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Neuroendocrine tumors head neck

Lisa M Rooper, Justin A Bishop, William H Westra
The head and neck is the site of a wide and sometimes bewildering array of neuroendocrine (NE) tumors. Although recognition of NE differentiation may be necessary for appropriate tumor classification and treatment, traditional NE markers such as synaptophysin, chromogranin, and CD56 are not always sufficiently sensitive or specific to make this distinction. Insulinoma-associated protein 1 (INSM1) is a novel transcription factor that has recently demonstrated excellent sensitivity and specificity for NE differentiation in various anatomic sites, but has not yet been extensively evaluated in tumors of the head and neck...
February 12, 2018: American Journal of Surgical Pathology
Trevan Fischer, Shrawan Gaitonde, Maris Jones, Bradley Bandera, Melanie Goldfarb
Paragangliomas (PGL) are rare neuroendocrine tumors. This study describes the largest collection of PGLs and evaluates factors that impact survival. Patients with PGL from 1998 to 2013 in the NCDB were reviewed. Independent predictors of overall survival (OS) were identified for patients with non-central nervous system (CNS) tumors. Of 867 PGLs, the primary site was CNS (39.9%), abdomen/pelvis (A&P) (21.0%), head and neck (H&N) (17.5%), thoracic (15.1%), bladder (3%), or unspecified (3.5%). Of 521 non-central nervous system (CNS) PGLs, there were differences in sex, comorbidities, treatment facility, tumor size, treatment modality (P < 0...
October 1, 2017: American Surgeon
Brendan C Dickson, Yun-Shao Sung, Marc K Rosenblum, Victor E Reuter, Mohammed Harb, Jay S Wunder, David Swanson, Cristina R Antonescu
NUT midline carcinoma is an aggressive tumor that occurs mainly in the head and neck and, less frequently, the mediastinum and lung. Following identification of an index case of a NUTM1 fusion positive undifferentiated soft tissue tumor, we interrogated additional cases of primary undifferentiated soft tissue and visceral tumors for NUTM1 abnormalities. Targeted next-generation sequencing was performed on RNA extracted from formalin-fixed paraffin-embedded tissue, and results validated by fluorescence in situ hybridization using custom bacterial artificial chromosome probes...
January 19, 2018: American Journal of Surgical Pathology
Tilmann Bochtler, Harald Löffler, Alwin Krämer
In cancer of unknown primary (CUP), metastases are clinically and histologically confirmed, but the primary tumor site remains elusive after extensive work-up. CUPs make up for 2-3% of all epithelial malignancies. The two prevailing histologies are adenocarcinomas and undifferentiated carcinomas, whereas squamous cell carcinomas, neuroendocrine carcinomas and rare histologies account for the remaining 10%. The diagnostic work-up in CUP relies strongly on a detailed immunohistological (IHC) analysis in order to characterize the tumor type, nowadays aided by molecular techniques...
November 26, 2017: Seminars in Diagnostic Pathology
Leilani B Mercado-Asis, Katherine I Wolf, Ivana Jochmanova, David Taïeb
OBJECTIVE: Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumors derived from adrenal or extra-adrenal locations, respectively. Upon suspicion of PPGL, specific metabolomic, molecular, biochemical, imaging, and histopathological studies are performed to prove, localize, treat, and monitor disease progression. Recently, improved diagnostic tools allow physicians to accurately diagnose PPGL, even in patients presenting with small (less than 1 cm) or biochemically silent tumors, which previously delayed proper detection and treatment...
November 16, 2017: Endocrine Practice
Q Luo, Y N Liu, H Y Ma, S Li, J Y Huang, G Li, G Jin
Objective: To discuss the clinical pathology characteristics, strategies of diagnosis and therapy, and related prognosis factors of pancreatic neuroendocrine tumors(pNET). Methods: Clinical data of 103 pNET cases in Department of Pancreatic Surgery, Changhai Hospital of Second Military Medical University were collected from January 2006 to December 2015. There were 44 males and 59 females, aged from 21 to 77 years with mean age of 48 years.Of the 103 patients, there were functional type in 21 cases and no functional type in 82 cases...
October 1, 2017: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
Mohammed N Islam, Hardeep Chehal, Molly Housley Smith, Sarah Islam, Indraneel Bhattacharyya
Merkel cell carcinoma (MCC) is an uncommon relatively aggressive neuroendocrine dermal neoplasm first described in 1972 as a tumor of the sun exposed skin. Although most MCC affect the skin of the head and neck, rare primarily oral mucosal cases have been documented. Merkel cells are nondendritic neuroendocrine cells that are found not only in the skin but also the oral mucosa and give rise to MCC. Neuroendocrine cells may be found as aggregates in organs or as diffuse or isolated cells within organs and their epithelial lining...
October 4, 2017: Head and Neck Pathology
Akihiko Kawahara, Takeharu Ono, Akiko Sumi, Hideyuki Abe, Yorihiko Takase, Kazuya Murata, Tomohiko Yamaguchi, Yoshiki Naito, Jun Akiba
Head and neck large cell neuroendocrine carcinoma (LCNEC) is a rare high-grade malignant tumor with neuroendocrine differentiation. We report a case of LCNEC causing difficulty in cytological diagnosis. A 60-year-old man with right-sided face pain presented with a swelling at the right cheek, and he complained of right nasal obstruction and lacrimation. Preoperative fine-needle aspiration cytology (FNAC) showed high cellularity, with a moderate number of clusters of tumor cells on an abundant necrotic background...
February 2018: Diagnostic Cytopathology
So Y Yim, Valeria M Moncayo, Francisco J Pasquel, Raghuveer K Halkar
Hereditary paraganglioma (PGL)-pheochromocytoma (PCC) syndrome is a genetic disorder caused by a mutation of the tumor suppressor gene SDHD that results in a predisposition for head and neck PGLs and PCCs. We present a case of a 33-year-old woman where F-FDG PET/CT showed areas of increased uptake in both the adrenal and cervical regions, consistent with PCCs and PGLs, respectively. Further imaging revealed that PCCs were I-MIBG avid, whereas the PGLs were In-octreotide avid. This demonstrates the varying sensitivities of different imaging modalities in regard to neuroendocrine tumors and the potential for treatment using multiple targeted therapies...
December 2017: Clinical Nuclear Medicine
S Uccella, G Ottini, C Facco, R Maragliano, S Asioli, F Sessa, S La Rosa
Neuroendocrine neoplasms (NENs) of the head and neck are a rare group of heterogeneous epithelial neoplastic proliferations arising in virtually all of the different organs of this region, particularly in the nasal cavity, the paranasal sinuses, the nasopharynx, the larynx, the salivary glands, and the middle ear. They encompass a wide spectrum of entities ranging from very indolent neuroendocrine tumors to highly aggressive neuroendocrine carcinomas. They may represent a challenge for radiologists, oncologists, and pathologists and a correct diagnosis is crucial for the management of patients...
March 2017: Pathologica
J S Lewis, R D Chernock, J A Bishop
The performance characteristics of neuroendocrine-specific and squamous-specific immunohistochemical markers in head and neck squamous cell carcinomas (SCC), in particular in oropharyngeal tumors in this era of human papillomavirus (HPV)-induced cases, are not well-established. The differential diagnosis for poorly differentiated SCCs, for nonkeratinizing oropharyngeal SCCs, and for other specific SCC variants such as basaloid SCC and undifferentiated (or lymphoepithelial-like) carcinomas includes neuroendocrine carcinomas...
May 20, 2017: Head and Neck Pathology
Lester D R Thompson, Alessandro Franchi
The World Health Organization recently published the 4th edition of the Classification of Head and Neck Tumors, including several new entities, emerging entities, and significant updates to the classification and characterization of tumor and tumor-like lesions, specifically as it relates to nasal cavity, paranasal sinuses, and skull base in this overview. Of note, three new entities (NUT carcinoma, seromucinous hamartoma, biphenotypic sinonasal sarcoma,) were added to this section, while emerging entities (SMARCB1-deficient carcinoma and HPV-related carcinoma with adenoid cystic-like features) and several tumor-like entities (respiratory epithelial adenomatoid hamartoma, chondromesenchymal hamartoma) were included as provisional diagnoses or discussed in the setting of the differential diagnosis...
April 25, 2017: Virchows Archiv: An International Journal of Pathology
Anand C Baxi, Qingwei Jiang, Jinghua Hao, Zhuo Yang, Kevin Woods, Steven Keilin, Field F Willingham, Qiang Cai
OBJECTIVES: To evaluate the effect of solid pancreatic masses on the pancreatic duct (PD) at the endoscopic ultrasound (EUS) and the relationship of the location/size of a mass and PD dilation. MATERIALS AND METHODS: Patients who underwent EUS for pancreatic indications from 2011 to 2013 at a single center were retrospectively identified. Those with biopsies that revealed adenocarcinoma or neuroendocrine tumors in the pancreas were identified and PD size was ascertained from EUS, computed tomography, or magnetic resonance imaging...
March 2017: Endoscopic Ultrasound
Roberta Marangoni, Simone Mauramati, Giulia Bertino, Antonio Occhini, Marco Benazzo, Patrizia Morbini
PURPOSE: A collision tumor consists of 2 different histologically distinct and topographically independent tumors merging in the same mass. In the head and neck region they are rare, with only 4 cases reported in the larynx. CASE REPORT: A 60-year-old heavy smoker complained of a left submandibular lesion in October 2014. The lesion was excised and showed a poorly differentiated carcinoma, suggestive for adenocarcinoma. After a positron emission tomography-computed tomography scan showed increased metabolic activity in the left laterocervical region and right vocal cord, the patient underwent endoscopic biopsy of the lesion of the left piriform sinus, which was positive for moderately differentiated squamous cell carcinoma (SCC)...
November 15, 2017: Tumori
Jacob Ruzevick, Eun Kyung Koh, Luis F Gonzalez-Cuyar, Patrick J Cimino, Kristen Moe, Lorena A Wright, Richard Failor, Manuel Ferreira
Head and neck paragangliomas are rare neuroendocrine tumors that arise from paraganglion cells of the parasympathetic nervous system. Paragangliomas arising from the midline skull base have only rarely been reported. Surgery is the mainstay of treatment and adjuvant radiation is often recommended. These tumors can rarely secrete metanephrines and normetanephrines which can complicate operative management. Here we present two cases of clival paragangliomas with unique clinical presentations and review the previous literature on skull base paragangliomas...
May 2017: Journal of Neuro-oncology
X J Cui, C Y Zhang, P Su, B Lyu, Y B Ren, X S Fan, X F Zhang, Y Wang, Y H Song, Z Y Liu
Objective: To investigate clinicopathological features of carcinoma showing thymus-like elements (CASTLE) in the head and neck regions. Methods: Clinicopathological data of 7 patients with CASTLE in the head and neck regions were retrospectively reviewed.Immunohistochemical staining and in situ hybridization for EBER were performed. BRAF(V600E) mutation was examined by ARMS method in 6 cases. Results: There were 5 females and 2 males with age between 49 and 78 years (average of 65.6 years). All tumors were solitary nodular lesions with an infiltrative border, including 6 intrathyroid tumors and 1 extrathyroid tumor in the laryngeal pharynx...
March 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Nina Gale, Mario Poljak, Nina Zidar
Chapter 3 "Tumours of the hypopharynx, larynx, trachea, and parapharyngeal space" of the World Health Organization (WHO) Blue Book 2017 "Classification of Head and Neck Tumours" shows a shortened list of entities, especially due to reducing the number of benign and malignant soft tissue tumours, malignant melanoma and some others, which are transferred to more frequently affected regions of the head and neck. The basic concept of the new edition is to assimilate all advances concerning the discussed tumours in a shorter framework, appropriate for daily work...
March 2017: Head and Neck Pathology
Michelle D Williams, Arthur S Tischler
Updated editions of The World Health Organization Classification of Tumours Pathology & Genetics for both Head and Neck Tumours and Tumours of Endocrine Organs took place in 2016 based on consensus conferences. These editions present unification of concepts in paragangliomas and highlight expanding knowledge of their etiology. There is a major emphasis in the new bluebooks on familial/syndromic paragangliomas, representing ~40% of all head and neck paragangliomas. Ancillary use of immunohistochemical evaluation, specifically of SDHB, allows the pathologist to screen for a large subset of these potentially hereditary cases...
March 2017: Head and Neck Pathology
Diana Amorim-Pires, Joana Peixoto, Jorge Lima
Pheochromocytomas (PCC) and sympathetic paragangliomas (PGL) are rare neuroendocrine tumors, which derive from chromaffin cells occurring in the adrenal medulla and extra-adrenal sympathetic paraganglia. PCC and PGL are often benign, catecholamine-producing tumors, responsible for a myriad of symptoms that may be potentially hazardous to the patient. In contrast, nonsecreting parasympathetic PGL, derived from chief cells, develop mainly in the head and neck region. Although PCC/PGL are more commonly sporadic tumors, germline mutations are present in up to 40% of the patients, ranking these tumors among those with the highest degree of heritability...
2016: Cytogenetic and Genome Research
Catalina Uribe Galeano, Joan Fabregat Prous, Juli Busquets Barenys, Nuria Pelaez Serra, Lluís Secanella Medayo, Emilio Ramos Rubio, Sandra Ruiz Osuna, Carlos Villabona Artero
OBJECTIVE: The availability of new imaging techniques has conditioned an increase in the incidental diagnosis of small nonfunctioning pancreatic neuroendocrine tumors (PNET-NF). The best treatment is controversial, some authors advise a conservative approach in selected cases. Our aim is to analyze the evolution of incidental, small size PNET-NF, treated with clinical follow-up without surgery. METHODS: We performed a retrospective analysis of a prospective database of patients diagnosed incidentally with PNET-NF since November 2007 to September 2015...
February 2, 2017: Cirugía Española
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