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Neuroendocrine carcinoma

Zhiyan Fu, Chunlai Zuo, Christine E Sheehan, Deepa T Patil, Jingmei Lin, Zhaohai Yang, Hwajeong Lee
Although nuclear immunostaining for paired box protein (PAX5) is widely used in practice, its cytoplasmic localization has not been evaluated. Recently we encountered cytoplasmic granular PAX5 staining in rectal well-differentiated neuroendocrine tumor (WD-NET) in the absence of nuclear staining. We investigated the specificity of this staining pattern for rectal NET (n=21) in comparison with 108 NETs, 1 WD rectal NET with elevated proliferation (WD-NET G3), and 40 poorly differentiated neuroendocrine carcinomas from the gastrointestinal and pancreatobiliary tract and liver...
March 20, 2018: Applied Immunohistochemistry & Molecular Morphology: AIMM
Benedito A Carneiro, Sahithi Pamarthy, Ami N Shah, Vinay Sagar, Kenji Unno, HuiYing Han, Ximing J Yang, Rubens B Costa, Rebecca J Nagy, Richard B Lanman, Timothy M Kuzel, Jeffrey S Ross, Laurie Gay, Julia A Elvin, Siraj M Ali, Massimo Cristofanilli, Young Kwang Chae, Francis J Giles, Sarki A Abdulkadir
PURPOSE: Small cell carcinoma of the prostate (SCCP) is an aggressive disease that can arise de novo or by transdifferentiation from prostate adenocarcinoma. Alterations in anaplastic lymphoma kinase (ALK) gene are involved in neuroblastoma, lung cancer, and other malignancies but its role in SCCP has not been documented. We describe a patient with refractory de novo SCCP with ALK F1174C activating mutation who obtained clinical benefit from treatment with ALK inhibitor. EXPERIMENTAL DESIGN: Next-generation sequencing (NGS) was used to analyze primary and circulating tumor DNA (ctDNA)...
March 20, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
Bayardo Perez-Ordoñez
Primary neuroendocrine carcinomas (NECs) of the larynx and head and neck are an uncommon and heterogeneous group of neoplasms categorized by the 2017 WHO Classification of Head and Neck Tumors as: (a) well-differentiated (WD-NEC), (b) moderately-differentiated (MD-NEC), and (c) poorly-differentiated (PD-NEC) with small cell and large cell types. The classification incorporates elements of differentiation and grading and closely correlates to the 5-year disease specific survival of 100, 52.8, 19.3 and 15.3% for each diagnostic category...
March 20, 2018: Head and Neck Pathology
William H Westra
Human papillomavirus (HPV)-related head and neck carcinoma (HNC) represents an important subgroup of head and neck cancer that is characterized by a consistent microscopic appearance and a favorable prognosis. A growing experience with HPV testing, however, has uncovered variants that deviate from the prototypic HPV-HNC with respect to morphology. While these HPV-HNCs may deviate morphologically from the prototype, they do not appear to stray far from the favorable clinical outcome assigned to HPV-positive status...
March 20, 2018: Head and Neck Pathology
Rebecca D Chernock, Eric J Duncavage
Salivary neuroendocrine carcinomas are rare and the overwhelming majority is high-grade. The parotid gland is the most commonly involved site followed by the submandibular gland. Most arise de novo but rare examples occurring as a high-grade transformation of another type of salivary gland neoplasm exist. There is significant morphologic and immunophenotypic overlap with neuroendocrine carcinomas of other sites, especially the skin. Like cutaneous neuroendocrine (or Merkel cell) carcinomas, approximately three-fourths are cytokeratin 20 positive...
March 20, 2018: Head and Neck Pathology
Michael T Tetzlaff, Priyadharsini Nagarajan
Merkel cell carcinoma (MCC) is an aggressive cutaneous neuroendocrine carcinoma. Incidence of MCC continues to rise, and risk factors include advanced age, pale skin, chronic sun exposure, and immune suppression. Diagnosing MCC utilizes a combination of morphology and immunohistochemistry. Merkel cell polyomavirus (MCPyV) is present in approximately 70-80% of MCCs and represents a key pathogenic driver in those MCCs. In contrast, MCPyV-negative MCCs arise through progressive accumulation of ultraviolet-light induced somatic mutations...
March 20, 2018: Head and Neck Pathology
Arnaud Jannin, Stéphanie Espiard, Kanza Benomar, Christine Do Cao, Bénédicte Mycinski, Henri Porte, Michèle D'Herbomez, Nicolas Penel, Marie-Christine Vantyghem
The purpose of this study was to analyse the characteristics of 6 patients managed in a university hospital between 1996 and 2016 for non-islet cell tumor hypoglycemia (NICTH), a form of hypoglycaemia due to the paraneoplastic secretion of IGF-2 or its related substances. RESULTS: Three of these 6 patients (50%), aged over 69 years, including 2 with acromegaloid phenotype, presented with a pleural solitary fibrous tumor (SFT), with median diameter 20 cm (interquartile range, 12.5-20.5) with a low median SUV (3...
March 16, 2018: Annales D'endocrinologie
Yiğit Düzköylü, Orhan Aras, Erdal Birol Bostancı, Tülay Keklik Temuçin, Murat Ulaş
AIMS: Mixed adeno-neuroendocrine carcinoma is a rare entity, diagnosed with immunohistochemical studies. Literature mainly includes case reports and series which are very few. In our study, we aimed to report a case series from a tertiary hospital with demographics of the patients, detailed tumor and clinical findings and follow-up plus survival conditions. METHODS: Pathology database was explored for patients with the pathological diagnosis of 'mixed adeno-neuroendocrine carcinoma' and patients were identified retrospectively and evaluated in means of demographics, histopathological examination, tumor properties...
March 19, 2018: Balkan Medical Journal
John R Sims, Travis E Grotz, Barbara A Pockaj, Richard W Joseph, Robert L Foote, Clark C Otley, Amy L Weaver, James W Jakub, Daniel L Price
BACKGROUND: Merkel cell carcinoma (MCC) is a rare cutaneous malignancy of neuroendocrine origin with a high propensity for lymph node metastasis. Sentinel lymph node (SLN) status is important for accurate staging; however, the optimal treatment following SLN biopsy, regardless of nodal status, remains unclear. METHODS: 150 patients with MCC who underwent SLN biopsy from 1995 to 2011 at 3 Mayo Clinic sites were reviewed. RESULTS: Of 150 patients with MCC who underwent SLN biopsy, 39 (26%) were positive and 111 (74%) were negative...
March 2018: Surgical Oncology
Seyran Yiğit, Neşe Ekinci, Leyla Hayrullah, İrfan Öcal, İncim Bezircioğlu
OBJECTIVE: Dedifferentiated endometrioid adenocarcinoma is a recently defined uterine tumor composed of low grade endometrioid adenocarcinoma and undifferentiated carcinoma. Herein we present clinicopathologic, morphologic and immunohistochemical features of 5 dedifferentiated endometrioid adenocarcinoma cases. MATERIAL AND METHODS: All cases diagnosed as mixed endometrial adenocarcinoma (endometrioid + undifferentiated carcinoma) or dedifferentiated endometrioid adenocarcinoma between January 2008 and December 2014 were retrieved from the archives of our institution's pathology department...
March 16, 2018: Journal of the Turkish German Gynecological Association
Mustafa Kupeli, Resit Dogan Koseoglu
Large cell neuroendocrine carcinoma (LCNC) is a rare and aggressive cancer accounting for 3% of all lung cancers. A small percentage of LCNC called combined LCNC, can be seen in combinations with other lung tumor types. Multimodal therapy is proposed for this type of tumor but there is no any significant therapy since it is very rare tumor. This patient was a 66-year male and a heavy smoker with no symptoms of lung disease. Computed tomography core biopsy showed suspected adenocarcinoma. Right lower lobectomy was performed with mediastinal lymph node dissection via mini thoracotomy...
March 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Natalie Herold, Barbara Wappenschmidt, Birgid Markiefka, Katharina Keupp, Sandra Kröber, Eric Hahnen, Rita Schmutzler, Kerstin Rhiem
Non-small cell neuroendocrine carcinomas (NSCNEC) account for 2% of gynecological cancer cases and are associated with a poor prognosis due to delayed diagnosis and aggressive tumor behavior. BRCA2 -associated ovarian carcinomas predominantly possess a high-grade serous phenotype, which respond to platinum and targeted therapy with PARP inhibitors. Presented here is the case of an adult patient with NSCNEC of the ovaries associated with a deleterious BRCA2 germline mutation. The pathogenic mutation was also confirmed on the somatic level, while the wild-type allele had a high variant fraction, suggesting loss of heterozygosity...
April 2018: Oncology Letters
Kentaro Inamura
Immunohistochemistry is a widely available technique that is less challenging and can provide clinically meaningful results quickly and cost-efficiently in comparison with other techniques. In addition, immunohistochemistry allows for the evaluation of cellular localization of proteins in the context of tumor structure. In an era of precision medicine, pathologists are required to classify lung cancer into specific subtypes and assess biomarkers relevant to molecular-targeted therapies. This review summarizes the hot topics of immunohistochemistry in lung cancer, including (i) adenocarcinoma vs squamous cell carcinoma; (ii) neuroendocrine markers; (iii) ALK, ROS1, and EGFR; (iv) PD-L1 (CD274); (v) lung carcinoma vs malignant mesothelioma; and (vi) NUT carcinoma...
March 14, 2018: Cancers
Anna F Farago, Florence K Keane
Small cell lung cancer (SCLC) is an aggressive high-grade neuroendocrine carcinoma. Despite over 30 years of clinical research, little progress has been made in the management of SCLC, and outcomes remain poor. Here, we review the current clinical standards for management of SCLC and the data supporting these strategies.
February 2018: Translational Lung Cancer Research
Kathryn Kostamo, Mishka Peart, Nathalie McKenzie, Conisha Holloman, S J Carlan, Li Ge, John Maksem
Background: Primary vaginal small-cell neuroendocrine carcinoma is an extremely rare and highly aggressive malignancy. Eighty-five percent of patients die within one year of diagnosis from metastatic disease despite multimodal therapy. Gene expression profiling of tumor tissue may be useful for treatment options for various malignancies. Case: A 34-year-old nulliparous woman was diagnosed with primary vaginal small-cell neuroendocrine carcinoma. Twenty weeks after the initial visit, she was diagnosed with recurrence and started on chemoradiation based on the results of gene expression profile of tumor tissue...
2018: Case Reports in Oncological Medicine
Liangli Hong, Ying Zhang, Zhaoyong Liu
Neuroendocrine carcinoma (NEC) of the esophagus and gastric cardia is a rare tumor, and the Chaoshan region has one of the highest incidences of esophageal and gastric cardia cancer (GCC) worldwide. The aim of this study was to characterize the clinicopathologic features of esophageal NEC ( n = 67) and gastric cardia NEC ( n = 13) cases identified over a 9-year period in the Chaoshan region. Esophageal NECs were either purely NEC ( n = 47) or mixed with squamous cell carcinoma or adenocarcinoma ( n = 20). For GCC; pure NEC was found in 5 cases, whereas 8 cases were mixed with adenocarcinomas...
February 13, 2018: Oncotarget
Peiye Shen, Ying Jing, Ruiyun Zhang, Mei-Chun Cai, Pengfei Ma, Haige Chen, Guanglei Zhuang
Neuroendocrine bladder cancer is a relatively rare but often lethal malignancy, with cell of origin, oncogenomic architecture and standard treatment poorly defined. Here we performed comprehensive whole-genome and transcriptome sequencing on a unique cohort of genitourinary neuroendocrine neoplasms, mainly small cell carcinomas of the urinary bladder. The mutational landscape and signatures of neuroendocrine bladder cancer strikingly resembled those in conventional urothelial carcinoma, along with typically mixed histologies, supporting a common cellular origin...
March 14, 2018: Oncogene
Julie George, Vonn Walter, Martin Peifer, Ludmil B Alexandrov, Danila Seidel, Frauke Leenders, Lukas Maas, Christian Müller, Ilona Dahmen, Tiffany M Delhomme, Maude Ardin, Noemie Leblay, Graham Byrnes, Ruping Sun, Aurélien De Reynies, Anne McLeer-Florin, Graziella Bosco, Florian Malchers, Roopika Menon, Janine Altmüller, Christian Becker, Peter Nürnberg, Viktor Achter, Ulrich Lang, Peter M Schneider, Magdalena Bogus, Matthew G Soloway, Matthew D Wilkerson, Yupeng Cun, James D McKay, Denis Moro-Sibilot, Christian G Brambilla, Sylvie Lantuejoul, Nicolas Lemaitre, Alex Soltermann, Walter Weder, Verena Tischler, Odd Terje Brustugun, Marius Lund-Iversen, Åslaug Helland, Steinar Solberg, Sascha Ansén, Gavin Wright, Benjamin Solomon, Luca Roz, Ugo Pastorino, Iver Petersen, Joachim H Clement, Jörg Sänger, Jürgen Wolf, Martin Vingron, Thomas Zander, Sven Perner, William D Travis, Stefan A Haas, Magali Olivier, Matthieu Foll, Reinhard Büttner, David Neil Hayes, Elisabeth Brambilla, Lynnette Fernandez-Cuesta, Roman K Thomas
Pulmonary large-cell neuroendocrine carcinomas (LCNECs) have similarities with other lung cancers, but their precise relationship has remained unclear. Here we perform a comprehensive genomic (n = 60) and transcriptomic (n = 69) analysis of 75 LCNECs and identify two molecular subgroups: "type I LCNECs" with bi-allelic TP53 and STK11/KEAP1 alterations (37%), and "type II LCNECs" enriched for bi-allelic inactivation of TP53 and RB1 (42%). Despite sharing genomic alterations with adenocarcinomas and squamous cell carcinomas, no transcriptional relationship was found; instead LCNECs form distinct transcriptional subgroups with closest similarity to SCLC...
March 13, 2018: Nature Communications
Luis V Syro, Fabio Rotondo, Leon D Ortiz, Kalman Kovacs
Temozolomide is an alkylating chemotherapeutic agent used in malignant neuroendocrine neoplasia, melanoma, brain metastases, and an essential component of adjuvant therapy in the treatment of glioblastoma multiforme and anaplastic astrocytoma. Since 2006 it has been used for the treatment of pituitary carcinomas and aggressive pituitary adenomas. Here, we discuss the current indications and results of temozolomide therapy in pituitary tumors, as well as frequently asked questions regarding temozolomide treatment, duration of therapy, dosage, tumor recurrence, and resistance...
March 13, 2018: Endocrine-related Cancer
Shira Ronen, Irene Aguilera-Barrantes, Tamara Giorgadze, Petr Šteiner, Petr Grossmann, Saul Suster
Polymorphous sweat gland carcinoma is an uncommon low-grade malignant adnexal tumor with a marked predilection for the distal extremities. Histologically, the lesions are characterized by a cellular proliferation showing a combination of growth patterns, including trabecular, solid, tubular, cribriform, or adenoid cystic and pseudopapillary. The immunohistochemical and molecular profile of these tumors has not yet been properly addressed. We have studied 3 cases of polymorphous sweat gland carcinoma using a broad panel of immunohistochemical markers including cytokeratin AE1/AE3, CK5/6, MOC31, p40, p63, p16, chromogranin, synaptophysin, CD56, MIB-1, estrogen receptor, progesterone receptor, androgen receptor, BER-EP4, smooth muscle actin, epithelial membrane antigen, carcinoembryonic antigen, CD117, S100 protein, HBME-1, DOG1, vimentin, and mammaglobin...
March 6, 2018: American Journal of Dermatopathology
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