoglycemia. Accordingly, an insulinoma was suspected. She underwent several studies to locate the lesion. Pancreatic CT and EUS failed to locate a lesion; however, SSRS showed a faint focus of increased uptake at the pancreatic head. The suspected insulinoma was not identified during a first operation. Thereafter other diagnostic methods were performed in an effort to locate the suspected insulinoma, including selective arterial calcium stimulation test. The result of the selective arterial calcium stimulation test was negative. Intraoperative ultrasonography during a second operation also failed to locate a tumor. Finally, a pancreatic head resection was performed according to SSRS result, yet capillary blood glucose levels did not increase after resection. In response, a 95% pancreatectomy was performed. The pathology report was consistent with diffuse hyperinsulinism. This report emphasizes that SSRS can give false positive result in NIPHS.
-
5.
Rare case of pancreatic cancer with leptomeningeal carcinomatosis.
Yoo, IK, Lee, HS, Kim, CD, Chun, HJ, Jeen, YT, Keum, B, Kim, ES, Choi, HS, Lee, JM, Kim, SH, et al
World journal of gastroenterology. 2015;(3):1020-3
Abstract
Leptomeningeal carcinomatosis occurs very rarely in patients with pancreatic cancer. Leptomeningeal carcinomatosis is characterized by multifocal seeding of the leptomeninges by malignant cells that originate from a solid tumor. To the best of our knowledge, brain metastasis from pancreatic cancer is extremely rare. Leptomeningeal carcinomatosis is estimated to occur in 3% to 8% of cases of solid tumors. The clinical manifestation usually involves neurological symptoms, including dizziness, headache, vomiting, nausea, and hemiparesis, symptoms similar to those of meningitis or brain tumors. Diagnostic methods for leptomeningeal carcinomatosis include brain magnetic resonance imaging and cerebrospinal fluid examination. Here, we describe a case of leptomeningeal carcinomatosis in which the primary tumor was later determined to be pancreatic cancer. Brain magnetic resonance imaging findings showed mild enhancement of the leptomeninges, and cerebrospinal fluid cytology was negative at first. However, after repeated spinal taps, atypical cells were observed on cerebrospinal fluid analysis and levels of tumor markers such as carbohydrate antigen 19-9 in cerebrospinal fluid were elevated. Abdominal computed tomography, performed to determine the presence of extracerebral tumors, revealed pancreatic cancer. Pancreatic cancer was confirmed histopathologically on examination of an endoscopic ultrasound-guided fine needle aspiration specimen.
-
6.
[Guidelines for contrast enhanced ultrasound (CEUS)--update 2008].
Correas, JM, Tranquart, F, Claudon, M
Journal de radiologie. 2009;(1 Pt 2):123-38; quiz 139-40
Abstract
These new recommendations are following the text published in 2004 by the group of experts of the EFSUMB that was augmented to reevaluate the indications of ultrasound contrast agents. Initially limited to focal liver lesions, new recommendations include the study of renal and pancreatic diseases, as well as vesico-ureteric reflux, blunt abdominal trauma and trans-cranial Doppler. Contrast-enhanced ultrasound improves the diagnosis of renal pseudo-tumors but does not allow characterization of solid focal masses. Its major contribution includes characterization of complex cystic masses, diagnosis of peripheral vascular disorders such as infarction and cortical necrosis, and radiofrequency ablation follow-up. It is also useful for the study of pancreatic masses visible at ultrasound in order to improve lesion visualization and characterization (adenocarcinoma and neuro-endocrine tumors), as well as for vascular staging.
-
7.
[Accessory spleen as a differential diagnosis of intrapancreatic tumors. Case report and review of the literature].
Domínguez, I, Franssen-Canovas, B, Uribe-Uribe, N, Franco, R, Campuzano, M, Uscanga, LF
Revista de gastroenterologia de Mexico. 2007;(4):376-8
Abstract
There are few reports of intrapancreatic accessory spleen. Most cases have been reported in Japan and some refer epithelial cysts within it. Most of these lesions are asymptomatic and may be misdiagnosed as a non-functioning neuroendocrine tumor due to their radiological characteristics and lack of symptomatology. We report a case of a 46 year old woman with an incidentally diagnosed intrapancreatic accessory spleen. Because of a history of weight loss and a severe acanthosis nigricans a hidden neoplasia was sought. A 2.5 x 1 cm lesion was found in the tail of the pancreas on computed tomography scan and later confirmed with endoscopic ultrasound. A non-diagnostic biopsy was undertaken. A distal pancreatectomy with splenectomy was performed and a final pathological diagnosis of intrapancreatic accessory spleen was given. The 99mTc-denatured RBC scan in addition to the octreotide scintigraphy have been proposed to differentiate it from other intrapancreatic tumors and avoid unnecessary surgery. Intrapancreatic accessory spleen should be considered as a differential diagnosis for pancreatic tumors.
-
8.
Modified hyperinsulinaemic, eu- and hypoglycaemic clamp technique using lispro-insulin for insulinoma diagnostic.
Roudovitch, N, Nauck, MA, Schatz, H, Pfeiffer, AF
Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association. 2001;(8):397-401
Abstract
Characterization of metabolically inadequate insulin secretion is essential for insulinoma diagnostics. Hyperinsulinaemic, eu- and hypoglycaemic clamp procedures have been used to suppress endogenous insulin secretion in healthy subjects. The use of exogenous insulin precluded the use of insulin as a parameter to be measured. We now suggest to use exogenous insulin lispro and an insulin-specific ELISA not cross reacting with insulin lispro. Thus, determination of insulin by ELISA in this experimental setting reflects endogenous insulin. A 39-year-old man with a surgically confirmed pancreatic insulinoma was studied under hyperinsulinaemic [lispro insulin 40 mU x m(-2) body surface x min(-1)] clamp conditions. Euglycaemia was achieved (3.8 +/- 0.5 mmol/L) for 1 h and hypoglycaemia (2.36 +/- 0.49 mmol/L) was achieved for another 30 min. Insulin was evaluated by ELISA (cross-reaction with lispro insulin < 0.006%, C-peptide < 0.01%, proinsulin < 0.001%) and by a nonselective RIA (cross-reaction with proinsulin 40%). In control subjects the euglycaemic hyperinsulinaemia suppressed C-peptide to 0.36 +/- 0.03 ng/ml and hypoglycaemic hyperinsulinaemia to 0.29 +/- 0.03 ng/ml. Endogenous insulin was suppressed to 2.8 +/- 0.03 mU/L under euglycaemia and to 2.6 +/- 0.03 mU/L under hypoglycaemia in control subjects. In the insulinoma patient apparently irregular but small changes in both C-peptide (1.43 +/- 0.1 ng/ml) and more pronounced changes in endogenous insulin concentrations 4.41 +/- 0.1 mU/l under euglycaemia and 5.35 +/- 0.3 mU/l under hypoglycaemic conditions, were observed. The basal level of insulin (ELISA insulin 4.6 mU/L) and C-peptide (1.7 ng/ml) were not markedly elevated. Determination of insulin allowed better characterization of irregular pulses because of the shorter half-life of insulin relative to C-peptide. The new modification of sequential eu- and hypoglycaemic clamp procedures should also be useful in pharmacological studies of insulinotropic substances. Direct measurement of peripheral insulin may be more sensitive than C-peptide to detect low levels of autonomous insulin secretion in small insulinomas.