Lung Equivalent Terms, Definitions, Charts, Tables and Illustrations C340-C349 (Excludes Lymphoma and Leukemia M9590-9989 and Kaposi Sarcoma M9140)
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Cholangiocarcinoma 2020: the Next Horizon in Mechanisms and Management
CONSENSUS STATEMENT Cholangiocarcinoma 2020: the next horizon in mechanisms and management Jesus M. Banales 1,2,3 ✉ , Jose J. G. Marin 2,4, Angela Lamarca 5,6, Pedro M. Rodrigues 1, Shahid A. Khan7, Lewis R. Roberts 8, Vincenzo Cardinale9, Guido Carpino 10, Jesper B. Andersen 11, Chiara Braconi 12, Diego F. Calvisi13, Maria J. Perugorria1,2, Luca Fabris 14,15, Luke Boulter 16, Rocio I. R. Macias 2,4, Eugenio Gaudio17, Domenico Alvaro18, Sergio A. Gradilone19, Mario Strazzabosco 14,15, Marco Marzioni20, Cédric Coulouarn21, Laura Fouassier 22, Chiara Raggi23, Pietro Invernizzi 24, Joachim C. Mertens25, Anja Moncsek25, Sumera Rizvi8, Julie Heimbach26, Bas Groot Koerkamp 27, Jordi Bruix2,28, Alejandro Forner 2,28, John Bridgewater 29, Juan W. Valle 5,6 and Gregory J. Gores 8 Abstract | Cholangiocarcinoma (CCA) includes a cluster of highly heterogeneous biliary malignant tumours that can arise at any point of the biliary tree. Their incidence is increasing globally, currently accounting for ~15% of all primary liver cancers and ~3% of gastrointestinal malignancies. The silent presentation of these tumours combined with their highly aggressive nature and refractoriness to chemotherapy contribute to their alarming mortality, representing ~2% of all cancer-related deaths worldwide yearly. The current diagnosis of CCA by non-invasive approaches is not accurate enough, and histological confirmation is necessary. Furthermore, the high heterogeneity of CCAs at the genomic, epigenetic and molecular levels severely compromises the efficacy of the available therapies. In the past decade, increasing efforts have been made to understand the complexity of these tumours and to develop new diagnostic tools and therapies that might help to improve patient outcomes. -
Scientific Framework for Pancreatic Ductal Adenocarcinoma (PDAC)
Scientific Framework for Pancreatic Ductal Adenocarcinoma (PDAC) National Cancer Institute February 2014 1 Table of Contents Executive Summary 3 Introduction 4 Background 4 Summary of the Literature and Recent Advances 5 NCI’s Current Research Framework for PDAC 8 Evaluation and Expansion of the Scientific Framework for PDAC Research 11 Plans for Implementation of Recommended Initiatives 13 Oversight and Benchmarks for Progress 18 Conclusion 18 Links and References 20 Addenda 25 Figure 1: Trends in NCI Funding for Pancreatic Cancer, FY2000-FY2012 Figure 2: NCI PDAC Funding Mechanisms in FY2012 Figure 3: Number of Investigators with at Least One PDAC Relevant R01 Grant FY2000-FY2012 Figure 4: Number of NCI Grants for PDAC Research in FY 2012 Awarded to Established Investigators, New Investigators, and Early Stage Investigators Table 1: NCI Trainees in Pancreatic Cancer Research Appendices Appendix 1: Report from the Pancreatic Cancer: Scanning the Horizon for Focused Invervention Workshop Appendix 2: NCI Investigators and Projects in PDAC Research 2 Scientific Framework for Pancreatic Ductal Carcinoma Executive Summary Significant scientific progress has been made in the last decade in understanding the biology and natural history of pancreatic ductal adenocarcinoma (PDAC); major clinical advances, however, have not occurred. Although PDAC shares some of the characteristics of other solid malignancies, such as mutations affecting common signaling pathways, tumor heterogeneity, development of invasive malignancy from precursor lesions, -
Sarcomatoid Renal Cell Carcinom Atoid Renal Cell Carcinoma
Research Article Sarcomatoid renal cell carcinoma: A case series K Subashree 1*, M Susruthan 2, N Priyathersini 3, Leena Dennis Joseph 4, Sandhya Sundaram 5 1PG Student, 2,3 Assistant Professor, 4,5 Professor, Department of Pathology , Sri Ramachandra Medical College and Research Institute Porur, Chennai-116, Tamil Nadu, INDIA. Email : [email protected] Abstract Introduction: Renal cell carcinoma is the most common form of Renal malignancy. Sarcomatoid renal cell carcinoma represents a high grade dedifferentiation of Renal cell carcinoma. This phenotype can occur in all subtypes of renal cell carcinomas, including clear cell, papillary, chromophob e, and collecting duct carcinoma. Although prognosis in sarcomatoid renal cell carcinoma is known to be extremely poor, little is known about the clinicopathologic information about them. Methods and Methodology: The population of this retrospective study consisted of all patients who underwent surgery for Renal cell carcinoma between January 2010 and February 2013 in the Department of Pathology, Sri Ramachandra university and Research Institute .A total of 64 renal cell carcinoma cases were diagnosed among which 9 had sarcomatoid changes. All 9 cases were reassessed and the diagnosis was confirmed on the basis of morphologic features. Complete baseline and follow -up data were available for analysis for all 9 patients. Conclusion: Present study showed that s arcomatoid changes were seen more commonly in younger age group with male preponderance and ne crosis was a consistent feature. Sarcomatoid areas constitute 1-25% in majority of cases which makes extensive sampling an important measure. Majority of renal cell carcinomas with sarcomatoid changes ha ve a high grade and high stage. -
The American Society of Colon and Rectal Surgeons Clinical Practice Guidelines for the Management of Inherited Polyposis Syndromes Daniel Herzig, M.D
CLINICAL PRACTICE GUIDELINES The American Society of Colon and Rectal Surgeons Clinical Practice Guidelines for the Management of Inherited Polyposis Syndromes Daniel Herzig, M.D. • Karin Hardimann, M.D. • Martin Weiser, M.D. • Nancy Yu, M.D. Ian Paquette, M.D. • Daniel L. Feingold, M.D. • Scott R. Steele, M.D. Prepared by the Clinical Practice Guidelines Committee of The American Society of Colon and Rectal Surgeons he American Society of Colon and Rectal Surgeons METHODOLOGY (ASCRS) is dedicated to ensuring high-quality pa- tient care by advancing the science, prevention, and These guidelines are built on the last set of the ASCRS T Practice Parameters for the Identification and Testing of management of disorders and diseases of the colon, rectum, Patients at Risk for Dominantly Inherited Colorectal Can- and anus. The Clinical Practice Guidelines Committee is 1 composed of society members who are chosen because they cer published in 2003. An organized search of MEDLINE have demonstrated expertise in the specialty of colon and (1946 to December week 1, 2016) was performed from rectal surgery. This committee was created to lead interna- 1946 through week 4 of September 2016 (Fig. 1). Subject tional efforts in defining quality care for conditions related headings for “adenomatous polyposis coli” (4203 results) to the colon, rectum, and anus, in addition to the devel- and “intestinal polyposis” (445 results) were included, us- opment of Clinical Practice Guidelines based on the best ing focused search. The results were combined (4629 re- available evidence. These guidelines are inclusive and not sults) and limited to English language (3981 results), then prescriptive. -
Rare Pancreatic Tumors
Published online: 2020-04-29 THIEME 64 ReviewRare Pancreatic Article Tumors Choudhari et al. Rare Pancreatic Tumors Amitkumar Choudhari1,2 Pooja Kembhavi1,2 Mukta Ramadwar3,4 Aparna Katdare1,2 Vasundhara Smriti1,2 Akshay D. Baheti1,2 1Department of Radiodiagnosis, Tata Memorial Hospital, Mumbai, Address for correspondence Akshay D. Baheti, MD, Department of Maharashtra, India Radiodiagnosis, Tata Memorial Hospital, Ernest , Borges Marg Parel 2Department of Radiodiagnosis, Homi Bhabha National University, Mumbai 400012, India (e-mail: [email protected]). Mumbai, Maharashtra, India 3Department of Pathology, Tata Memorial Hospital, Mumbai, Maharashtra, India 4Department of Pathology, Homi Bhabha National University, Mumbai, Maharashtra, India J Gastrointestinal Abdominal Radiol ISGAR 2020;3:64–74 Abstract Pancreatic ductal adenocarcinoma, neuroendocrine tumor, and cystic pancreatic neo- plasms are the common pancreatic tumors most radiologists are familiar with. In this Keywords article we review the clinical presentation, pathophysiology, and radiology of rare pan- ► pancreatic cancer creatic neoplasms. While the imaging features are usually nonspecific and diagnosis is ► uncommon based on pathology, the radiology along with patient demographics, history, and lab- ► pancreatoblastoma oratory parameters can often help indicate the diagnosis of an uncommon pancreatic ► acinar cell neoplasm and guide appropriate management in these cases. ► lymphoma Introduction hyperlipasemia may rarely lead to extraabdominal manifes- tations like ectopic subcutaneous fat necrosis and polyarthri- Pancreatic tumors of various histological subtypes can be tis (lipase hypersecretion syndrome).4 encountered in clinical practice, most common being pan- These tumors are hypoenhancing compared with the pan- creatic ductal adenocarcinoma (PDAC), which constitutes creas and are frequently associated with cystic or necrotic 85% of all pancreatic neoplasms.1 Histologically pancreat- areas as well as calcifications5,6 (►Fig. -
Rare Sarcomatoid Carcinoma of the Liver in a Patient with No History of Hepatocellular Carcinoma: a Case Report
Rare sarcomatoid carcinoma of the liver in a patient with no history of hepatocellular carcinoma: a case report Abstract Sarcomatoid carcinoma is a rare malignant tumor of unknown pathogenesis characterized by poorly differentiated carcinoma tissue containing sarcoma-like differentiation of either spindle or giant cell and rarely occurs in the gastrointestinal tract and hepatobiliary-pancreatic system.1 Primary hepatic sarcomatoid carcinoma accounts for only 0.2 % of primary malignant liver tumors, and 1.8% of all surgically resected hepatocellular carcinomas.2 The majority of hepatic sarcomatoid carcinoma cases appear to occur simultaneously with hepatocellular or cholangiocellular carcinoma.3 The preferred treatment for hepatic sarcomatoid carcinoma is surgical resection and the overall prognosis is poor.4 This case depicts a 62-year-old female who underwent initial resection of a cavernous hemangioma in 2010. Seven years after her initial diagnosis, she developed what was initially felt to be local recurrence of the hemangioma but additional diagnostic workup with a liver biopsy confirmed primary hepatic sarcomatoid carcinoma. Keywords Sarcomatoid Carcinoma, Hepatocellular Carcinoma, Primary Hepatic Sarcomatoid Carcinoma Case Report A 62-year-old female with past medical history significant for vegan diet, hypothyroidism, iron deficiency anemia, and cavernous liver hemangioma presented with weight loss and abdominal fullness for approximately one month with two days of acute altered mental status, fatigue, and weakness. Patient had a complicated gastrointestinal history and underwent surgical resection of a cavernous hemangioma in 2010. Six years later, she developed abdominal fullness with right upper quadrant pain and an abdominal ultrasound at that time suggested hemangioma recurrence. In 2017, she underwent laparoscopy with unroofing of the hemangioma, drainage of an old organizing hematoma, removal of debris, and placement of an omental patch. -
Germline Fumarate Hydratase Mutations in Patients with Ovarian Mucinous Cystadenoma
European Journal of Human Genetics (2006) 14, 880–883 & 2006 Nature Publishing Group All rights reserved 1018-4813/06 $30.00 www.nature.com/ejhg SHORT REPORT Germline fumarate hydratase mutations in patients with ovarian mucinous cystadenoma Sanna K Ylisaukko-oja1, Cezary Cybulski2, Rainer Lehtonen1, Maija Kiuru1, Joanna Matyjasik2, Anna Szyman˜ska2, Jolanta Szyman˜ska-Pasternak2, Lars Dyrskjot3, Ralf Butzow4, Torben F Orntoft3, Virpi Launonen1, Jan Lubin˜ski2 and Lauri A Aaltonen*,1 1Department of Medical Genetics, Biomedicum Helsinki, University of Helsinki, Helsinki, Finland; 2International Hereditary Cancer Center, Department of Genetics and Pathology, Pomeranian Medical University, Szczecin, Poland; 3Department of Clinical Biochemistry, Aarhus University Hospital, Skejby, Denmark; 4Pathology and Obstetrics and Gynecology, University of Helsinki, Helsinki, Finland Germline mutations in the fumarate hydratase (FH) gene were recently shown to predispose to the dominantly inherited syndrome, hereditary leiomyomatosis and renal cell cancer (HLRCC). HLRCC is characterized by benign leiomyomas of the skin and the uterus, renal cell carcinoma, and uterine leiomyosarcoma. The aim of this study was to identify new families with FH mutations, and to further examine the tumor spectrum associated with FH mutations. FH germline mutations were screened from 89 patients with RCC, skin leiomyomas or ovarian tumors. Subsequently, 13 ovarian and 48 bladder carcinomas were analyzed for somatic FH mutations. Two patients diagnosed with ovarian mucinous cystadenoma (two out of 33, 6%) were found to be FH germline mutation carriers. One of the changes was a novel mutation (Ala231Thr) and the other one (435insAAA) was previously described in FH deficiency families. These results suggest that benign ovarian tumors may be associated with HLRCC. -
Familial Adenomatous Polyposis Polymnia Galiatsatos, M.D., F.R.C.P.(C),1 and William D
American Journal of Gastroenterology ISSN 0002-9270 C 2006 by Am. Coll. of Gastroenterology doi: 10.1111/j.1572-0241.2006.00375.x Published by Blackwell Publishing CME Familial Adenomatous Polyposis Polymnia Galiatsatos, M.D., F.R.C.P.(C),1 and William D. Foulkes, M.B., Ph.D.2 1Division of Gastroenterology, Department of Medicine, The Sir Mortimer B. Davis Jewish General Hospital, McGill University, Montreal, Quebec, Canada, and 2Program in Cancer Genetics, Departments of Oncology and Human Genetics, McGill University, Montreal, Quebec, Canada Familial adenomatous polyposis (FAP) is an autosomal-dominant colorectal cancer syndrome, caused by a germline mutation in the adenomatous polyposis coli (APC) gene, on chromosome 5q21. It is characterized by hundreds of adenomatous colorectal polyps, with an almost inevitable progression to colorectal cancer at an average age of 35 to 40 yr. Associated features include upper gastrointestinal tract polyps, congenital hypertrophy of the retinal pigment epithelium, desmoid tumors, and other extracolonic malignancies. Gardner syndrome is more of a historical subdivision of FAP, characterized by osteomas, dental anomalies, epidermal cysts, and soft tissue tumors. Other specified variants include Turcot syndrome (associated with central nervous system malignancies) and hereditary desmoid disease. Several genotype–phenotype correlations have been observed. Attenuated FAP is a phenotypically distinct entity, presenting with fewer than 100 adenomas. Multiple colorectal adenomas can also be caused by mutations in the human MutY homologue (MYH) gene, in an autosomal recessive condition referred to as MYH associated polyposis (MAP). Endoscopic screening of FAP probands and relatives is advocated as early as the ages of 10–12 yr, with the objective of reducing the occurrence of colorectal cancer. -
1 Effective January 1, 2018 ICD‐O‐3 Codes, Behaviors and Terms Are Site‐Specific Alpha Order Last Updat
Effective January 1, 2018 ICD‐O‐3 codes, behaviors and terms are site‐specific Alpha Order Last updated 8/22/18 Status ICD‐O‐3 Term Reportable Comments Morphology Y/N Code New Term 8551/3 Acinar adenocarcinoma (C34. _) Y Lung primaries diagnosed prior to 1/1/2018 use code 8550/3 For prostate (all years) see 8140/3 New Term 8140/3 Acinar adenocarcinoma (C61.9 ONLY) Y For prostate only, do not use 8550/3 New Term 8572/3 Acinar adenocarcinoma, sarcomatoid (C61.9) Y New Term 8550/3 Acinar cell carcinoma Y Excludes C61.9‐ see 8140/3 New Term 8316/3 Acquired cystic disease‐associated renal cell carcinoma (RCC) Y (C64.9) New 8158/1 ACTH‐producing tumor N code/term New Term 8574/3 Adenocarcinoma admixed with neuroendocrine carcinoma (C53. _) Y Behavior 8253/2 Adenocarcinoma in situ, mucinous (C34. _) Y Important note: lung Code/term primaries ONLY: For cases diagnosed 1/1/2018 forward do not use code 8480 (mucinous adenocarcinoma) for in‐ situ adenocarcinoma, mucinous or invasive mucinous adenocarcinoma. 1 Status ICD‐O‐3 Term Reportable Comments Morphology Y/N Code Behavior 8250/2 Adenocarcinoma in situ, non‐mucinous (C34. _) Y code/term New Term 9110/3 Adenocarcinoma of rete ovarii (C56.9) Y New 8163/3 Adenocarcinoma, pancreatobiliary‐type (C24.1) Y Cases diagnosed prior to code/term 1/1/2018 use code 8255/3 Behavior 8983/3 Adenomyoepithelioma with carcinoma (C50. _) Y Code/term New Term 8620/3 Adult granulosa cell tumor (C56.9 ONLY) N Not reportable for 2018 cases New Term 9401/3 Anaplastic astrocytoma, IDH‐mutant (C71. -
Risk Factors of Esophageal Squamous Cell Carcinoma Beyond Alcohol and Smoking
cancers Review Risk Factors of Esophageal Squamous Cell Carcinoma beyond Alcohol and Smoking Munir Tarazi 1 , Swathikan Chidambaram 1 and Sheraz R. Markar 1,2,* 1 Department of Surgery and Cancer, Imperial College London, London W2 1NY, UK; [email protected] (M.T.); [email protected] (S.C.) 2 Department of Molecular Medicine and Surgery, Karolinska Institutet, Karolinska University Hospital, 17164 Stockholm, Sweden * Correspondence: [email protected] Simple Summary: Esophageal squamous cell carcinoma (ESCC) is the sixth most common cause of death worldwide. Incidence rates vary internationally, with the highest rates found in Southern and Eastern Africa, and central Asia. Initial studies identified multiple factors associated with an increased risk of ESCC, with subsequent work then focused on developing plausible biological mechanistic associations. The aim of this review is to summarize the role of risk factors in the development of ESCC and propose future directions for further research. A systematic literature search was conducted to identify risk factors associated with the development of ESCC. Risk factors were divided into seven subcategories: genetic, dietary and nutrition, gastric atrophy, infection and microbiome, metabolic, epidemiological and environmental, and other risk factors. Risk factors from each subcategory were summarized and explored. This review highlights several current risk factors of ESCC. Further research to validate these results and their effects on tumor biology is necessary. Citation: Tarazi, M.; Chidambaram, S.; Markar, S.R. Risk Factors of Abstract: Esophageal squamous cell carcinoma (ESCC) is the sixth most common cause of death Esophageal Squamous Cell worldwide. Incidence rates vary internationally, with the highest rates found in Southern and Eastern Carcinoma beyond Alcohol and Africa, and central Asia. -
Squamous Cell Carcinoma of the Renal Parenchyma
Zhang et al. BMC Urology (2020) 20:107 https://doi.org/10.1186/s12894-020-00676-5 CASE REPORT Open Access Squamous cell carcinoma of the renal parenchyma presenting as hydronephrosis: a case report and review of the recent literature Xirong Zhang1,2, Yuanfeng Zhang1, Chengguo Ge1, Junyong Zhang1 and Peihe Liang1* Abstract Background: Primary squamous cell carcinoma of the renal parenchyma is extremely rare, only 5 cases were reported. Case presentation: We probably report the fifth case of primary Squamous cell carcinoma (SCC) of the renal parenchyma in a 61-year-old female presenting with intermittent distending pain for 2 months. Contrast-enhanced computed tomography (CECT) revealed hydronephrosis of the right kidney, but a tumor cannot be excluded completely. Finally, nephrectomy was performed, and histological analysis determined that the diagnosis was kidney parenchyma squamous cell carcinoma involving perinephric adipose tissue. Conclusions: The present case emphasizes that it is difficult to make an accurate preoperative diagnosis with the presentation of hidden malignancy, such as hydronephrosis. Keywords: Kidney, Renal parenchyma, Squamous cell carcinoma, Hydronephrosis, Malignancy Background Case presentation Squamous cell carcinoma (SCC) of the renal pelvis is a The patient is a 61-year-old female. After suffering from rare neoplasm, accounting for only 0.5 to 0.8% of malig- intermittent pain in the right flank region for 2 months nant renal tumors [1], SCC of the renal parenchyma is she was referred to the urology department at an outside even less common. A review of the literature shows that hospital. The patient was diagnosed with hydronephrosis only five cases of primary SCC of the renal parenchyma of the right kidney and underwent a right ureteroscopy have been reported to date [2–6]. -
Squamous Cell Carcinoma of Pancreas with High PD-L1 Expression: a Rare Presentation
CASE REPORT published: 01 July 2021 doi: 10.3389/fonc.2021.680398 Case Report: Squamous Cell Carcinoma of Pancreas With High PD-L1 Expression: A Rare Presentation Baohong Yang, Haipeng Ren* and Guohua Yu Oncology Department, Weifang People’s Hospital, Weifang Medical University, Weifang, China Primary pancreatic squamous cell carcinoma is sporadic. The diagnosis is usually made following surgery or needle biopsy and requires a thorough workup to exclude metastatic squamous cell carcinoma. Squamous cell carcinoma of the pancreas often has a very poor prognosis. There is no treatment guideline for this type of cancer, and to date, no therapeutic regimen has been proven effective. Here, we report the effectiveness of Edited by: immunotherapy in combination with chemotherapy against locally advanced squamous Lujun Chen, First People’s Hospital of Changzhou, cell carcinoma of the pancreas with high programmed cell death ligand 1 (PD-L1) China expression. Regional intra-arterial infusion chemotherapy consisting of nab-Paclitaxel Reviewed by: followed by gemcitabine infused via gastroduodenal artery every three weeks for two Helmout Modjathedi, Kingston University, United Kingdom cycles. This therapy resulted in the depletion of carcinoma, and the patient continues to Saikiran Raghavapuram, lead a high-quality life with no symptoms for more than 16 months. South Georgia Medical Center, United States Keywords: pancreas cancer, immunotherapy, chemotherapy, squamous cell carcinoma, anti-PD 1 *Correspondence: Haipeng Ren [email protected] STUDY HIGHLIGHT Specialty section: This article was submitted to 1. Regional intra-arterial infusion chemotherapy and partial splenic embolization is administered Cancer Immunity along with Immunotherapy targeting PD-L1 with gemcitabine to achieve partial remission of a and Immunotherapy, primary locally advanced squamous cell carcinoma of the pancreas with a high level of PD-L1 a section of the journal expression.