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Justin Lim - One of the best experts on this subject based on the ideXlab platform.
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a clinical scoring system to identify patients with sebaceous Neoplasms at risk for the muir torre variant of lynch syndrome
Genetics in Medicine, 2014Co-Authors: Maegan E Roberts, Douglas L Riegertjohnson, Brittany C Thomas, Kandelaria M Rumilla, Colleen S Thomas, Michael G Heckman, Jennifer U Purcell, Nancy Hanson, Kathleen A Leppig, Justin LimAbstract:The Muir–Torre syndrome variant of Lynch syndrome is characterized by the presence of sebaceous Neoplasms (adenoma, epithelioma/sebaceoma, carcinoma) and Lynch syndrome–associated cancers (colon, endometrial, and others). Several clinical scoring systems have been developed to identify patients with colon cancer at high risk of Lynch syndrome. However, no such system has been described for patients presenting with sebaceous Neoplasms. Based on logistic regression analysis, a scoring system was developed for patients with sebaceous neoplasm to identify those with the highest likelihood of having Muir–Torre syndrome. The final version of the scoring system included variables such as age at presentation of initial sebaceous neoplasm, total number of sebaceous Neoplasms, personal history of a Lynch-related cancer, and family history of Lynch-related cancers. Patients with a score of 3 or more were more likely to have Muir–Torre syndrome (28 of 29 patients), those with a score of 2 had intermediate likelihood (12 of 20 patients), and no patient with a score of 0 or 1 was diagnosed with Muir–Torre syndrome. The Mayo Muir–Torre syndrome risk scoring system appears to identify whether patients who present with sebaceous Neoplasms are in need of further Lynch syndrome evaluation using easily ascertained clinical information. Abnormal mismatch repair gene immunohistochemistry of a sebaceous neoplasm is a poor predictor in regard to diagnosing Lynch syndrome. Genet Med 16 9, 711–716.
Ralph H Hruban - One of the best experts on this subject based on the ideXlab platform.
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PIK3CA Mutations in Mucinous Cystic Neoplasms of the Pancreas
Pancreas, 2014Co-Authors: Dario Garcia-carracedo, Zong Ming Chen, Wanglong Qiu, Alicia S. Huang, Sophia M. Tang, Ralph H HrubanAbstract:Objectives Mucinous cystic Neoplasms (MCNs) are rare, potentially curable, mucin-producing Neoplasms of the pancreas. We have previously reported PIK3CA (phosphoinositide-3-kinase catalytic subunit, p110α) mutations in intraductal papillary mucinous Neoplasms, another mucin-producing neoplasm of the pancreas. In this study, we analyzed the presence of PIK3CA and AKT1/PKB (V-akt murine thymoma viral oncogene homolog 1) hot-spot mutations in MCN specimens.
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reporting precursors to invasive pancreatic cancer pancreatic intraepithelial neoplasia intraductal Neoplasms and mucinous cystic neoplasm
Diagnostic Histopathology, 2012Co-Authors: Ralph H Hruban, Anirban Maitra, Roeland F. De Wilde, Johan G A OfferhausAbstract:Abstract Invasive ductal adenocarcinoma of the pancreas remains an almost universally lethal disease. Despite strenuous research efforts, the prognosis of the disease has not improved in the past decades. However, knowledge of pancreatic tumorigenesis and the identification and characterization of the precursor lesions that give rise to invasive pancreatic cancer have dramatically improved. This, coupled with the finding that it takes almost two decades for a pancreatic cell with an initial mutation to develop into a metastatic pancreatic cancer provides hope for the early detection of curable pancreatic Neoplasms. We present a review of established precursor lesions of pancreatic cancer, including pancreatic intraepithelial neoplasia, intraductal papillary mucinous Neoplasms (including intraductal oncocytic papillary neoplasm and intraductal tubulopapillary neoplasm), and mucinous cystic neoplasm.
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intraductal papillary mucinous neoplasm
Human Pathology, 2012Co-Authors: Ralph H HrubanAbstract:Summary Intraductal papillary mucinous neoplasm (IPMN) is a grossly visible (≥1 cm), mucin-producing neoplasm that arises in the main pancreatic duct and/or its branches. Patients with intraductal papillary mucinous neoplasm can present with symptoms caused by obstruction of the pancreatic duct system, or they can be asymptomatic. There are 3 clinical subtypes of intraductal papillary mucinous neoplasm: main duct, branch duct, and mixed. Five histologic types of intraductal papillary mucinous neoplasm are recognized: gastric foveolar type, intestinal type, pancreatobiliary type, intraductal oncocytic papillary neoplasm, and intraductal tubulopapillary neoplasm. Noninvasive intraductal papillary mucinous Neoplasms are classified into 3 grades based on the degree of cytoarchitectural atypia: low-, intermediate-, and high-grade dysplasia. The most important prognosticator, however, is the presence or absence of an associated invasive carcinoma. Some main duct-intraductal papillary mucinous Neoplasms progress into invasive carcinoma, mainly tubular adenocarcinoma (conventional pancreatic ductal adenocarcinoma) and colloid carcinoma. Branch duct-intraductal papillary mucinous Neoplasms have a low risk for malignant transformation. Preoperative prediction of the malignant potential of an intraductal papillary mucinous neoplasm is of growing importance because pancreatic surgery has its complications, and many small intraductal papillary mucinous Neoplasms, especially branch duct–intraductal papillary mucinous Neoplasms, have an extremely low risk of progressing to an invasive cancer. Although most clinical decision making relies on imaging, a better understanding of the molecular genetics of intraductal papillary mucinous neoplasm could help identify molecular markers of high-risk lesions. When surgery is performed, intraoperative frozen section assessment of the pancreatic resection margin can guide the extent of resection. Intraductal papillary mucinous Neoplasms are often multifocal, and surgically resected patients should be followed for metachronous disease.
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Pdx1 Expression in Pancreatic Precursor Lesions and Neoplasms
Applied Immunohistochemistry & Molecular Morphology, 2011Co-Authors: Jason Y. Park, Anirban Maitra, David S. Klimstra, Seung-mo Hong, Michael Goggins, Ralph H HrubanAbstract:Pancreatic and duodenal homeobox (Pdx1) is a homeobox transcription factor required for the embryonic development of the pancreas. Pdx1 expression has been earlier identified in pancreatic ductal adenocarcinomas and endocrine Neoplasms. This study characterizes Pdx1 protein expression in pancreatic precursor lesions and Neoplasms, including pancreatic intraepithelial neoplasia (PanIN, n=32), intraductal papillary mucinous neoplasm (IPMN, n=88), mucinous cystic neoplasm (MCN, n=3), acinar cell carcinoma (ACC, n=8), pancreatic endocrine neoplasm (PEN, n=44), pancreatoblastoma (PB, n=1), solid pseudopapillary neoplasm (n=8), invasive ductal adenocarcinoma (n=67), and nondysplastic ductal epithelium. A mouse monoclonal antibody for Pdx1 was used to examine archived surgical pathology cases and tissue microarrays containing >655 tissue cores from more than 250 pancreatic specimens. Immunohistochemical labeling for Pdx1 was performed using standard methods and scored for percentage and intensity of nuclear labeling. Among non-neoplastic pancreatic tissues, Pdx1 nuclear protein was expressed in islet cells, cells of the centroacinar cell compartment, and non-neoplastic ductal epithelium. No expression of Pdx1 was seen in non-neoplastic acinar cells. Among pancreatic Neoplasms, Pdx1 consistently labeled >50% of the tumor cells in 87.5% of ACC cases and 38.6% of PEN cases. Pdx1 expression was variable in invasive ductal adenocarcinoma and precursor lesions of ductal adenocarcinomas (PanIN, IPMN, and MCN). A single case of PB was examined and it showed Pdx1 in the acinar component, but no expression in squamoid nests. Solid pseudopapillary Neoplasms did not express Pdx1. This study shows Pdx1 expression in precursor lesions of ductal adenocarcinomas, PEN, ACC, and a case of PB. In the immunohistochemical evaluation of Neoplasms of the pancreas, Pdx1 expression is not a finding specific to PENs and ductal adenocarcinomas, but also occurs in precursor lesions (PanIN, IPMN, MCN) and other Neoplasms of the pancreas.
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Mucinous cystic Neoplasms of the pancreas.
Seminars in Diagnostic Pathology, 2000Co-Authors: Robb E. Wilentz, Albores-saavedra J, Ralph H HrubanAbstract:: Since their initial description, mucinous cystic Neoplasms have been difficult to classify. This article attempts to clarify histological, clinical, and genetic criteria so that the pathologist can categorize each mucinous cystic neoplasm into 1 of 4 possible categories. Mucinous cystadenomas contain a single layer of mucin-producing, columnar epithelium lacking significant atypia. Borderline mucinous cystic Neoplasms contain cells with moderate atypia. Mucinous cystic Neoplasms with in situ carcinoma show significant architectural and cytological atypia. When invasive carcinoma is present in association with a mucinous cystic neoplasm, then the diagnosis of invasive mucinous cystadenocarcinoma should be made. The categorization of mucinous cystic Neoplasms into these groups is essential because it accurately predicts outcome, provided that the tumor has been sampled and examined thoroughly. Completely removed mucinous cystadenomas, borderline mucinous cystic Neoplasms, and mucinous cystic Neoplasms with in situ carcinoma follow benign courses. Partial resection should be avoided as evidence suggests that mucinous cystic Neoplasms can progress from adenomas to borderline lesions to carcinomas in situ to invasive carcinomas over time; partial resection should be avoided if possible. Modern molecular genetic techniques are helping to unravel the origins of rare variants of mucinous cystic tumors, such as the mucinous cystic tumor with an associated osteoclast-like giant cell tumor and the mucinous cystic tumor with sarcomatous stroma.
Maegan E Roberts - One of the best experts on this subject based on the ideXlab platform.
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a clinical scoring system to identify patients with sebaceous Neoplasms at risk for the muir torre variant of lynch syndrome
Genetics in Medicine, 2014Co-Authors: Maegan E Roberts, Douglas L Riegertjohnson, Brittany C Thomas, Kandelaria M Rumilla, Colleen S Thomas, Michael G Heckman, Jennifer U Purcell, Nancy Hanson, Kathleen A Leppig, Justin LimAbstract:The Muir–Torre syndrome variant of Lynch syndrome is characterized by the presence of sebaceous Neoplasms (adenoma, epithelioma/sebaceoma, carcinoma) and Lynch syndrome–associated cancers (colon, endometrial, and others). Several clinical scoring systems have been developed to identify patients with colon cancer at high risk of Lynch syndrome. However, no such system has been described for patients presenting with sebaceous Neoplasms. Based on logistic regression analysis, a scoring system was developed for patients with sebaceous neoplasm to identify those with the highest likelihood of having Muir–Torre syndrome. The final version of the scoring system included variables such as age at presentation of initial sebaceous neoplasm, total number of sebaceous Neoplasms, personal history of a Lynch-related cancer, and family history of Lynch-related cancers. Patients with a score of 3 or more were more likely to have Muir–Torre syndrome (28 of 29 patients), those with a score of 2 had intermediate likelihood (12 of 20 patients), and no patient with a score of 0 or 1 was diagnosed with Muir–Torre syndrome. The Mayo Muir–Torre syndrome risk scoring system appears to identify whether patients who present with sebaceous Neoplasms are in need of further Lynch syndrome evaluation using easily ascertained clinical information. Abnormal mismatch repair gene immunohistochemistry of a sebaceous neoplasm is a poor predictor in regard to diagnosing Lynch syndrome. Genet Med 16 9, 711–716.
Kenji Kashima - One of the best experts on this subject based on the ideXlab platform.
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malignant serous cystic neoplasm of the pancreas report of a case and review of the literature
Journal of Clinical Gastroenterology, 2005Co-Authors: Toshifumi Matsumoto, Seitaro Hirano, Kazuhiro Yada, Masayuki Ohta, Seigo Kitano, Kohei Shibata, Atsushi Sasaki, Tetsuro Kamimura, Kenji KashimaAbstract:Background:In general, serous cystic Neoplasms of the pancreas are thought to be benign. Malignant serous cystic neoplasm of the pancreas is a rare clinical entity.Case Report:We report the case of an 87-year-old woman with a serous microcystic neoplasm in the tail of the pancreas that behaved in a
Kamal K Khurana - One of the best experts on this subject based on the ideXlab platform.
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Incidence of Non-Salivary Gland Neoplasms in Patients with Warthin Tumor: A Study of 73 Cases
Head and Neck Pathology, 2019Co-Authors: Daniel J. Zaccarini, Kamal K KhuranaAbstract:Warthin tumor is the second most common benign parotid neoplasm. Its association with non-salivary gland Neoplasms has been sporadically reported. We reviewed clinical records of Warthin tumor diagnosed on aspiration cytology and surgical pathology to determine if there is any association with other extra-salivary gland malignant Neoplasms. Computer search was made for all cases of Warthin tumor diagnosed in the parotid gland by aspiration cytology and surgical pathology at our institution between January 2007 and August 2016. Clinical records of all cases were reviewed for any associated malignant Neoplasms and any surgical follow up. All available cytology and histologic material was reviewed. Seventy-three patients (mean 66.9, M:F 1.1:1, age range 43 to 87 years) with Warthin tumor were identified. 45 (62%) were diagnosed on aspiration cytology only, 19 (26%) had cytologic diagnosis as well as concordant surgical follow up, and 9 (12%) were diagnosed based on surgical pathology only. Average age for patients with and without secondary malignancy was 70.5-years, and 63.4-years, respectively (p 0.05). Twenty-seven (37.0%) patients harbored a malignant neoplasm. Association of extra salivary gland malignant Neoplasms in 37.0% of our cases suggest that the prevalence of secondary non-salivary Neoplasms in patients harboring Warthin tumor might have been underestimated. Squamous cell carcinoma was the most commonly associated non-salivary malignant neoplasm. The association of Warthin tumor with smoking plays an important role in this increased rate of malignancy, and this is supported by the fact that smoking is highly associated with head and neck and lung cancers.
