Evicore Oncology Imaging Guidelines
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Contents 1 Tumours of the pituitary gland 11 Spindle epithelial tumour with thymus-like differentiation 123 WHO classifi cation of tumours of the pituitary 12 Intrathyroid thymic carcinoma 125 Introduction 13 Paraganglioma and mesenchymal / stromal tumours 127 Pituitary adenoma 14 Paraganglioma 127 Somatotroph adenoma 19 Peripheral nerve sheath tumours 128 Lactotroph adenoma 24 Benign vascular tumours 129 Thyrotroph adenoma 28 Angiosarcoma 129 Corticotroph adenoma 30 Smooth muscle tumours 132 Gonadotroph adenoma 34 Solitary fi brous tumour 133 Null cell adenoma 37 Haematolymphoid tumours 135 Plurihormonal and double adenomas 39 Langerhans cell histiocytosis 135 Pituitary carcinoma 41 Rosai–Dorfman disease 136 Pituitary blastoma 45 Follicular dendritic cell sarcoma 136 Craniopharyngioma 46 Primary thyroid lymphoma 137 Neuronal and paraneuronal tumours 48 Germ cell tumours 139 Gangliocytoma and mixed gangliocytoma–adenoma 48 Secondary tumours 142 Neurocytoma 49 Paraganglioma 50 3 Tumours of the parathyroid glands 145 Neuroblastoma 51 WHO classifi cation of tumours of the parathyroid glands 146 Tumours of the posterior pituitary 52 TNM staging of tumours of the parathyroid glands 146 Mesenchymal and stromal tumours 55 Parathyroid carcinoma 147 Meningioma 55 Parathyroid adenoma 153 Schwannoma 56 Secondary, mesenchymal and other tumours 159 Chordoma 57 Haemangiopericytoma / Solitary fi brous tumour 58 4 Tumours of the adrenal cortex 161 Haematolymphoid tumours 60 WHO classifi cation of tumours of the adrenal cortex 162 Germ cell tumours 61 TNM classifi -
A Rare Case of Urachal Adenocarcinoma with Bone Marrow Metastasis Joshua Van Allen1,2
Case report BMJ Case Rep: first published as 10.1136/bcr-2021-242315 on 15 April 2021. Downloaded from A rare case of urachal adenocarcinoma with bone marrow metastasis Joshua Van Allen1,2 1Department of Medicine, SUMMARY non-urachal adenocarcinomas whose median age is University of Connecticut Health Urachal cancer is a rare and aggressive cancer that often around 69 years old.10 14 Urachal cancer also displays Center, Farmington, Connecticut, presents in advanced stages. Given the rarity of this male predilection, with males representing between USA 7 8 2 malignancy, medical case studies provide one of the few 60% and 68% of all cases. Hartford HealthCare Cancer Patients with urachal cancer often present Institute at The Hospital of sources of literature available through which clinicians with locally advanced or metastatic disease, most Central Connecticut, Plainville, can guide medical management. Surgery is widely Connecticut, USA considered to be the mainstay of therapy when disease commonly metastatic to the lungs, liver, perito- is localised and surgically resectable, therefore most neum, lymph nodes, brain and/or bone.6 8 12 13 Correspondence to current case studies on urachal cancer focus on surgical Given the aggressive nature of urachal cancer, early Dr. Joshua Van Allen; management, occasionally with adjuvant chemotherapy. diagnosis is paramount as there is significant diver- jvanallen@ uchc. edu However, few case studies discuss chemotherapy alone gence in both treatment and prognosis depending in the treatment of metastatic disease. Most studies on stage. A retrospective study performed by Accepted 6 April 2021 indicate a median overall survival between 12 and 24 Mayo Clinic in Rochester, Minnesota evaluated 49 months for metastatic urachal adenocarcinoma. -
Mutations of KRAS, NRAS, BRAF, EGFR, and PIK3CA Genes in Urachal Carcinoma: Occurence and Prognostic Significance
View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Jagiellonian Univeristy Repository www.impactjournals.com/oncotarget/ Oncotarget, Vol. 7, No. 26 Research Paper: Pathology Mutations of KRAS, NRAS, BRAF, EGFR, and PIK3CA genes in urachal carcinoma: Occurence and prognostic significance Orsolya Módos1, Henning Reis2, Christian Niedworok3, Herbert Rübben3, Attila Szendröi1, Marcell A. Szász4, József Tímár4, Kornélia Baghy5, Ilona Kovalszky5, Tomasz Golabek6, Piotr Chlosta6, Krzysztof Okon7, Benoit Peyronnet8, Romain Mathieu8, Shahrokh F. Shariat9, Péter Hollósi5,10, Péter Nyirády1 and Tibor Szarvas1,3 1 Department of Urology, Semmelweis University, Budapest, Hungary 2 Institute of Pathology, University of Duisburg-Essen, Essen, Germany 3 Department of Urology, University of Duisburg-Essen, Essen, Germany 4 2nd Department of Pathology, Semmelweis University, Budapest, Hungary 5 1st Department of Pathology and Experimental Cancer Research, Semmelweis University, Budapest, Hungary 6 Department of Urology, Jagiellonian University, Krakow, Poland 7 Department of Pathomorphology, Jagiellonian University, Krakow, Poland 8 Department of Urology, Rennes University Hospital, Rennes, France 9 Department of Urology, Medical University of Vienna, Vienna General Hospital, Vienna, Austria 10 Tumor Progression Research Group, Hungarian Academy of Sciences, Budapest, Hungary Correspondence to: Tibor Szarvas, email: [email protected] Keywords: urachal carcinoma, urachal cancer, urachus, mutation, EGFR, Pathology Section Received: April 08, 2016 Accepted: May 29, 2016 Published: June 05, 2016 ABSTRACT Purpose: Targeted therapy represents an attractive alternative for rare tumors such as urachal carcinoma (UrC). The aim of this study was to assess the mutations of the most commonly affected 5 genes in the targetable EGFR-pathway in UrC and comapre their frequencies to those of found in urothelial and colorectal cancer. -
Multiple Endocrine Neoplasia Type 1 (MEN1)
Lab Management Guidelines V1.0.2020 Multiple Endocrine Neoplasia Type 1 (MEN1) MOL.TS.285.A v1.0.2020 Introduction Multiple Endocrine Neoplasia Type 1 (MEN1) is addressed by this guideline. Procedures addressed The inclusion of any procedure code in this table does not imply that the code is under management or requires prior authorization. Refer to the specific Health Plan's procedure code list for management requirements. Procedures addressed by this Procedure codes guideline MEN1 Known Familial Mutation Analysis 81403 MEN1 Deletion/Duplication Analysis 81404 MEN1 Full Gene Sequencing 81405 What is Multiple Endocrine Neoplasia Type 1 Definition Multiple Endocrine Neoplasia Type 1 (MEN1) is an autosomal dominant disorder characterized by the development of multiple endocrine and non-endrocrine tumors. Incidence or Prevalence MEN1 has a prevalence of 1/10,000 to 1/100,000 individuals.1 Symptoms The presenting symptom in approximately 90% of individuals with MEN1 is primary hyperparathyroidism (PHPT). Parathyroid tumors cause overproduction of parathyroid hormone which leads to hypercalcemia. The average age of onset is 20-25 years. Parathyroid carcinomas are rare in individuals with MEN1.2,3,4 Pituitary tumors are seen in 30-40% of individuals and are the first clinical manifestation in 10% of familial cases and 25% of simplex cases. Tumors are typically solitary and there is no increased prevalence of pituitary carcinoma in individuals with MEN1.2,5 © 2020 eviCore healthcare. All Rights Reserved. 1 of 8 400 Buckwalter Place Boulevard, Bluffton, SC 29910 (800) 918-8924 www.eviCore.com Lab Management Guidelines V1.0.2020 Prolactinomas are the most commonly seen pituitary subtype and account for 60% of pituitary adenomas. -
Ganglioneuroblastoma As Vasoactive Intestinal Polypeptide-Secreting10.5005/Jp-Journals-10002-1167 Tumor: Rare Case Report in a Child Case Report
WJOES Ganglioneuroblastoma as Vasoactive Intestinal Polypeptide-secreting10.5005/jp-journals-10002-1167 Tumor: Rare Case Report in a Child CASE REPORT Ganglioneuroblastoma as Vasoactive Intestinal Polypeptide-secreting Tumor: Rare Case Report in a Child 1Basant Kumar, 2Vijai D Upadhyaya, 3Ram Nawal Rao, 4Sheo Kumar ABSTRACT than 60 cases of pediatric VIP-secreting tumors.3 Most Pathologically elevated vasoactive intestinal polypeptide (VIP) of them are either adrenal pheochromocytoma or mixed plasma levels cause secretory diarrhea with excessive loss of pheochromocytoma-ganglioneuroma tumors. Mason water and electrolyte and is characterized by the typical symp- et al,4 first described the secretory nature of neuroblas- toms of hypokalemia and metabolic acidosis. It rarely occurs toma and vasoactive intestinal peptide (VIP) can be in patients with non-pancreatic disease. Despite the clinical severity, diagnosis of a VIP-secreting tumor is often delayed. produced by the mature neurogenic tumors. We herein We herein present a 14-month-old boy having prolonged present a 14 months old boy having prolonged therapy- therapy-resistant secretory diarrhea, persistent hypokalemia resistant secretory diarrhea, persistent hypokalemia with with tissue diagnosis of ganglioneuroblastoma and raised plasma VIP-levels. tissue diagnosis of ganglioneuroblastoma and briefly review the literature. Keywords: Ganglioneuroblastoma, Secretory diarrhea, VIPoma. CASE REPORT How to cite this article: Kumar B, Upadhyaya VD, Rao RN, Kumar S. Ganglioneuroblastoma as Vasoactive Intestinal A 14-month-old boy, weighing 9 kg with advanced symp- Polypeptide-secreting Tumor: Rare Case Report in a Child. World J Endoc Surg 2015;7(2):47-50. toms of persistent secretory diarrhea, hypokalemia and metabolic acidosis was referred to us with radiological Source of support: Nil (computed tomography) diagnosis of retroperitoneal Conflict of interest: None mass. -
Neuroendocrine Tumors of the Pancreas (Including Insulinoma, Gastrinoma, Glucogacoma, Vipoma, Somatostatinoma)
Neuroendocrine tumors of the pancreas (including insulinoma, gastrinoma, glucogacoma, VIPoma, somatostatinoma) Neuroendocrine pancreatic tumors (pancreatic NETs or pNETs) account for about 3% of all primary pancreatic tumors. They develop in neuroendocrine cells called islet cells. Neuroendocrine tumors of the pancreas may be nonfunctional (not producing hormones) or functional (producing hormones). Most pNETs do not produce hormones and, as a result, these tumors are diagnosed incidentally or after their growth causes symptoms such as abdominal pain, jaundice or liver metastasis. pNETs that produce hormones are named according to the type of hormone they produce and / or clinical manifestation: Insulinoma - An endocrine tumor originating from pancreatic beta cells that secrete insulin. Increased insulin levels in the blood cause low glucose levels in blood (hypoglycemia) with symptoms that may include sweating, palpitations, tremor, paleness, and later unconsciousness if treatment is delayed. These are usually benign and tend to be small and difficult to localize. Gastrinoma - a tumor that secretes a hormone called gastrin, which causes excess of acid secretion in the stomach. As a result, severe ulcerative disease and diarrhea may develop. Most gastrinomas develop in parts of the digestive tract that includes the duodenum and the pancreas, called "gastrinoma triangle". These tumors have the potential to be malignant. Glucagonoma is a rare tumor that secretes the hormone glucagon, which may cause a typical skin rash called migratory necrolytic erythema, elevated glucose levels, weight loss, diarrhea and thrombotic events. VIPoma - a tumor that secretes Vasoactive peptide (VIP) hormone causing severe diarrhea. The diagnosis is made by finding a pancreatic neuroendocrine tumor with elevated VIP hormone in the blood and typical clinical symptoms. -
UEMS 2020.11 Syllabus of the ETR in Rare Adult Cancers
UNION EUROPÉENNE DES MÉDECINS SPÉCIALISTES EUROPEAN UNION OF MEDICAL SPECIALISTS Association internationale sans but lucratif International non-profit organisation RUE DE L’INDUSTRIE, 24 T +32 2 649 51 64 BE- 1040 BRUSSELS F +32 2 640 37 30 www.uems.eu [email protected] UEMS 2020.11 Syllabus for residents and trainees in Rare Adult Solid Cancers The basic goal of this syllabus is to provide an understanding between the instructor and trainee so there is minimal confusion in the topics, with clear expectations. It is not a classical syllabus as it contains descriptions from different areas, but it still summarizes major and specific topics that should be covered during the training course of a resident. This syllabus is intended as supporting reference material, and the precise content and priorities of training may vary in different training institutions. The syllabus can also be modified to reflect each instructor's teaching philosophy towards the trainees. 1. There are scientific publications, web pages, and conference materials available online that could be used for educational purposes for various types of rare adult solid cancers. This is a comprehensive summary of them. 2. There are significant differences in the number of available scientific publications and reviews for different rare adult solid cancers. Some, like sarcomas, have a very robust literature, while others have been sparsely researched and consequently the availability of study materials is quite poor. 3. These differences also apply to life events and natural history. In the list of the EU CE accredited events there is a strong underrepresentation for some types of rare adult solid cancers. -
Multiple Endocrine Neoplasia Type 1 (MEN1)
Lab Management Guidelines v2.0.2019 Multiple Endocrine Neoplasia Type 1 (MEN1) MOL.TS.285.A v2.0.2019 Introduction Multiple Endocrine Neoplasia Type 1 (MEN1) is addressed by this guideline. Procedures addressed The inclusion of any procedure code in this table does not imply that the code is under management or requires prior authorization. Refer to the specific Health Plan's procedure code list for management requirements. Procedures addressed by this Procedure codes guideline MEN1 Known Familial Mutation Analysis 81403 MEN1 Deletion/Duplication Analysis 81404 MEN1 Full Gene Sequencing 81405 What is Multiple Endocrine Neoplasia Type 1 Definition Multiple Endocrine Neoplasia Type 1 (MEN1) is an inherited form of tumor predisposition characterized by multiple tumors of the endocrine system. Incidence or Prevalence MEN1 has a prevalence of 1/10,000 to 1/100,000 individuals.1 Symptoms The presenting symptom in 90% of individuals with MEN1 is primary hyperparathyroidism (PHPT). Parathyroid tumors cause overproduction of parathyroid hormone which leads to hypercalcemia. The average age of onset is 20-25 years. Parathyroid carcinomas are rare in individuals with MEN1.2,3,4 Pituitary tumors are seen in 30-40% of individuals and are the first clinical manifestation in 10% of familial cases and 25% of simplex cases. Tumors are typically solitary and there is no increased prevalence of pituitary carcinoma in individuals with MEN1.2,5 © eviCore healthcare. All Rights Reserved. 1 of 9 400 Buckwalter Place Boulevard, Bluffton, SC 29910 (800) 918-8924 www.eviCore.com Lab Management Guidelines v2.0.2019 Prolactinomas are the most commonly seen pituitary subtype and account for 60% of pituitary adenomas. -
Rising Incidence of Neuroendocrine Tumors
Rising Incidence of Neuroendocrine Tumors Dasari V, Yao J, et al. JAMA Oncology 2017 S L I D E 1 Overview Pancreatic Neuroendocrine Tumors • Tumors which arise from endocrine cells of the pancreas • 5.6 cases per million – 3% of pancreatic tumors • Median age at diagnosis 60 years • More indolent course compared to adenocarcinoma – 10-year overall survival 40% • Usually sporadic but can be associated with hereditary syndromes – Core genetic pathways altered in sporadic cases • DNA damage repair (MUTYH) Chromatin remodeling (MEN1) • Telomere maintenance (MEN1, DAXX, ATRX) mTOR signaling – Hereditary: 17% of patients with germline mutation Li X, Wang C, et al. Cancer Med 2018 Scarpa A, Grimond S, et al. NatureS L I2017 D E 2 Pathology Classification European American Joint World Health Organization Neuroendocrine Committee on Cancer (WHO) Tumor Society (AJCC) (ENETS) Grade Ki-67 Mitotic rt TNM TNM T1: limit to pancreas, <2 cm T1: limit to pancreas, ≦2 cm T2: limit to pancreas, 2-4 cm T2: >limit to pancreas, 2 cm T3: limit to pancreas, >4 cm, T3: beyond pancreas, no celiac or Low ≤2% <2 invades duodenum, bile duct SMA T4: beyond pancreas, invasion involvement adjacent organs or vessels T4: involves celiac or SMA N0: node negative No: node negative Intermed 3-20% 2-20 N1: node positive N1: node positive M0: no metastases M0: no metastases High >20% >20 M1: metastases M1: metastases S L I D E 3 Classification Based on Functionality • Nonfunctioning tumors – No clinical symptoms (can still produce hormone) – Accounts for 40% of tumors – 60-85% -
New Jersey State Cancer Registry List of Reportable Diseases and Conditions Effective Date March 10, 2011; Revised March 2019
New Jersey State Cancer Registry List of reportable diseases and conditions Effective date March 10, 2011; Revised March 2019 General Rules for Reportability (a) If a diagnosis includes any of the following words, every New Jersey health care facility, physician, dentist, other health care provider or independent clinical laboratory shall report the case to the Department in accordance with the provisions of N.J.A.C. 8:57A. Cancer; Carcinoma; Adenocarcinoma; Carcinoid tumor; Leukemia; Lymphoma; Malignant; and/or Sarcoma (b) Every New Jersey health care facility, physician, dentist, other health care provider or independent clinical laboratory shall report any case having a diagnosis listed at (g) below and which contains any of the following terms in the final diagnosis to the Department in accordance with the provisions of N.J.A.C. 8:57A. Apparent(ly); Appears; Compatible/Compatible with; Consistent with; Favors; Malignant appearing; Most likely; Presumed; Probable; Suspect(ed); Suspicious (for); and/or Typical (of) (c) Basal cell carcinomas and squamous cell carcinomas of the skin are NOT reportable, except when they are diagnosed in the labia, clitoris, vulva, prepuce, penis or scrotum. (d) Carcinoma in situ of the cervix and/or cervical squamous intraepithelial neoplasia III (CIN III) are NOT reportable. (e) Insofar as soft tissue tumors can arise in almost any body site, the primary site of the soft tissue tumor shall also be examined for any questionable neoplasm. NJSCR REPORTABILITY LIST – 2019 1 (f) If any uncertainty regarding the reporting of a particular case exists, the health care facility, physician, dentist, other health care provider or independent clinical laboratory shall contact the Department for guidance at (609) 633‐0500 or view information on the following website http://www.nj.gov/health/ces/njscr.shtml. -
Multiple Endocrine Neoplasia Type 1 (MEN1)
Page 1 of 2 Multiple Endocrine Neoplasia Type 1 (MEN1) MEN1 is an autosomal dominant syndrome caused by germline mutations in the MEN1 gene. Endocrine tumours come to attention because of the overproduction of hormones and/or tumour growth. A clinical MEN1 diagnosis requires the diagnosis of 2 endocrine tumours in the parathyroid, pituitary and/or gastro-entero-pancreatic (GEP) tract. MEN1 is also associated with a number of other endocrine (e.g. carcinoid, adrenocortical) and non-endocrine tumours (e.g. facial angiofibromas, collagenomas, lipomas, meningiomas, ependymomas, leiomyomas) in some families. MEN2 is a separate syndrome with medullary thyroid cancer and pheochromocytoma as key features. Referral Criteria Note: close relatives include: children, brothers, sisters, parents, aunts, uncles, grandchildren & grandparents on the same side of the family . History of cancer in cousins and more distant relatives from the same side of the family may also be relevant. • family member with a confirmed MEN1 g ene mutation – refer for carrier testing • a person with 2 or more of the 3 key MEN 1-associated tumours: o parathyroid tumour or hyperplasia (primary hyperparathyroidism) o pituitary adenoma (prolactinoma is the most common) o well-differentiated gastro-entero-pancreatic neuroendocrine tumour (e.g. gastrinoma, insulinoma, glucagonoma, pancreatic islet tumour, VIPoma) • a person with gastro-entero-pancreatic NET (neuroendocrine tumour) before age 40 • a person with parathyroid tumour or hyperplasia before age 40 • a person with primary hyperparathyroidism and a close relative with the same diagnosis • a person with features described above and close relative(s) with related tumours • a person with a close relative with features described above • a person with additional endocrine and non-endocrine features associated with MEN1 may be referred for assessment Referral of children is appropriate for this syndrome because it may inform their medical management. -
Clinical Presentation and Diagnosis of Pancreatic Neuroendocrine Tumors
Clinical Presentation and Diagnosis of Pancreatic Neuroendocrine Tumors Carinne W. Anderson, MD*, Joseph J. Bennett, MD KEYWORDS Pancreatic neuroendocrine tumor Nonfunctional pancreatic neuroendocrine tumor Insulinoma Gastrinoma Glucagonoma VIPoma Somatostatinoma KEY POINTS Pancreatic neuroendocrine tumors are a rare group of neoplasms, most of which are nonfunctioning. Functional pancreatic neoplasms secrete hormones that produce unique clinical syndromes. The key management of these rare tumors is to first suspect the diagnosis; to do this, cli- nicians must be familiar with their clinical syndromes. Pancreatic neuroendocrine tumors (PNETs) are a rare group of neoplasms that arise from multipotent stem cells in the pancreatic ductal epithelium. Most PNETs are nonfunctioning, but they can secrete various hormones resulting in unique clinical syn- dromes. Clinicians must be aware of the diverse manifestations of this disease, as the key step to management of these rare tumors is to first suspect the diagnosis. In light of that, this article focuses on the clinical features of different PNETs. Surgical and medical management will not be discussed here, as they are addressed in other arti- cles in this issue. EPIDEMIOLOGY Classification PNETs are classified clinically as nonfunctional or functional, based on the properties of the hormones they secrete and their ability to produce a clinical syndrome. Nonfunctional PNETs (NF-PNETs) do not produce a clinical syndrome simply because they do not secrete hormones or because the hormones that are secreted do not The authors have nothing to disclose. Department of Surgery, Helen F. Graham Cancer Center, 4701 Ogletown-Stanton Road, S-4000, Newark, DE 19713, USA * Corresponding author. E-mail address: [email protected] Surg Oncol Clin N Am 25 (2016) 363–374 http://dx.doi.org/10.1016/j.soc.2015.12.003 surgonc.theclinics.com 1055-3207/16/$ – see front matter Ó 2016 Elsevier Inc.