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Neuroendocrine tumors

Kathrin Fielitz, Kristina Althoff, Katleen De Preter, Julie Nonnekens, Jasmin Ohli, Sandra Elges, Wolfgang Hartmann, Günter Klöppel, Thomas Knösel, Marc Schulte, Ludger Klein-Hitpass, Daniela Beisser, Henning Reis, Annette Eyking, Elke Cario, Johannes H Schulte, Alexander Schramm, Ulrich Schüller
Amplification or overexpression of MYCN is involved in development and maintenance of multiple malignancies. A subset of these tumors originates from neural precursors, including the most aggressive forms of the childhood tumors, neuroblastoma and medulloblastoma. In order to model the spectrum of MYCN-driven neoplasms in mice, we transgenically overexpressed MYCN under the control of the human GFAP-promoter that, among other targets, drives expression in neural progenitor cells. However, LSL-MYCN;hGFAP-Cre double transgenic mice did neither develop neural crest tumors nor tumors of the central nervous system, but presented with neuroendocrine tumors of the pancreas and, less frequently, the pituitary gland...
October 19, 2016: Oncotarget
Ersin Özaslan, Halit Karaca, Sinan Koca, Alper Sevinç, Bekir Hacioğlu, Metin Özkan, Melike Özçelik, Ayşe O Duran, İlhan Hacibekiroğlu, Yaşar Yildiz, Özgür Tanriverdi, Serkan Menekşe, Asude Aksoy, Oktay Bozkurt, Semiha Urvay, Mükremin Uysal, Hacer Demir, Aydin Çiltaş, Faysal Dane
The objectives of this study were to compare progression-free survival (PFS) with somatostatin analog (SSA) versus chemotherapy (CTx) in first-line therapy and to determine the patient group in which these treatments were more effective in neuroendocrine tumors (NETs) with a Ki-67 index of 20% or less. Patients who received SSA or CTx and had unresectable locally advanced and metastatic NETs with a Ki-67 index of 20% or less were retrospectively selected from 13 centers in the Turkish database between 2000 and 2015...
October 20, 2016: Anti-cancer Drugs
Sundhar Ramalingam, Adva Eisenberg, Wen Chi Foo, Jennifer Freedman, Andrew J Armstrong, Larry G Moss, Michael R Harrison
Here we present, to the best of our knowledge, the first case of a paraneoplastic Cushing's syndrome (hypercortisolism) resulting from treatment-related neuroendocrine prostate cancer - a highly aggressive and difficult disease to treat. A 51-year-old man was started on androgen deprivation therapy after presenting with metastatic prostate cancer, characterized by diffuse osseous metastasis. Shortly thereafter, he developed progressive disease with biopsy proven neuroendocrine prostate cancer as well as symptoms of increased skin pigmentation, hypokalemia, hypertension, hyperglycemia and profound weakness, consistent with ectopic Cushing's syndrome...
October 21, 2016: International Journal of Urology: Official Journal of the Japanese Urological Association
Sirong Chen, Shing Kee Cheung, Ka-Nin Wong, Kwok Kee Wong, Chi-Lai Ho
A bedridden 90-year-old man with fever and elevated prostate-specific antigen (PSA) (49 ng/mL) was referred for differentiation between infection and tumor. F-FDG PET/CT was negative for infection, but Ga-PSMA PET/CT showed multiple lesions in prostate gland with infiltration to bladder wall and seminal vesicle, consistent with locally advanced prostate cancer. The lesion with the highest Ga-PSMA uptake was strongly avid for Ga-DOTATOC, suggesting neuroendocrine tumor differentiation. After hormonal therapy, PSA normalized, but chromogranin-A increased (from 251 to 398 ng/mL), inferring progression of neuroendocrine tumor differentiation...
October 18, 2016: Clinical Nuclear Medicine
Yalan Bi, Yinghui Deng, Shanqing Li, Xiaoyun Zhou, Yeye Chen, Dongjie Ma, Xinxin Mao, Yuzhou Guan, Jie Chen, Yunxiao Meng
OBJECTIVES: To investigate the immunoreactivity of TTF-1 and PAX8 in neuroendocrine carcinoma of thymic (TNEC) and pulmonary origins (PNEC), and whether their immunophenotyping could be used to distinguish between NEC of the two sites, as well as prognosis of patients with TNEC. METHODS: Twenty-two cases of TNEC and 20 cases of PNEC were selected for immunohistochemical analysis using PAX8 and TTF-1. Clinical data and follow-up information were obtained for survival analyses...
October 20, 2016: Journal of Surgical Oncology
Elisabeth Sterrer, Franz Windisch, Kurt Frey, Klaus Rettensteiner, Benjamin Loader
BACKGROUND: Middle ear adenoma with neuroendocrine differentiation (MEA-ND), also known as carcinoid tumor of the middle ear, is an extremely rare neoplasm, especially when located within the tympanic cavity. To the best of our knowledge, this represents the first pediatric case of a MEA-ND described in Europe. Excluding the present case, only three other pediatric cases of this disease have been published globally. CASE REPORT: We report on a 15-year-old female patient who presented with conductive hearing loss of the right ear...
October 19, 2016: Wiener Klinische Wochenschrift
Fabiana Policeni, Brittany Pakalniskis, Limin Yang
Metastatic tumors are rare in the breast. Well-differentiated neuroendocrine tumors (WDNETs) are slow-growing neoplasms that arise from neuroendocrine cells, particularly in the gastrointestinal tract and bronchial tree. Metastatic WDNET to the breast is a rare entity. We present a case report of ileal WDNET metastatic to the breast which was initially identified as a small mass in the patient's left breast on screening mammography. Targeted ultrasound identified a suspicious mass, and ultrasound-guided percutaneous core biopsy was performed...
2016: Journal of Clinical Imaging Science
Silje Hovden, Marie Louise Jespersen, Peter H Nissen, Per Løgstrup Poulsen, Lars Rolighed, Søren A Ladefoged, Lars Rejnmark
Familial hypocalciuric hypercalcemia type 3 should be considered as differential diagnosis in patients with suspected primary hyperparathyroidism and/or suspected multiple neoplasia syndrome, as correct diagnosis will spare the patients for going through multiple futile parathyroidectomies and for the worry of being diagnosed with a cancer susceptibility syndrome.
October 2016: Clinical Case Reports
Tatsuya Umemoto, Takeshi Nomoto, Satoshi Kuroda, Takahiro Ogawa, Kentaro Nagao, Yuki Shimizu, Nobuyuki Nakajima, Hakushi Kim, Masahiro Nitta, Kazuya Hanai, Akio Hoshi, Toshiro Terachi
A 40-year-old woman was referred to our hospital with right lower back pain as the chief complaint. Contrast-enhanced computed tomography (CT) showed a partially-solid tumor within a cyst measuring approximately 6 cm in diameter in the right renal hilum. The solid part was enhanced in the early phase and contrast medium was washed out earlier in the solid part than in the parenchyma in the equilibrium phase. Plain CT revealed partial cyst wall calcification. A soft tissue shadow approximately 10 mm in diameter in the dorsal inferior vena cava at the upper pole of the kidney and a solid tumor adjacent to the iliopsoas muscle and the kidney were detected...
September 2016: Hinyokika Kiyo. Acta Urologica Japonica
Nitya Raj, Emily Valentino, Marinela Capanu, Laura H Tang, Olca Basturk, Brian R Untch, Peter J Allen, David S Klimstra, Diane Reidy-Lagunes
OBJECTIVES: Emerging data suggest that not all grade 3 (G3) pancreatic neuroendocrine neoplasms (panNENs) behave the same; tumor differentiation may predict outcome. METHODS: Patients with G3 panNENs treated at our institution between 1999 and 2014 were identified. Demographics, response to therapy, and overall survival were determined. RESULTS: Forty-five patients were identified, 16 with G3 well differentiated pancreatic neuroendocrine tumors (WD-panNETs) and 29 with poorly differentiated neuroendocrine carcinomas (PDNEC)...
October 18, 2016: Pancreas
Nancy Sharma, Boris G Naraev, Eric G Engelman, M Bridget Zimmerman, David L Bushnell, Thomas M OʼDorisio, M Sue OʼDorisio, Yusuf Menda, Jan Müller-Brand, James R Howe, Thorvardur R Halfdanarson
OBJECTIVES: The objective of this study was to describe the outcomes of patients in the University of Iowa Neuroendocrine Tumor (NET) Database treated with peptide receptor radionuclide therapy (PRRT). METHODS: One hundred thirty-five patients from the University of Iowa NET Database who received PRRT were analyzed, their characteristics were described, and survival was calculated. RESULTS: The median age at diagnosis was 51 years, and 64% were men...
October 18, 2016: Pancreas
Parnian Ahmadi Moghaddam, Kristine M Cornejo, Lloyd Hutchinson, Keith Tomaszewicz, Karen Dresser, April Deng, Patrick OʼDonnell
Merkel cell carcinoma (MCC) is a rare primary cutaneous neuroendocrine tumor that typically occurs on the head and neck of the elderly and follows an aggressive clinical course. Merkel cell polyomavirus (MCPyV) has been identified in up to 80% of cases and has been shown to participate in MCC tumorigenesis. Complete spontaneous regression of MCC has been rarely reported in the literature. We describe a case of a 79-year-old man that presented with a rapidly growing, 3-cm mass on the left jaw. An incisional biopsy revealed MCC...
October 12, 2016: American Journal of Dermatopathology
R M Suresh, H C Lokesh, B S Harsha, Sri S Chandana
Pheochromocytoma, a neuroendocrine tumor of the adrenal medulla, arising from chromaffin cells, usually presents with the clinical triad of paroxysmal headache, profuse sweating and palpitations, associated with labile hypertension. Here, we present the case of an adult male with an unusual manifestation of pheochromocytoma in the form of acute pain abdomen with nausea and abdominal guarding, mimicking acute peritonitis. He had fluctuating blood pressure recordings. On subsequent investigation, a mass lesion in the left suprarenal area on an abdominal CT scan and a 24-hour urinary metanephrine assay confirmed the diagnosis of pheochromocytoma...
July 2016: Journal of the Association of Physicians of India
Michèle d'Herbomez, Lucie Coppin, Catherine Bauters, Nathalie Rouaix-Emery, Bruno Carnaille, Christine Do Cao
Neuroendocrine tumors (NET) are rare heterogenous tumors which prevalence is increasing. Their features vary by anatomical location, functionality and hormonal production. Their management needs a multidisciplinary approach. Functional tumors develop characteristic clinical syndromes in contrast to non-functional tumors that are diagnosed fortuitously or at advanced stage. NET can secrete many specific and general biomarkers. CgA is the most sensitive general marker. Its value should be interpreted along with the renal function and the gastrin level...
October 19, 2016: Annales de Biologie Clinique
Yugang Cheng, Hanxiang Zhan, Lei Wang, Jianwei Xu, Guangyong Zhang, Zongli Zhang, Sanyuan Hu
The incidence rate of pancreatic neuroendocrine neoplasms (pNENs) has increased rapidly in recent years. However, the clinicopathological characteristics of pNENs are poorly understood. Medical records of patients who underwent surgery and were confirmed as pNENs by pathological examination from January 2003 to February 2015 in Qilu Hospital were reviewed retrospectively. A total of 100 patients, 36 males and 64 females, were included with a mean operation age of 46.26 + 13.41 years. Among the 100 cases, 76 had insulinomas and 24 had non-functional pNENs...
October 17, 2016: Frontiers of Medicine
Fernando Pereira Pérez
Neuroendocrine tumors (NETs) are a heterogeneous group of neoplasms characterized by a common embryonic origin and highly variable clinical outcome.
October 19, 2016: Revista Española de Enfermedades Digestivas
Hiroki Sugita, Hideyuki Kuroki, Takahiko Akiyama, Nobuya Daitoku, Rumi Tashima, Hiroshi Tanaka, Shinobu Honda, Masahiko Hirota
INTRODUCTION: In a distal pancreatectomy combined with a distal gastrectomy, the splenic artery and vein must be conserved. However, it is not easy in pure laparoscopic surgery. We performed a hand-assisted laparoscopic spleen-preserving distal pancreatectomy (HALS-SPDP) combined with a laparoscopic distal gastrectomy (LDG) for the treatment of a pancreatic neuroendocrine tumor (NET) with early gastric cancer. PRESENTATION OF CASE: A 67-year-old male was hospitalized with no complaint...
October 8, 2016: International Journal of Surgery Case Reports
Marina Parra-Robert, Aida Orois, Josep Maria Augé, Irene Halperin, Xavier Filella, Rafael Molina
BACKGROUND: Medullary thyroid carcinoma (MTC) is a neuroendocrine tumor caused by a malignant transformation in the parafollicular C-cells of the thyroid, where calcitonin (CT) is released. Nowadays the main tumor markers (TM) used in the diagnosis and follow-up of MTC patients are CT and carcinoembryonic antigen (CEA). Nonetheless, progastrin releasing peptide (proGRP) has been recently proposed as a TM useful in the MTC. Our aims were to investigate the release of proGRP in thyroid tumors, its role in the assessment of advanced MTC and its utility in the differential diagnosis between MTC and non-MTC thyroid tumors...
October 18, 2016: Clinical Chemistry and Laboratory Medicine: CCLM
Maud-Emmanuelle Gilles, Federica Maione, Mélissande Cossutta, Gilles Carpentier, Laure Caruana, Sylvia Di Maria, Claire Houppe, Damien Destouches, Ksenya Shchors, Christopher Prochasson, Fabien Mongelard, Simona Lamba, Alberto Bardelli, Philippe Bouvet, Anne Couvelard, José Courty, Enrico Giraudo, Ilaria Cascone
Pancreatic cancer is a highly aggressive tumor, mostly resistant to the standard treatments. Nucleolin (NCL) is overexpressed in cancers and its inhibition impairs tumor growth. Herein we showed that NCL was overexpressed in human specimens of pancreatic ductal adenocarcinoma (PDAC) and that the overall survival significantly increased in patients with low levels of NCL. The NCL antagonist N6L strongly impaired the growth of primary tumors and liver metastasis in an orthotopic mouse model of PDAC (mPDAC). Similar anti-tumor effect of N6L has been observed in a highly angiogenic mouse model of pancreatic neuroendocrine tumor RIP-Tag2...
October 17, 2016: Cancer Research
Maria G Kuba, Allison Wasserman, Cindy L Vnencak-Jones, Julia A Bridge, Lan Gellert, Omar Hameed, Giovanna A Giannico
OBJECTIVES: Primary carcinoid tumor of the renal pelvis is a rare neoplasm with few cases reported in the literature. Here we present the clinical and histopathologic findings of a primary carcinoid tumor arising in the left renal pelvis of a horseshoe kidney in a 61-year-old female patient. MATERIALS AND METHODS: Pathologic features were evaluated with standard hematoxylin and eosin sections and immunohistochemical studies. A literature review was performed to place our case in context to previous reports...
October 7, 2016: Applied Immunohistochemistry & Molecular Morphology: AIMM
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