Benign Brain Tumors
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Malignant Glioma Arising at the Site of an Excised Cerebellar Hemangioblastoma After Irradiation in a Von Hippel-Lindau Disease Patient
DOI 10.3349/ymj.2009.50.4.576 Case Report pISSN: 0513-5796, eISSN: 1976-2437 Yonsei Med J 50(4): 576-581, 2009 Malignant Glioma Arising at the Site of an Excised Cerebellar Hemangioblastoma after Irradiation in a von Hippel-Lindau Disease Patient Na-Hye Myong1 and Bong-Jin Park2 1Department of Pathology, Dankook University College of Medicine, Cheonan; 2Department of Neurosurgery, Kyunghee University Hospital, Seoul, Korea. We describe herein a malignant glioma arising at the site of the resected hemangioblastoma after irradiation in a patient with von Hippel-Lindau disease (VHL). The patient was a 25 year-old male with multiple heman- gioblastomas at the cerebellum and spinal cord, multiple pancreatic cysts and a renal cell carcinoma; he was diagnosed as having VHL disease. The largest hemangioblastoma at the right cerebellar hemisphere was completely removed, and he received high-dose irradiation postoperatively. The tumor recurred at the same site 7 years later, which was a malignant glioma with no evidence of hemangioblastoma. The malignant glioma showed molecular genetic profiles of radiation-induced tumors because of its diffuse p53 immunostaining and the loss of p16 immunoreactivity. The genetic study to find the loss of heterozygosity (LOH) of VHL gene revealed that only the cerebellar hemangioblastoma showed allelic losses for the gene. To the best of our knowledge, this report is the first to show a malignant glioma that developed in a patient with VHL disease after radiation therapy at the site of an excised hemangioblastoma. This report also suggests that radiation therapy should be performed very carefully in VHL patients with hemangioblastomas. -
INTRINSIC FACTORS in the ETIOLOGY of NEOPLASMS' It Is
INTRINSIC FACTORS IN THE ETIOLOGY OF NEOPLASMS' CARL V. WELLER, MS., M.D. (From the Department of Pathology, University of Michigan) It is as true as it is trite that the cause of disease is never a single factor. Two elements always enter into etiology. One of these is inherent in the germ plasm of the individual; the other brought to bear upon the organism from beyond the confines of the germinal elements from which it has devel- oped. Better than internal and external, endogenous and exogenous, or con- stitutional and environmental, to designate these two groups of factors, are the terms intrinsic and extrinsic. Practical experience directs us to seek the relative importance of intrinsic and extrinsic factors whenever the causation of disease is studied, and we find that the two ingredients may be combined in every possible proportion. At one end of the series are diseases such as deaf mutism, transmitted as a simple recessive, in which the intrinsic element almost crowds out all other considerations. In the recessively sex-linked hemophilia, again the intrinsic element is so prominent that we see at once the mendelian significance of the process, although the bleeder may not be made known until the extrinsic factor, trauma, opens his blood vessels. The asthenic, dolichomorphic bodily configuration, which marks the phthisical habitus, is a familial character and therefore intrinsic, but it conditions infection by the tubercle bacillus in such a way as to give it serious or even lethal possibilities. When we consider syphilis, we find that the Treponema is no respecter of genetic constitution, yet sex and race may modify the character of the disease. -
Current Diagnostic and Therapeutic Strategies in Treatment of CNS Hemangioblastomas in Patients with VHL
Journal of Central Translational Medicine & Epidemiology Special Issue on von Hippel Lindau Disease Edited by: Hiroshi Kanno Professor, Department of Neurosurgery, Yokohama City University School of Medicine, Japan Review Article *Corresponding author Sven Gläsker, Department of Neurosurgery, Freiburg University Medical Center, Breisacher Str. 64, D-79106, Current Diagnostic and Freiburg, Germany, Tel: 49(0)761-270-50010; Fax: 49(0)761-270-50080; Email: Therapeutic Strategies Submitted: 11 November 2013 Accepted: 03 January 2014 in Treatment of CNS Published: 06 January 2014 Copyright Hemangioblastomas in Patients © 2014 Gläsker et al. OPEN ACCESS with VHL Keywords • Hemangioblastoma Marie T. Krüger1, Jan-Helge Klingler1, Christine Steiert1, Cordula • von Hippel-Lindau disease Jilg2, Stefan Zschiedrich3, Birke Bausch4, Vera Van Velthoven1 and • Surgical treatment • Diagnosis; Follow-up Sven Gläsker1* 1Department of Neurosurgery, Freiburg University Medical Center, Germany 2Department of Urology, Freiburg University Medical Center, Germany 3Department of Internal Medicine, Freiburg University Medical Center, Germany 42nd Department of Internal Medicine, Freiburg University Medical Center, Germany Abstract Hemangioblastomas are a rare form of benign vascular tumors of the CNS. They can occur sporadically or as component of the von Hippel-Lindau (VHL) disease - an autosomal dominant tumor syndrome. The tumors are typically located in the posterior fossa and spinal cord. Patients with associated VHL disease are usually affected at an early age and develop multiple lesions. Therefore they need a special routine for diagnosis, treatment and follow-up strategies. In modern neurosurgery, hemangioblastomas are well resectable tumors. Symptomatic lesions should be removed. Resection should furthermore be considered for asymptomatic progressive tumors for the following reason: If a tumor has already caused neurological deficits, the chance to reverse these by surgical resection is reduced and surgical resection is usually possible with low morbidity. -
Updates in Molecular Pathology of Central Nervous System Gliomas in Adults
UPDATES IN ONCOLOGY: PART 2 Updates in Molecular Pathology of Central Nervous System Gliomas in Adults MICHAEL PUNSONI, MD; JOHN E. DONAHUE, MD; HEINRICH D. ELINZANO, MD; TIMOTHY KINSELLA, MD 17 19 EN ABSTRACT Epidemiology Central nervous system (CNS) tumors are a heteroge- The incidence of CNS malignancies has been increasing. neous group of neoplasms divided into two broad cate- Most commonly, CNS tumors arise from glial cells, partic- gories, glial and non-glial. Non-glial tumors are derived ularly astrocytes and oligodendrocytes. Gliomas account from such diverse structures as the pineal gland, menin- for approximately 77% of primary malignant brain tumors ges, germ cells, and hematopoietic cells, as well as metas- (3). Most patients present between the fifth and seventh tases. Primary glial neoplasms, or those which originate decade of life. High-grade tumors are more common than from astrocytes, oligodendrocytes, or ependymal cells, low-grade and present a high risk of morbidity and mortal- include astrocytomas, oligodendrogliomas, ependymo- ity. The World Health Organization (WHO) in 2000 formu- mas, and mixed gliomas. Each entity has a unique mor- lated a classification system based on histologic findings phology and pattern of biologic behavior which portends that is used to stratify brain tumors into prognostic grades. a distinct prognosis and outcome. Individual outcomes High-grade gliomas (WHO grade III and IV) are most com- show some variability based on tumor location and age of monly seen in middle-aged to older adults, while grade II symptom onset; however, the underlying aggressiveness astrocytomas mainly affect younger adults. Management for of the tumor often dictates the time course of the disease. -
Central Nervous System Tumors General ~1% of Tumors in Adults, but ~25% of Malignancies in Children (Only 2Nd to Leukemia)
Last updated: 3/4/2021 Prepared by Kurt Schaberg Central Nervous System Tumors General ~1% of tumors in adults, but ~25% of malignancies in children (only 2nd to leukemia). Significant increase in incidence in primary brain tumors in elderly. Metastases to the brain far outnumber primary CNS tumors→ multiple cerebral tumors. One can develop a very good DDX by just location, age, and imaging. Differential Diagnosis by clinical information: Location Pediatric/Young Adult Older Adult Cerebral/ Ganglioglioma, DNET, PXA, Glioblastoma Multiforme (GBM) Supratentorial Ependymoma, AT/RT Infiltrating Astrocytoma (grades II-III), CNS Embryonal Neoplasms Oligodendroglioma, Metastases, Lymphoma, Infection Cerebellar/ PA, Medulloblastoma, Ependymoma, Metastases, Hemangioblastoma, Infratentorial/ Choroid plexus papilloma, AT/RT Choroid plexus papilloma, Subependymoma Fourth ventricle Brainstem PA, DMG Astrocytoma, Glioblastoma, DMG, Metastases Spinal cord Ependymoma, PA, DMG, MPE, Drop Ependymoma, Astrocytoma, DMG, MPE (filum), (intramedullary) metastases Paraganglioma (filum), Spinal cord Meningioma, Schwannoma, Schwannoma, Meningioma, (extramedullary) Metastases, Melanocytoma/melanoma Melanocytoma/melanoma, MPNST Spinal cord Bone tumor, Meningioma, Abscess, Herniated disk, Lymphoma, Abscess, (extradural) Vascular malformation, Metastases, Extra-axial/Dural/ Leukemia/lymphoma, Ewing Sarcoma, Meningioma, SFT, Metastases, Lymphoma, Leptomeningeal Rhabdomyosarcoma, Disseminated medulloblastoma, DLGNT, Sellar/infundibular Pituitary adenoma, Pituitary adenoma, -
797 Circulating Tumor DNA and Circulating Tumor Cells for Cancer
Medical Policy Circulating Tumor DNA and Circulating Tumor Cells for Cancer Management (Liquid Biopsy) Table of Contents • Policy: Commercial • Coding Information • Information Pertaining to All Policies • Policy: Medicare • Description • References • Authorization Information • Policy History • Endnotes Policy Number: 797 BCBSA Reference Number: 2.04.141 Related Policies Biomarkers for the Diagnosis and Cancer Risk Assessment of Prostate Cancer, #336 Policy1 Commercial Members: Managed Care (HMO and POS), PPO, and Indemnity Plasma-based comprehensive somatic genomic profiling testing (CGP) using Guardant360® for patients with Stage IIIB/IV non-small cell lung cancer (NSCLC) is considered MEDICALLY NECESSARY when the following criteria have been met: Diagnosis: • When tissue-based CGP is infeasible (i.e., quantity not sufficient for tissue-based CGP or invasive biopsy is medically contraindicated), AND • When prior results for ALL of the following tests are not available: o EGFR single nucleotide variants (SNVs) and insertions and deletions (indels) o ALK and ROS1 rearrangements o PDL1 expression. Progression: • Patients progressing on or after chemotherapy or immunotherapy who have never been tested for EGFR SNVs and indels, and ALK and ROS1 rearrangements, and for whom tissue-based CGP is infeasible (i.e., quantity not sufficient for tissue-based CGP), OR • For patients progressing on EGFR tyrosine kinase inhibitors (TKIs). If no genetic alteration is detected by Guardant360®, or if circulating tumor DNA (ctDNA) is insufficient/not detected, tissue-based genotyping should be considered. Other plasma-based CGP tests are considered INVESTIGATIONAL. CGP and the use of circulating tumor DNA is considered INVESTIGATIONAL for all other indications. 1 The use of circulating tumor cells is considered INVESTIGATIONAL for all indications. -
Choroid Plexus Papilloma Arising from the Temporal Horn with a Bilateral Hypersecretory Hydrocephalus: a Case Report and Review of Literature
Elmer ress Case Report World J Oncol. 2016;7(2-3):51-56 Choroid Plexus Papilloma Arising From the Temporal Horn With a Bilateral Hypersecretory Hydrocephalus: A Case Report and Review of Literature Sureswar Mohantya, Suman Saurav Routb, d, Gouri Sankar Sarangia, Kumudini Devic Abstract occur in the third ventricle. These tumors are benign histologi- cally and are neuroectodermal in origin and assigned a WHO Cerebrospinal fluid (CSF) within the cerebral ventricular system is grade I. Complete or gross total resection of these tumors often secreted by a neuroepithelial tissue which is called as the choroid results in a cure and almost recurrence free survival. The spe- plexus. Tumors arising from these tissues are rare. Choroid plexus cial challenges in the management of these tumors are mostly papillomas (CPPs) have been denoted as WHO grade I of the cho- due to its several unique features which include the young age roid plexus tumors. Among the intracranial tumors, neoplasms of the at presentation, high vascularity of these tumors and the poten- choroid plexus constitute around 0.36-0.6%. CPPs are mostly slow tial for hypersecretion of CSF. growing and cause symptoms due to mass effect and obstructive hy- drocephalus, resulting in increased intracranial pressure. We report a Case Report case of CPP arising from the temporal horn in a 7-year-old girl pre- senting with progressive head enlargement since birth due to bilateral massive hydrocephalus without any obstruction, making it purely a A 7-year-old girl presented with progressive head enlargement hypersecretory hydrocephalus. A drainage procedure followed by since birth, features of raised intracranial pressure in the form complete tumor resection was carried out in our case and the patient of headache, vomiting, excessive crying and excessive drowsi- showed marked relief from her symptoms. -
Connections Between Warburg's and Szentgyorgyi's Approach About The
Research iMedPub Journals Journal of Neoplasm 2017 http://www.imedpub.com/ ISSN 2576-3903 Vol.1 No.2:8 DOI: 10.217672576-3903.100008 Connections between Warburg’s and Szentgyorgyi’s Approach about the Causes of Cancer Szigeti GP1, Szasz O2 and Hegyi G3 1Institute of Human Physiology and Clinical Experimental Research, Semmelweis University, Hungary 2Department of Biotechnics, St. Istvan University, Hungary 3Department of Complementary and Alternative Medicine, University of Pecs, Hungary Corresponding author: Gabriella Hegyi, Department of Complementary and Alternative Medicine, University of Pecs, Hungary, Tel: +36 30 922-5347; E-mail: [email protected] Rec date: November 30, 2016; Acc date: December 26, 2016; Pub date: December 30, 2016 Citation: Szigeti GP, Szasz O, Hegyi G. Connections Between Warburg’s and Szentgyorgyi’s Approach About Causes of Cancer. J Neoplasm. 2017, 1: 2. to the environmental, diet and habit origins of malignant Abstract diseases [21-23]. Most widely, cancer is believed to be an abnormal tissue Numerous theories and hypotheses are published about triggered by a gene mutation. However, the proto-oncogene the causes of cancer and its hallmarks. Two remarkable and the oncogene appear not only in cancerous cases [24], but principles were established and debated for a long time. with pregnancy [25], with embryogenesis [26,27], with the The first is the Warburg-effect, which based on healing of wounds [28] and with the synthesis of growth mitochondrial dysfunction, connected to the intensive factors [29]. Oncogenes show a great variety of anti-apoptotic metabolic activity of the malignancy. The other is the functions with the cells taking part in the wound-healing [30]. -
Injury and Cancer
Case Western Reserve Law Review Volume 5 Issue 2 Article 4 1954 Injury and Cancer Lester Adelson Follow this and additional works at: https://scholarlycommons.law.case.edu/caselrev Part of the Law Commons Recommended Citation Lester Adelson, Injury and Cancer, 5 W. Rsrv. L. Rev. 150 (1954) Available at: https://scholarlycommons.law.case.edu/caselrev/vol5/iss2/4 This Article is brought to you for free and open access by the Student Journals at Case Western Reserve University School of Law Scholarly Commons. It has been accepted for inclusion in Case Western Reserve Law Review by an authorized administrator of Case Western Reserve University School of Law Scholarly Commons. [Winter Injury and Cancer by Lester Adelson, M.D. WITH LEGAL ANNOTATIONS BY OLIVER SCHROEDER, JR.* INTRODUCTION THE WORD "cancer" is one which is charged with emotion and fear, carrying with it implications of prolonged disability and slow, certain and painful death.' In considering and discussing the subject of cancer, diffi- culties frequently arise, not because not enough is known, but because so much is known that-is incorrect and untrue. A vast fund of misinformation and false information mis- leads the unwary stranger THE AUTHOR (A.B., 1935, Harvard Univer- and the uncritical observer. sity; M.D., 1939, Tufts College Medical School) is Chief Deputy Coroner and Patholo- Cancer is the second gist at the Coroner's Office, Cuyahoga County, leading cause of death, and Ohio, and Assistant Professor of Legal Medi- problems which arise in cine at the School of Medicine of Western Reserve University. connection with it are seen frequently. -
Hematopoietic and Lymphoid Neoplasm Coding Manual
Hematopoietic and Lymphoid Neoplasm Coding Manual Effective with Cases Diagnosed 1/1/2010 and Forward Published August 2021 Editors: Jennifer Ruhl, MSHCA, RHIT, CCS, CTR, NCI SEER Margaret (Peggy) Adamo, BS, AAS, RHIT, CTR, NCI SEER Lois Dickie, CTR, NCI SEER Serban Negoita, MD, PhD, CTR, NCI SEER Suggested citation: Ruhl J, Adamo M, Dickie L., Negoita, S. (August 2021). Hematopoietic and Lymphoid Neoplasm Coding Manual. National Cancer Institute, Bethesda, MD, 2021. Hematopoietic and Lymphoid Neoplasm Coding Manual 1 In Appreciation NCI SEER gratefully acknowledges the dedicated work of Drs, Charles Platz and Graca Dores since the inception of the Hematopoietic project. They continue to provide support. We deeply appreciate their willingness to serve as advisors for the rules within this manual. The quality of this Hematopoietic project is directly related to their commitment. NCI SEER would also like to acknowledge the following individuals who provided input on the manual and/or the database. Their contributions are greatly appreciated. • Carolyn Callaghan, CTR (SEER Seattle Registry) • Tiffany Janes, CTR (SEER Seattle Registry) We would also like to give a special thanks to the following individuals at Information Management Services, Inc. (IMS) who provide us with document support and web development. • Suzanne Adams, BS, CTR • Ginger Carter, BA • Sean Brennan, BS • Paul Stephenson, BS • Jacob Tomlinson, BS Hematopoietic and Lymphoid Neoplasm Coding Manual 2 Dedication The Hematopoietic and Lymphoid Neoplasm Coding Manual (Heme manual) and the companion Hematopoietic and Lymphoid Neoplasm Database (Heme DB) are dedicated to the hard-working cancer registrars across the world who meticulously identify, abstract, and code cancer data. -
Cancer Risk Assessment and Cancer Prevention: Promises and Challenges
CommentaryCancer Prevention Research Cancer Risk Assessment and Cancer Prevention: Promises and Challenges Brian J. Reid “Acquired genetic lability permits stepwise selection of variant cancer itself, but morphologic assessment may be limited in its sublines and underlies tumor progression” (1). ability to distinguish asymptomatic indolent conditions from Despite intense efforts to cure cancers through advances in those that will progress to advanced malignancies and death staging, surgery, chemo- and radiation therapy, treatment of (3–5, 13). Examining cancer from an evolutionary perspective most advanced, symptomatic epithelial malignancies con- can open new approaches for cancer risk assessment, diagnosis, tinues to be challenging, and age-adjusted cancer mortality therapy and prevention. The evolution of multicellular organ- in the United States has decreased by only 5%since 1950 isms has been accompanied by constraint of cellular evolution, (2). The clinical course of treated cancer patients all too often and cancer represents a breakdown of the mechanisms that culminates in relapse and death. Ironically, growing evidence suppress cellular evolution. Cairns hypothesized that the archi- also suggests that many patients with premalignancy or even tecture of proliferating epithelial tissues would suppress tumor malignancy follow benign courses and die far more often of formation by restricting and sequestering stem cells in epithe- non-cancer causes than of cancer (3–5). These paradoxical phe- lial proliferative units, for example, at the base of intestinal nomena form the dilemma of early detection-underdetection crypts (14). Opportunities for competition between variants of life-threatening early disease and overdetection of indolent that might arise would be restrained by shedding differentiated early disease. -
Stereotactic Radiosurgery in Hemangioblastoma
Published online: 2019-09-25 Original Article Stereotactic radiosurgery in hemangioblastoma: Experience over 14 years Nishant Goyal, Deepak Agrawal, Raghav Singla, Shashank Sharad Kale, Manmohan Singh, Bhawani Shankar Sharma Department of Neurosurgery and Gamma Knife, All India Institute of Medical Sciences, New Delhi, India ABSTRACT Background: Although gamma knife has been advocated for hemangioblastomas, it is not used widely by neurosurgeons. Objective: We review our experience over 14 years in an attempt to define the role of stereotactic radiosurgery (SRS) in the management of hemangioblastomas. Patients and Methods: A retrospective study was conducted on all patients of hemangioblastoma who underwent SRS at our institute over a period of 14 years (1998–2011). Gamma knife plans, clinical history, and radiology were reviewed for all patients. Results: A total of 2767 patients underwent gamma knife during the study period. Of these, 10 (0.36%) patients were treated for 24 hemangioblastomas. Eight patients (80%) had von Hippel‑Lindau disease while two had sporadic hemangioblastomas. The median peripheral dose (50% isodose) delivered to the tumors was 29.9 Gy. Clinical and radiological follow‑up data were available for eight patients. Of these, two were re‑operated for persisting cerebellar symptoms. The remaining six patients were recurrence‑free at a mean follow‑up of 48 months (range 19–108 months). One patient had an increase in cyst volume along with a decrease in the size of the mural nodule. Conclusions: SRS should be the first option for asymptomatic hemangioblastomas. Despite the obvious advantages, gamma knife is not widely used as an option for hemangioblastomas. Key words: Gamma knife radiosurgery, hemangioblastomas, stereotactic radiosurgery, von Hippel‑Lindau syndrome Introduction view of site, vascularity, and number.