DOCSLIB.ORG

Lam-Guideline-Pt2.Pdf

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Lam-Guideline-Pt2.Pdf AMERICAN THORACIC SOCIETY DOCUMENTS Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline Nishant Gupta, Geraldine A. Finlay, Robert M. Kotloff, Charlie Strange, Kevin C. Wilson, Lisa R. Young, Angelo M. Taveira-DaSilva, Simon R. Johnson, Vincent Cottin, Steven A. Sahn, Jay H. Ryu, Kuniaki Seyama, Yoshikazu Inoue, Gregory P. Downey, MeiLan K. Han, Thomas V. Colby, Kathryn A. Wikenheiser-Brokamp, Cristopher A. Meyer, Karen Smith, Joel Moss*, and Francis X. McCormack*; on behalf of the ATS Assembly on Clinical Problems THIS OFFICIAL CLINICAL PRACTICE GUIDELINE WAS APPROVED BY THE AMERICAN THORACIC SOCIETY OCTOBER 2017 AND BY THE JAPANESE RESPIRATORY SOCIETY AUGUST 2017 Background: Recommendations regarding key aspects Results: For women who have cystic changes on high-resolution related to the diagnosis and pharmacological treatment of computed tomography of the chest characteristic of LAM, but who have lymphangioleiomyomatosis (LAM) were recently published. We no additional confirmatory features of LAM (i.e., clinical, radiologic, or now provide additional recommendations regarding four specific serologic), the guideline panel made conditional recommendations questions related to the diagnosis of LAM and management of against making a clinical diagnosis of LAM on the basis of the high- pneumothoraces in patients with LAM. resolution computed tomography findings alone and for considering transbronchial lung biopsy as a diagnostic tool. The guideline panel also Methods: Systematic reviews were performed and then discussed made conditional recommendations for offering pleurodesis after an by a multidisciplinary panel. For each intervention, the panel initial pneumothorax rather than postponing the procedure until the considered its confidence in the estimated effects, the balance of fi fi rst recurrence and against pleurodesis being used as a reason to desirable (i.e., bene ts) and undesirable (i.e., harms and burdens) exclude patients from lung transplantation. consequences, patient values and preferences, cost, and feasibility. Evidence-based recommendations were then formulated, written, and Conclusions: Evidence-based recommendations for the diagnosis graded using the GRADE (Grading of Recommendations, Assessment, and treatment of patients with LAM are provided. Frequent Development, and Evaluation) approach. reassessment and updating will be needed. Contents Formulating Questions and Questions and Recommendations Overview Outcomes Question 1 Introduction Literature Search and Study Question 2 Methods Selection Question 3 Committee Composition Evidence Synthesis Question 4 Conflict-of-Interest Development of Conclusions Management Recommendations Guideline Panel Meetings Manuscript Preparation *These authors share joint senior authorship. ORCID ID: 0000-0001-9112-1315 (N.G.). Correspondence and requests for reprints should be addressed to Joel Moss, M.D., Ph.D., Cardiovascular and Pulmonary Branch, NHLBI, National Institutes of Health, Bethesda, MD 20892. E-mail: [email protected] Am J Respir Crit Care Med Vol 196, Iss 10, pp 1337–1348, Nov 15, 2017 Copyright © 2017 by the American Thoracic Society DOI: 10.1164/rccm.201709-1965ST Internet address: www.atsjournals.org American Thoracic Society Documents 1337 AMERICAN THORACIC SOCIETY DOCUMENTS Overview recommendation, low confidence in the believed that the yield of transbronchial estimated effects). lung biopsy likely correlates with This guideline is the continuation of a prior ∘ Remarks: In the guideline panelists’ markers of parenchymal LAM burden lymphangioleiomyomatosis (LAM) clinical practices, a clinical diagnosis of (such as cyst profusion, abnormal guideline document developed by the LAM is based on a combination of diffusing capacity of the lung for carbon American Thoracic Society (ATS) and the characteristic HRCT features plus one monoxide, abnormal FEV1)andthat Japanese Respiratory Society (JRS) (1). or more of the following: presence of appropriate patient selection is required fi The current guideline collates the evidence tuberous sclerosis complex (TSC), to optimize the safety and ef cacy of for emerging advancements in LAM and angiomyolipomas, chylous effusions, this diagnostic approach. Consultation then uses this evidence to formulate lymphangioleiomyomas with an expert center before recommendations pertaining to the (lymphangiomyomas), or elevated undertaking transbronchial lung biopsy, diagnosis and treatment of patients with serum vascular endothelial growth combined with a critical review of the LAM. The intent of the guideline is to factor-D (VEGF-D) greater than or tissue specimens by a pathologist with empower clinicians to apply the equal to 800 pg/ml. expertise in LAM, can help avoid false- d fi recommendations in the context of the When a de nitive diagnosis is required negative test results and the need for a values and preferences of individual in patients who have parenchymal cysts surgical lung biopsy. d patients and to tailor their decisions to the on HRCT that are characteristic of LAM, We suggest that patients with LAM be fi offered ipsilateral pleurodesis after clinical situation at hand. The guideline but no additional con rmatory features their initial pneumothorax rather than panel’s recommendations (Table 1) are of LAM (i.e., clinical, radiologic, or waiting for a recurrent pneumothorax as follows: serologic), we suggest a diagnostic approach that includes transbronchial before intervening with a pleural d Forpatientswhohavecysticchangeson lung biopsy before a surgical lung biopsy symphysis procedure (conditional high-resolution computed tomography (conditional recommendation, very low recommendation, very low confidence (HRCT) of the chest that are confidence in the estimated effects). in the estimated effects). characteristic of LAM, but have no ∘ Remarks: The advantage of ∘ Remarks: This approach is based on the additional confirmatory features of transbronchial lung biopsy is that it high rate of recurrence of spontaneous LAM (i.e., clinical, radiologic, or offers a less-invasive method to obtain pneumothoraces in patients with LAM. serologic), we suggest NOT using the histopathological confirmation of LAM, Nonetheless, the final decision to HRCT features in isolation to make a as compared with surgical lung biopsy. perform pleurodesis and the type of clinical diagnosis of LAM (conditional Although not proven, the panelists pleurodesis (chemical vs. surgical) Table 1. Summary of the Recommendations Provided in This Guideline Strength of Confidence in Context Recommendation Recommendation Estimates of Effect HRCT as sole confirmatory For patients who have cystic changes on HRCT of the Conditional Low feature for LAM diagnosis chest that are characteristic of LAM, but have no additional confirmatory features of LAM (i.e., clinical, radiologic, or serologic), we suggest NOT using the HRCT features in isolation to make a clinical diagnosis of LAM. Transbronchial lung biopsy for When a definitive diagnosis is required in patients who Conditional Very low histopathological diagnosis of have parenchymal cysts on HRCT that are LAM characteristic of LAM, but no additional confirmatory features of LAM (i.e., clinical, radiologic, or serologic), we suggest a diagnostic approach that includes transbronchial lung biopsy before a surgical lung biopsy. Pleurodesis after a sentinel We suggest that patients with LAM be offered Conditional Very low pneumothorax to prevent ipsilateral pleurodesis after their initial recurrence pneumothorax rather than waiting for a recurrent pneumothorax before intervening with a pleural symphysis procedure. Pleurodesis as a contraindication to We suggest that previous unilateral or bilateral pleural Conditional Very low future lung transplant procedures (i.e., pleurodesis or pleurectomy) NOT be considered a contraindication to lung transplantation in patients with LAM. Definition of abbreviations: HRCT = high-resolution computed tomography; LAM = lymphangioleiomyomatosis. 1338 American Journal of Respiratory and Critical Care Medicine Volume 196 Number 10 | November 15 2017 AMERICAN THORACIC SOCIETY DOCUMENTS should be based on shared decision- Methods panel discussed the scope of the document, making between the clinician(s) and the questions to be addressed, the evidence, patient, after education about various Committee Composition and the recommendations. The management options. Every effort must The guideline development panel was co- cosponsoring societies (the ATS and JRS) be made to ensure that the pleurodesis is chaired by F.X.M and J.M. and consisted of provided financial support for the meetings handled by clinicians familiar with clinicians and researchers with recognized and conference calls, as well as travel management of pleural disease in LAM. expertise in LAM (1). A methodologist expenses. Additional support for travel of d We suggest that previous unilateral or (K.C.W.) with expertise in the guideline panelists to meetings was provided by the bilateral pleural procedures development process and application of the not-for-profit LAM Foundation and LAM (i.e., pleurodesis or pleurectomy) NOT Grading of Recommendations, Assessment, Treatment Alliance. The ATS, JRS, and be considered a contraindication to lung Development, and Evaluation (GRADE) Foundations had no influence on question transplantation in patients with LAM approach
Load more

Recommended publications
  • Surgical Management of Empysema
    Surgical Management of Empysema John A Odell MB ChB, FRCS(Ed), FACS Emeritus Professor of Surgery Mayo Clinic College of Medicine Previously Surgical Director Lung Transplantation ©2013 MFMER | slide-1 Problem case. How would you manage? • 64yr male • Previous RLL bullectomy. • Recent left pneumothorax managed elsewhere with chest tube placement. • Because of continued air-leak talc pleurodesis. • Air-leak continues. ©2013 MFMER | slide-2 • On nasal oxygen. Dyspneic on walking • Significant air-leak. More dyspneic when suction applied to chest drain. • FEV1 40% predicted. DLCO 12% predicted. Options? • Thoracotomy and close air leak surgically • Videothoracoscopy and closure of air-leak • Thoracotomy and decortication • Remove chest drain • List for transplantation Historical Treatment of Emphysema • Abdominal compression belts. The stimulus was the observation that emphysematous patients lean forward when breathing. • Pneumoperitoneum. In an attempt to restore diaphragmatic curvature. • Lungs too large for the chest – costochondrectomy or transverse sternotomy to provide more room. Multiple wedge excisions. • Chest grown too large – thoracoplasty • Pleurodesis. Emphysema results from alveolar wall ischemia. • Phrenectomy. Overvigerous inspiration was ripping alveolar walls. • Hilar denervation. To decrease bronchoconstriction and mucous production mediated by the parasympathetic nervous system. • Whole lung irradiation. To increase elastic recoil by inducing fibrosis. ©2013 MFMER | slide-7 Variants of Emphysema that may be Surgically
    [Show full text]
  • Effect of an Indwelling Pleural Catheter Vs Talc Pleurodesis On
    This supplement contains the following items: 1. Original protocol, final protocol, summary of changes. 2. Original statistical analysis plan. There were no further changes to the original statistical analysis plan. Downloaded From: https://jamanetwork.com/ on 10/02/2021 The Australasian Malignant Pleural Effusion Trial (AMPLE) A Multicentre Randomized Study Comparing Indwelling Pleural Catheter vs Talc Pleurodesis in Patients with Malignant Pleural Effusions Ethics Registration number 2012-005 Protocol version number 1.0 Protocol date 10/01/2012 Authorised by: Name: Prof YC Gary Lee Role: Chief Investigator Signature: Date: 10/01/2012 Downloaded From: https://jamanetwork.com/ on 10/02/2021 General Information This document describes the Western Australian Randomised Malignant Effusion trial for the purpose of submission for review by the relevant human research and ethics committees. It provides information about procedures for entering patients into the trial and this protocol should not be used as a guide for the treatment of other patients; every care was taken in its drafting, but corrections or amendments may be necessary. Questions or problems relating to this study should be referred to the Chief Investigator or Trial Coordinator. Compliance The trial will be conducted in compliance with this protocol, the National Statement on Ethical Conduct in Human Research, data protection laws and other guidelines as appropriate. It will be registered with the Australia and New Zealand Clinical Trials Registry, once ethical approval is secured.
    [Show full text]
  • The Health-Related Quality of Life of Sarcoma Patients and Survivors In
    Cancers 2020, 12 S1 of S7 Supplementary Materials The Health-Related Quality of Life of Sarcoma Patients and Survivors in Germany—Cross-Sectional Results of A Nationwide Observational Study (PROSa) Martin Eichler, Leopold Hentschel, Stephan Richter, Peter Hohenberger, Bernd Kasper, Dimosthenis Andreou, Daniel Pink, Jens Jakob, Susanne Singer, Robert Grützmann, Stephen Fung, Eva Wardelmann, Karin Arndt, Vitali Heidt, Christine Hofbauer, Marius Fried, Verena I. Gaidzik, Karl Verpoort, Marit Ahrens, Jürgen Weitz, Klaus-Dieter Schaser, Martin Bornhäuser, Jochen Schmitt, Markus K. Schuler and the PROSa study group Includes Entities We included sarcomas according to the following WHO classification. - Fletcher CDM, World Health Organization, International Agency for Research on Cancer, editors. WHO classification of tumours of soft tissue and bone. 4th ed. Lyon: IARC Press; 2013. 468 p. (World Health Organization classification of tumours). - Kurman RJ, International Agency for Research on Cancer, World Health Organization, editors. WHO classification of tumours of female reproductive organs. 4th ed. Lyon: International Agency for Research on Cancer; 2014. 307 p. (World Health Organization classification of tumours). - Humphrey PA, Moch H, Cubilla AL, Ulbright TM, Reuter VE. The 2016 WHO Classification of Tumours of the Urinary System and Male Genital Organs—Part B: Prostate and Bladder Tumours. Eur Urol. 2016 Jul;70(1):106–19. - World Health Organization, Swerdlow SH, International Agency for Research on Cancer, editors. WHO classification of tumours of haematopoietic and lymphoid tissues: [... reflects the views of a working group that convened for an Editorial and Consensus Conference at the International Agency for Research on Cancer (IARC), Lyon, October 25 - 27, 2007]. 4. ed.
    [Show full text]
  • 2019 Coding and Payment Information Pleural Effusions and Ascites Management
    2019 coding and payment information Pleural effusions and ascites management The information contained in this document, including Reimbursement rates shown are Medicare national the codes supplied, is provided for informational payments for 2019 and do not reflect actual payments purposes only. BD makes no statement, promise or made to individual providers, as payments are adjusted guarantee concerning the appropriateness of any codes specific to particular geographic regions. for a particular procedure, actual levels of All information is subject to change without notice. In reimbursement, payment or charge or that addition, payers or local carriers may have their own reimbursement will be made. coding and billing requirements. Consult your payer This is not intended to be a comprehensive guide to all organization with regard to local reimbursement policies. coding and payment information. 2019 Medicare outpatient facility rates CPT® 2019 APC OPPS 2019 ASC Description APC Status code base rate* base rate Pleural catheter procedures 32550 Insertion of indwelling tunneled pleural catheter 5341 J1 $2,947 $1,790 32552 Removal of indwelling tunneled pleural catheter with cuff 5181 Q2 $620 $319 32560 Instillation via chest tube/catheter, agent for pleurodesis 5181 T $620 N/A 32650 Thoracoscopy, surgical, with pleurodesis (e.g., mechanical or chemical) N/A; inpatient procedure Peritoneal catheter procedures Insertion of tunneled intraperitoneal catheter (e.g., dialysis, intraperitoneal chemotherapy instillation, management of ascites), 49418
    [Show full text]
  • FAQ Document
    The Society of Thoracic Surgeons Frequently Asked Questions: General Thoracic Database Version 2.07 December 2008 How to use the “interactive” FAQ Document: 1. To review all clinical questions in an individual section, click on the section title below. Section A: seq# 10 - 190 Section C: seq# 285 - 650 Section E: seq# 770 - 1210 Section B: seq# 200 - 260 Section D: seq# 660 - 750 Section F: seq# 1220 - 1340 2. To review an individual Seq# clinical question, click on the Seq# title below. Participation in both General Thoracic and Adult Cardiac Databases GENERAL STATEMENT #2 Seq# 200: Zubrod Score Seq# 775: Postop Events Seq# 300: WtLoss3Kg Seq# 860: Pneumonia Seq# 310: Category of disease Seq# 930: Other Pulmonary Event Seq# 390: PreOp Chemotherapy Seq# 940: Atrial Arrhythmia Seq# 400: PreOp Thoracic RT Seq# 1020: Anastomotic leak Seq# 430: Other Cormorbidity Seq# 1190: Empyema Seq# 540: Clinical Stage Not Applicable (2.06) Seq# 1200: Other event req. Rx Seq# 725: Reoperation Seq# 1250: 30 Day Status Seq# 740: Procedure Seq# 1280: Chest Tube Out Date Seq# 750: Primary Procedure Seq# 751: Thoracoscopy Approach NEW Date SeqNo FieldName Definition 3/06 Participation in both General We participate in both Adult Cardiac and Yes, enter into both. In the Adult Cardiac DB, the Thoracic and Adult Cardiac General Thoracic Database. Our question primary procedure would be Seq# 1310 pertains to whether the patient should end up OpOCard=Yes; Seq# 2510 ONCAoAn=Yes; Databases in both databases. Scenario--We had a Seq# 2530 ONCArch=Yes; Seq# 2540 patient who had an aortic aneurysm repair ONCDesc=Yes; Seq# 3220 Readm30=Yes; (arch/descending thoracic) and was entered Seq# ReadmRsn=either Pneumonia or other into the Adult Cardiac Database.
    [Show full text]
  • The Nutrition and Food Web Archive Medical Terminology Book
    The Nutrition and Food Web Archive Medical Terminology Book www.nafwa.
    [Show full text]
  • Agenda Item 5
    List A (O) Respiratory system treatments and procedures. (1) Biopsy and/or excision (removal) of lesion of larynx, vocal cords, trachea (A) Loss or change of voice. (B) Swallowing or breathing difficulties. (C) Perforation (hole) or fistula (connection) in esophagus (tube from throat to stomach). (2) Rhinoplasty (surgery to change the shape of the nose) or nasal reconstruction with or without septoplasty A) Deformity of skin, bone or cartilage. (B) Creation of new problems, such as perforation of the nasal septum (hole in wall between the right and left halves of the nose) or breathing difficulty. (3) Submucous resection of nasal septum or nasal septoplasty (surgery to remove blockage in or straighten the nose) (A) Persistence, recurrence or worsening of the obstruction. (B) Perforation of nasal septum (hole in wall between the right and left halves of the nose) with dryness and crusting. (C) External deformity of the nose. (4) Sinus surgery/endoscopic sinus surgery (A) Spinal fluid leak (B) Visual loss or other eye injury (C) Numbness in front teeth and palate (D) Loss or reduction in sense of taste or smell (E) Recurrence of disease (F) Empty Nose Syndrome (sensation of nasal congestion, sensation of not being able to take in adequate air through nose) (G) Injury to tear duct causing drainage of tears down the cheek (H) Brain injury and/or infection (I) Injury to nasal septum (J) Nasal obstruction (5) Lung biopsy (A) Air leak with pneumothorax (leak of air from lung to inside of chest causing the lung to collapse) with need for insertion of chest drainage tube into space between lung and chest wall or repeat surgery.
    [Show full text]
  • About Soft Tissue Sarcoma Overview and Types
    cancer.org | 1.800.227.2345 About Soft Tissue Sarcoma Overview and Types If you've been diagnosed with soft tissue sarcoma or are worried about it, you likely have a lot of questions. Learning some basics is a good place to start. ● What Is a Soft Tissue Sarcoma? Research and Statistics See the latest estimates for new cases of soft tissue sarcoma and deaths in the US and what research is currently being done. ● Key Statistics for Soft Tissue Sarcomas ● What's New in Soft Tissue Sarcoma Research? What Is a Soft Tissue Sarcoma? Cancer starts when cells start to grow out of control. Cells in nearly any part of the body can become cancer and can spread to other areas. To learn more about how cancers start and spread, see What Is Cancer?1 There are many types of soft tissue tumors, and not all of them are cancerous. Many benign tumors are found in soft tissues. The word benign means they're not cancer. These tumors can't spread to other parts of the body. Some soft tissue tumors behave 1 ____________________________________________________________________________________American Cancer Society cancer.org | 1.800.227.2345 in ways between a cancer and a non-cancer. These are called intermediate soft tissue tumors. When the word sarcoma is part of the name of a disease, it means the tumor is malignant (cancer).A sarcoma is a type of cancer that starts in tissues like bone or muscle. Bone and soft tissue sarcomas are the main types of sarcoma. Soft tissue sarcomas can develop in soft tissues like fat, muscle, nerves, fibrous tissues, blood vessels, or deep skin tissues.
    [Show full text]
  • A Case of Lymphangioleiomyomatosis Originated in the Pelvic Cavity
    J Gynecol Oncol Vol. 19, No. 3:195-198, September 2008 DOI:10.3802/jgo.2008.19.3.195 Case Report A case of lymphangioleiomyomatosis originated in the pelvic cavity Jung-Mi Han, Kyung-Hee Lee, Sung-Joo Kim, Chae-Chun Rhim, Young-Han Park, Jung-Bae Kang, Sun-Young Jeon1 Departments of Obstetrics and Gynecology, 1Pathology, Hallym University Medical College, Anyang, Korea Lymphangioleiomyomatosis is a rare disease that is characterized by proliferation of abnormal smooth muscle-like cells, especially that which occurs in the pulmonary parenchyme. It primarily affects women of child-bearing age. The majority of primary lymphangioleiomyomatosis occurs in the lung, but there are a few reports of extrapulmonary cases. We experienced a rare case of lymphangioleiomyomatosis which originated in the pelvic cavity (in the posterior portion of the uterus), and report with brief review of literatures. Key Words: Lymphangioleiomyomatosis, Pelvis, Uterus INTRODUCTION hypervascular tumor between the uterus and the right ovary, and two small myomas about 2 cm in size (Fig. 1). Under the Lymphangioleiomyomatosis is a very rare disease which impression of ovarian malignancy she had admitted for shows typical features of abnormal smooth muscle cell further evaluation including MRI. Her initial serum CA-125 proliferation and which develops in females during the level was 26.7 U/ml and CA 19-9 level was below 2 U/ml, and reproductive period.1,2 The majority cases of this disease other hematologic findings were all within the normal range. primarily occur in the lungs, but extrapulmonary regions such Magnetic resonance imaging study of the abdomen-pelvis as the pelvis and retroperitoneal spaces are occasionally demonstrated an approximately 4.0×5.0×4.0 cm sized tumor primary sites.
    [Show full text]
  • Hyperbaric Medicine Center During the Actual Questions About the Treatment Process Are Treatment Process, for All Patients’ Confidentiality, No Always Welcomed
    Patient Label / Name HBO - HYPERBARIC OXYGEN PATIENT EDUCATION - Page | 1 FAQs HBO is a medical treatment which enhances the body’s natural healing process by inhalation of 100% oxygen in a total body chamber, where atmospheric pressure is WHAT IS HBO? increased and controlled. It is used for a wide variety of treatments usually as an adjunctive part of an overall HBO or HBOT stands for: Hyperbaric Oxygen Therapy medical care plan that can be done on an inpatient or WHAT DOES IT DO? outpatient basis. HBO or HBOT, simply, HBOT is a means of providing WAIT! CAN YOU TELL ME MORE ABOUT THE additional oxygen to your body tissues. “ENTIRELY CLOSED PRESSURE CHAMBER”? HOW DOES IT WORK? Your entire body will go in a closely monitored, TRANSPARENT, 100% ACRYLIC topped, sealed chamber. By exposing the Most patients have plenty of room to get comfortable entire body to and can enjoy their 1.5-2 hour treatments while watching oxygen under TV (installed on each chamber with internal speakers) or increased napping. atmospheric A highly qualified Hyperbaric Tech will be in the room pressure. with you at all times during your treatment, and you will be closely monitored before, during and after all HBO The patient is entirely enclosed in a pressure chamber Treatments. breathing 100% oxygen (O2) at greater than one atmosphere HBO Chambers have microphones that allow your HBO (atm) pressure. Tech to hear you without need to push any buttons. SO, HOW DOES THAT HELP? Simply call out and you will be heard. If you should need to cut your treatment short or want to get out at any HBO therapy serves 4 PRIMARY FUNCTIONS: time, your HBO Tech is always right there to safely decompress the chamber and help you out and call for 1.
    [Show full text]
  • Lymphangioleiomyomatosis and Tuberous Sclerosis: Where Is the Border?
    Eur Respir J, 1996, 9, 399–401 Copyright ERS Journals Ltd 1996 DOI: 10.1183/09031936.96.09030399 European Respiratory Journal Printed in UK - all rights reserved ISSN 0903 - 1936 EDITORIAL Lymphangioleiomyomatosis and tuberous sclerosis: Where is the border? F. Bonetti*, P. Chiodera** Pulmonary lymphangioleiomyomatosis (PLAM) is a The key to addressing this unresolved question seems rare disease, characterized by an abnormal proliferation to be the definition of the diagnostic criteria of TS. The of smooth muscle throughout the lung; it occurs exclu- classical Vogt triad (seizures, mental retardation and facial sively in women, generally of reproductive age [1]. The angiofibroma) was the first important attempt to clini- muscle cells proliferating in PLAM show little or no cally define the syndrome. This diagnostic triad was atypia at histological level. Therefore, PLAM has been universally accepted and was, for a long time, consi- considered to be a hamartomatous lesion rather than a dered the hallmark of the disease. Whilst useful for clini- true neoplastic process. However, in spite of the bland cians, this approach led to a delay in the recognition of cytological features, the disease causes a progressive other diagnostic signs of TS, particularly the presence structural remodelling of the lung, leading to serious and classification of visceral lesions, such as angio- impairment of pulmonary function. Whilst survival curves myolipoma, so characteristic in this hereditary disease of now seem better than previously believed and hormonal autosomal dominant transmission. With the accumula- treatment has been introduced with encouraging results tion of knowledge on TS and the introduction of new [2], some patients progress to a condition necessitating diagnostic techniques, the Vogt triad has lost much of lung transplantation.
    [Show full text]
  • Differential Diagnostic Considerations
    Radiology Case Reports Volume 10, Issue 2, 2015 Chylothorax in a patient with metastatic Kaposi sarcoma: Differential diagnostic considerations Ryan Alexander, DO; Magda Rizer, DO; William Burke, MD; and Lawrence Ciment, MD Kaposi sarcoma (KS) is a low-grade mesenchymal tumor involving blood and lymphatic vessels. There are four types, based on clinical presentation: classic, endemic (Africana), iatrogenic (typically, involving renal allograft recipients), and AIDS-associated (epidemic). Kaposi’s sarcoma-associated herpes virus in- fection has been linked along with other factors to the development of KS. The Kaposi’s sarcoma- associated herpes virus interacts and encodes for numerous molecular proteins that play a role in the pathogenesis of KS, including latency-associated nuclear antigen, viral G protein-coupled receptor, viral FLICE inhibitory protein, and viral IL-6. KS primarily affects the skin and causes disseminated disease in a variety of organs. Involvement of visceral organs other than the lining of the alimentary tract is ex- tremely rare. While the chylous pleural effusions of KS may resemble other pulmonary diseases (includ- ing lymphangioma, lymphangectasis, and lymphangioleiomyomatosis) with chylous effusions at thoracic CT, differentiating features may allow for more prompt diagnosis and treatment. The presumptive diag- nosis of AIDS-related pulmonary KS is often clinical. A tissue diagnosis is not required to establish the diagnosis of pulmonary KS. There are a variety of causes of chylothorax. The primary finding is a near- water-attenuating pleural effusion. The secondary findings of chylothorax can help differentiate the etiology. Introduction be diagnosed on CT or MRI. The CT finding of chylous Chylothorax is a relatively uncommon cause of pleural effusions is typically a large unilateral effusion of water effusion (1).
    [Show full text]