DOCSLIB.ORG

The North American Neuroendocrine Tumor Society Consensus

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
The North American Neuroendocrine Tumor Society Consensus NANETS GUIDELINES The North American Neuroendocrine Tumor Society Consensus Guideline for the Diagnosis and Management of Neuroendocrine Tumors Pheochromocytoma, Paraganglioma, and Medullary Thyroid Cancer Herbert Chen, MD,* Rebecca S. Sippel, MD,* M. Sue O’Dorisio, MD, PhD,Þ Aaron I. Vinik, MD, PhD,þ Ricardo V. Lloyd, MD, PhD,§ and Karel Pacak, MD, PhD, DSc|| gliomas in the abdomen most commonly arise from chromaf- Abstract: Pheochromocytomas, intra-adrenal paraganglioma, and extra- fin tissue around the inferior mesenteric artery (the organ of adrenal sympathetic and parasympathetic paragangliomas are neu- Zuckerkandl) and aortic bifurcation, less commonly from any roendocrine tumors derived from adrenal chromaffin cells or similar cells other chromaffin tissue in the abdomen, pelvis, and thorax.2 Extra- in extra-adrenal sympathetic and parasympathetic paraganglia, respec- adrenal parasympathetic paragangliomas are most commonly tively. Serious morbidity and mortality rates associated with these tumors found in the neck and head. are related to the potent effects of catecholamines on various organs, es- Pheochromocytomas and sympathetic extra-adrenal para- pecially those of the cardiovascular system. Before any surgical procedure gangliomas almost all produce, store, metabolize, and secrete is done, preoperative blockade is necessary to protect the patient against catecholamines or their metabolites. Recent studies have found significant release of catecholamines due to anesthesia and surgical ma- that approximately 20% of head and neck paragangliomas also nipulation of the tumor. Treatment options vary with the extent of the produce significant amounts of catecholamines.3 disease, with laparoscopic surgery being the preferred treatment for re- Main signs and symptoms of catecholamine excess include moval of primary tumors. Medullary thyroid cancer (MTC) is a ma- hypertension, palpitations, headache, sweating, and pallor. Less lignancy of the thyroid C cells or parafollicular cells. Thyroid C cells common signs and symptoms are fatigue, nausea, weight loss, elaborate a number of peptides and hormones, such as calcitonin, carci- constipation, flushing, and fever. According to the degree of cat- noembryonic antigen, and chromogranin A. Some or all of these markers echolamine excess, patients may present with myocardial infarc- are elevated in patients with MTC and can be used to confirm the diagnosis tion, arrhythmia, stroke, or other vascular manifestations (eg, any as well as to follow patients longitudinally for recurrence. Medullary thy- organ ischemia). Similar signs and symptoms are produced by roid cancer consists of a spectrum of diseases that ranges from extremely numerous other clinical conditions, and therefore, pheochromo- indolent tumors that are stable for many years to aggressive types associ- cytoma is often referred to as the Bgreat mimic.[ ated with a high mortality rate. Genetic testing for RET mutations has allowed identification of familial cases and prophylactic thyroidectomy for Epidemiology cure. The only curative treatment is complete surgical resection. Pheochromocytomas and paragangliomas are rare and Key Words: neuroendocrine tumors, pheochromocytoma, occur in approximately 0.05% to 0.1% of patients with sustained paraganglioma, medullary thyroid cancer hypertension. However, this probably accounts for only 50% of people harboring pheochromocytoma or paraganglioma because (Pancreas 2010;39: 775Y783) approximately half of patients with pheochromocytoma or paraganglioma have paroxysmal hypertension or normotension. The prevalence of pheochromocytoma and paraganglioma can PHEOCHROMOCYTOMA AND PARAGANGLIOMA be estimated to lie between 1:6500 and 1:2500, with the annual In 2004, the World Health Organization defined a pheochro- incidence in the United States of 500 to 1600 cases per year. mocytoma as an intra-adrenal paraganglioma, whereas closely related tumors of extra-adrenal sympathetic or parasympathetic Pathology and Molecular Genetics paraganglia are classified as extra-adrenal paragangliomas. In All pheochromocytomas and paragangliomas display sim- general, approximately 80% of pheochromocytomas are located ilar basic histopathologic characteristics, although some differ- in the adrenal medulla.1 Extra-adrenal sympathetic paragan- ences between familial tumors have been described. According to the 2004 World Health Organization criteria,4 malignancy is defined by the presence of metastases, not local invasion (although a significant invasion is considered by some patholo- From the *Department of Surgery, University of Wisconsin, Madison, gists as the sign of malignancy). There is currently no consensus WI; †Department of Pediatrics, University of Iowa, Iowa City, IA; ‡Eastern Virginia Medical School, Strelitz Diabetes Research Center, Norfolk, VA; on the adoption of a formal scoring system for these tumors. §Department of Pathology and Laboratory Medicine, University of Wis- Improvements in genetics, diagnosis, and treatment of pheo- consin, Madison, WI; and ||Section on Medical Neuroendocrinology, Re- chromocytomas have changed the approaches to these tumors in productive and Adult Endocrinology Branch, National Institute of Child recent years. The formerly used rule of 10% for pheochromo- Health and Human Development, National Institute of Health Bethesda, MD. cytoma (10% malignant, 10% bilateral, and 10% extra-adrenal) Reprints: Herbert Chen, MD, Department of Surgery, University of 5 Wisconsin, H4/722 CSC, 600 Highland Ave, Madison, WI 53792-7375 has been increasingly challenged. At present, it is estimated (e-mail: [email protected]); or Karel Pacak, MD, PhD, DSc, that at least 24% to 27% of pheochromocytomas or paragan- Section on Medical Neuroendocrinology Reproductive and Adult gliomas are associated with known genetic mutations; in chil- Endocrinology Branch NICHD, Bldg 10, CRC, 1-East Room 1-3140, 6Y11 10 Center Dr, MSC-1109 Bethesda, MD 20892-1109 dren, this prevalence may be as high as 40%. (e-mail: [email protected]). Pheochromocytomas may occur sporadically or as part of Copyright * 2010 by Lippincott Williams & Wilkins hereditary syndrome. According to the latest studies, among Pancreas & Volume 39, Number 6, August 2010 www.pancreasjournal.com 775 Copyright © 2010 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. Chen et al Pancreas & Volume 39, Number 6, August 2010 TABLE 1. Characteristics in Various Pheochromocytomas or Paragangliomas Tumor and Clinical Characteristics Sporadic SDHB MEN, VHL, NF-1 Malignancy rate 10%Y36% High (950%) but to be determined G5% Most common location of primary tumor Adrenal Extra-adrenal Adrenal Biochemical phenotype NE NE, DA NE EPI Most common sites of metastatic lesions Bones, LNs, liver, lungs Bones, LNs, liver, lungs Bones, LNs Unfavorable prognostic factors Younger age, large tumor, Younger age, large tumor, low NE Large tumor low NE levels and high DA levels NE indicates norepinephrine; EPI, epinephrine; DA, dopamine; LNs, lymphatic nodes. patients with nonsyndromic pheochromocytoma, up to approx- imately 24% of tumors may be hereditary.6,11,12 Hereditary TABLE 2. Presence of Various Tumors in Common Hereditary pheochromocytoma is associated with multiple endocrine neo- Syndromes Associated With Pheochromocytoma or plasia type 2 (MEN-2A or MEN-2B), neurofibromatosis type 1 Paraganglioma (NF-1), von Hippel-Lindau (VHL) syndrome, and familial Paraganglioma syndromes (SDH) paragangliomas and pheochromocytomas due to germ-line mutations of genes encoding succinate dehydrogenase subunits SDHD Head and neck paraganglioma B, C, and D (SDHB, SDHC, SDHD) (Tables 1 and 2). In general, Pheochromocytoma the traits are inherited in an autosomal dominant pattern.12 Extra-adrenal paraganglioma* The panel at the First International Symposium on Pheo- SDHB Head and neck paraganglioma chromocytoma recommended that it is neither appropriate nor Pheochromocytoma currently cost-effective to test every disease-causing gene in Extra-adrenal paraganglioma* every patient with a pheochromocytoma and paraganglioma. To Renal carcinoma choose a proper genetic test, the biochemical profile of cate- MEN-2 cholamine secretion, age of the patient, localization of the pri- Type 2A Medullary thyroid carcinoma mary tumor, and previous family history must be carefully Pheochromocytoma evaluated and included in the genetic algorithm. Specifically, MEN-2Y and NF-1Yrelated pheochromocytoma always secrete Hyperparathyroidism epinephrine; VHL-related pheochromocytomas always secrete Cutaneous lichen amyloidosis norepinephrine; and elevation of dopamine together with nor- Type 2b Medullary thyroid carcinoma epinephrine is seen in some SDHB-related paragangliomas. In Pheochromocytoma contrast to MEN-2, VHL, and NF-1 tumors that are almost al- Multiple neuromas ways found in the adrenal gland, SDHB-related tumors are found Marfanoid habitus in extra-adrenal localizations. In those patients with malignant FMTC Familial medullary thyroid carcinoma disease secondary to an extra-adrenal paraganglioma, almost 13 VHL syndrome type 2 50% had SDHB mutations. Some studies suggested that more Type 2A Retinal and central nervous than two thirds of patients with SDHB-related pheochromocy- 14,15 system hemangioblastomas toma or paraganglioma will develop metastatic disease. Pheochromocytoma Family history is often helpful in MEN-2, VHL, and NF-1 Endolymphatic sac tumors tumors, but only 10% of the currently investigated patients with SDHB mutations have a positive
Load more

Recommended publications
  • Endo4 PRINT.Indb
    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
    [Show full text]
  • Thyroid Research Biomed Central
    Thyroid Research BioMed Central Case report Open Access Solitary intrathyroidal metastasis of renal clear cell carcinoma in a toxic substernal multinodular goiter Gianlorenzo Dionigi*1, Silvia Uccella2, Myriam Gandolfo3, Adriana Lai3, Valentina Bertocchi1, Francesca Rovera1 and Maria Laura Tanda3 Address: 1Department of Surgical Sciences, University of Insubria, Varese, Italy, 2Human Morphology, University of Insubria, Varese, Italy and 3Clinical Medicine, University of Insubria, Varese, Italy Email: Gianlorenzo Dionigi* - [email protected]; Silvia Uccella - [email protected]; Myriam Gandolfo - [email protected]; Adriana Lai - [email protected]; Valentina Bertocchi - [email protected]; Francesca Rovera - [email protected]; Maria Laura Tanda - [email protected] * Corresponding author Published: 24 October 2008 Received: 29 May 2008 Accepted: 24 October 2008 Thyroid Research 2008, 1:6 doi:10.1186/1756-6614-1-6 This article is available from: http://www.thyroidresearchjournal.com/content/1/1/6 © 2008 Dionigi et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Abstract Introduction: Thyroid gland is a rare site of clinically detectable tumor metastasis. Case report: A 71-year-old woman was referred to our department for an evaluation of toxic multinodular substernal goiter. She had a history of renal clear cell carcinoma of the left kidney, which had been resected 2 years previously. US confirmed the multinodular goiter. Total thyroidectomy with neuromonitoring was performed on March 2008.
    [Show full text]
  • A Comprehensive Review on MEN2B
    25 2 Endocrine-Related F Castinetti et al. Multiple endocrine neoplasia 25:2 T29–T39 Cancer type 2B THEMATIC REVIEW A comprehensive review on MEN2B Frederic Castinetti1, Jeffrey Moley2, Lois Mulligan3 and Steven G Waguespack4 1Department of Endocrinology, Aix Marseille University, CNRS UM 7286, Assistance Publique Hopitaux de Marseille, Marseille, France 2Department of Surgery, Washington University School of Medicine, St Louis, Missouri, USA 3Division of Cancer Biology and Genetics, Cancer Research Institute, Queen’s University, Kingston, Ontario, Canada 4Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA Correspondence should be addressed to F Castinetti: [email protected] This paper is part of a thematic review section on 25 Years of RET and MEN2. The guest editors for this section were Lois Mulligan and Frank Weber. Abstract MEN2B is a very rare autosomal dominant hereditary tumor syndrome associated with Key Words medullary thyroid carcinoma (MTC) in 100% cases, pheochromocytoma in 50% cases and f medullary thyroid cancer multiple extra-endocrine features, many of which can be quite disabling. Only few data f pheochromocytoma are available in the literature. The aim of this review is to try to give further insights into f ganglioneuromas the natural history of the disease and to point out the missing evidence that would help f RET clinicians optimize the management of such patients. MEN2B is mainly characterized by f marfanoid the early occurrence of MTC, which led the American Thyroid Association to recommend preventive thyroidectomy before the age of 1 year. However, as the majority of mutations are de novo, improved knowledge of the nonendocrine signs would help to lower the age of diagnosis and improve long-term outcomes.
    [Show full text]
  • (MEN2) the Risk
    What you should know about Multiple Endocrine Neoplasia Type 2 (MEN2) MEN2 is a condition caused by mutations in the RET gene. Approximately 25% (1 in 4) individuals with medullary thyroid cancer have a mutation in the RET gene. Individuals with RET mutations may also develop tumors in their parathyroid and adrenal glands (pheochromocytoma). There are three types of MEN2, based on the family history and specific mutation found in the RET gene: • MEN2A is the most common type of MEN2, with medullary thyroid cancer developing in young adulthood. MEN2A is also associated with adrenal and parathyroid tumors. • MEN2B is the most aggressive form of MEN2, with medullary thyroid cancer developing in early childhood. MEN2B is associated with adrenal tumors, but parathyroid tumors are rare. Individuals with MEN2B can also develop benign nodules on their lips and tongue, abnormalities of the gastrointestinal tract, and are usually tall in comparison to their family members. • Familial Medullary Thyroid Cancer (FMTC) is characterized by medullary thyroid cancer (usually in young adulthood) without adrenal or parathyroid tumors. The risk for cancer associated with MEN2 • MEN2A is associated with a ~100% risk for medullary thyroid cancer; 50% risk of adrenal tumors; and 25% risk of parathyroid tumors • MEN2B is associated with a 100% risk for medullary thyroid cancer; 50% risk of adrenal tumors; and rare risk of parathyroid tumors • FMTC is associated with ~ 100% risk for medullary thyroid cancer; and no risk for adrenal or parathyroid tumors Tumors that develop in the adrenal glands in individuals with MEN2 are typically not cancerous, but can produce excessive amounts of hormones called catecholamines, which can cause very high blood pressure.
    [Show full text]
  • Genetic Landscape of Papillary Thyroid Carcinoma and Nuclear Architecture: an Overview Comparing Pediatric and Adult Populations
    cancers Review Genetic Landscape of Papillary Thyroid Carcinoma and Nuclear Architecture: An Overview Comparing Pediatric and Adult Populations 1, 2, 2 3 Aline Rangel-Pozzo y, Luiza Sisdelli y, Maria Isabel V. Cordioli , Fernanda Vaisman , Paola Caria 4,*, Sabine Mai 1,* and Janete M. Cerutti 2 1 Cell Biology, Research Institute of Oncology and Hematology, University of Manitoba, CancerCare Manitoba, Winnipeg, MB R3E 0V9, Canada; [email protected] 2 Genetic Bases of Thyroid Tumors Laboratory, Division of Genetics, Department of Morphology and Genetics, Universidade Federal de São Paulo/EPM, São Paulo, SP 04039-032, Brazil; [email protected] (L.S.); [email protected] (M.I.V.C.); [email protected] (J.M.C.) 3 Instituto Nacional do Câncer, Rio de Janeiro, RJ 22451-000, Brazil; [email protected] 4 Department of Biomedical Sciences, University of Cagliari, 09042 Cagliari, Italy * Correspondence: [email protected] (P.C.); [email protected] (S.M.); Tel.: +1-204-787-2135 (S.M.) These authors contributed equally to this paper. y Received: 29 September 2020; Accepted: 26 October 2020; Published: 27 October 2020 Simple Summary: Papillary thyroid carcinoma (PTC) represents 80–90% of all differentiated thyroid carcinomas. PTC has a high rate of gene fusions and mutations, which can influence clinical and biological behavior in both children and adults. In this review, we focus on the comparison between pediatric and adult PTC, highlighting genetic alterations, telomere-related genomic instability and changes in nuclear organization as novel biomarkers for thyroid cancers. Abstract: Thyroid cancer is a rare malignancy in the pediatric population that is highly associated with disease aggressiveness and advanced disease stages when compared to adult population.
    [Show full text]
  • Validation of Algorithms to Identify Pancreatic Cancer and Thyroid Neoplasms from Health Insurance Claims Data in a 10-Year Foll
    Validation of Algorithms to Identify Pancreatic Cancer and Thyroid Neoplasms From Health Insurance Claims Data in a 10-Year Follow-Up Study Caihua Liang, MD, PhD1*; Monica L Bertoia, MPH, PhD1; C Robin Clifford, MS1; Yan Ding, MSc1; Qing Qiao, MD, PhD2; Joshua J Gagne, PharmD, ScD1,3; David D Dore, PharmD, PhD1 1Optum Epidemiology, Boston, MA/Ann Arbor, MI, USA; 2Global Medical Affair AstraZeneca, Gothenburg, Sweden; 3Division of Pharmacoepidemiology, Department of Medicine, Brigham and Women’s Hospital and Harvard Medical School, Boston, USA. *Corresponding author: [email protected]. This study was funded through a research contract between Optum Epidemiology and AstraZeneca. Background Methods (cont.) Identification of cancer events in health insurance claims data can be Figure 1. Pancreatic cancer and thyroid neoplasm relaxed and restricted challenging and subject to misclassification. Given the low incidence of algorithms certain cancers, robust performance evaluation requires a large sample size PANCREATIC CANCER THYROID NEOPLASMS with long-term follow-up. ICD-9 Codes ICD-9 Codes (Malignant neoplasm of …) 193 Malignant neoplasm of thyroid gland 157.X … pancreas 226 Benign neoplasm of thyroid gland Objective 157.0 … head of pancreas 157.1 … body of pancreas Thyroid Cancer Benign Thyroid Neoplasm To validate algorithms for potential incident pancreatic cancer and thyroid 157.2 … tail of pancreas neoplasms in a 10-year follow-up study using a health insurance claims 157.3 … pancreatic duct Restricted Algorithm: Restricted Algorithm: 157.4 … islets of Langerhans a+b+c a+b+c database. 157.8 … other specified sites of Relaxed Algorithm: Relaxed Algorithm: pancreas a+b or a+c a+b or a+c 157.9 … pancreas, part unspecified Data Source a.
    [Show full text]
  • Current and Future Role of Tyrosine Kinases Inhibition in Thyroid Cancer: from Biology to Therapy
    International Journal of Molecular Sciences Review Current and Future Role of Tyrosine Kinases Inhibition in Thyroid Cancer: From Biology to Therapy 1, 1, 1,2,3, 3,4 María San Román Gil y, Javier Pozas y, Javier Molina-Cerrillo * , Joaquín Gómez , Héctor Pian 3,5, Miguel Pozas 1, Alfredo Carrato 1,2,3 , Enrique Grande 6 and Teresa Alonso-Gordoa 1,2,3 1 Medical Oncology Department, Hospital Universitario Ramón y Cajal, 28034 Madrid, Spain; [email protected] (M.S.R.G.); [email protected] (J.P.); [email protected] (M.P.); [email protected] (A.C.); [email protected] (T.A.-G.) 2 The Ramon y Cajal Health Research Institute (IRYCIS), CIBERONC, 28034 Madrid, Spain 3 Medicine School, Alcalá University, 28805 Madrid, Spain; [email protected] (J.G.); [email protected] (H.P.) 4 General Surgery Department, Hospital Universitario Ramón y Cajal, 28034 Madrid, Spain 5 Pathology Department, Hospital Universitario Ramón y Cajal, 28034 Madrid, Spain 6 Medical Oncology Department, MD Anderson Cancer Center, 28033 Madrid, Spain; [email protected] * Correspondence: [email protected] These authors have contributed equally to this work. y Received: 30 June 2020; Accepted: 10 July 2020; Published: 13 July 2020 Abstract: Thyroid cancer represents a heterogenous disease whose incidence has increased in the last decades. Although three main different subtypes have been described, molecular characterization is progressively being included in the diagnostic and therapeutic algorithm of these patients. In fact, thyroid cancer is a landmark in the oncological approach to solid tumors as it harbors key genetic alterations driving tumor progression that have been demonstrated to be potential actionable targets.
    [Show full text]
  • Follicular Variant of Papillary Thyroid Cancer Encapsulated, Nonencapsulated, and Diffuse: Distinct Biologic and Clinical Entities
    ORIGINAL ARTICLE Follicular Variant of Papillary Thyroid Cancer Encapsulated, Nonencapsulated, and Diffuse: Distinct Biologic and Clinical Entities Sachin Gupta, MD; Oluyomi Ajise, MD; Linda Dultz, MD; Beverly Wang, MD; Daisuke Nonaka, MD; Jennifer Ogilvie, MD; Keith S. Heller, MD; Kepal N. Patel, MD Objective: To examine genotypic and clinical differ- tions in BRAF, H-RAS 12/13, K-RAS 12/13, N-RAS 12/13, ences between encapsulated, nonencapsulated, and dif- H-RAS 61, K-RAS 61, N-RAS 61, and RET/PTC1. fuse follicular variant of papillary thyroid carcinoma (EFVPTC, NFVPTC, and diffuse FVPTC, respectively), Results: No patient with EFVPTC had central lymph node to characterize the entities and identify predictors of their metastasis, and in this group, 1 patient (4.5%) had a BRAF behavior. V600E mutation and 2 patients (9%) had RAS mutations. Of the patients with NFVPTC, none had central lymph Design: Retrospective medical chart review and mo- node metastasis (PϾ.99) and 2 (11%) had a BRAF V600E lecular analysis. mutation (P=.59). Of the patients with diffuse FVPTC, all had central lymph node metastasis (PϽ.001), and 2 (50%) Setting: Referral center of a university hospital. had a BRAF V600E mutation (P=.06). Patients: The pathologic characteristics of 484 con- Conclusions: FVPTC consists of several distinct sub- secutive patients with differentiated thyroid cancer who types. Diffuse FVPTC seems to present and behave in a underwent surgery by the 3 members of the New York more aggressive fashion. It has a higher rate of central University Endocrine Surgery Associates from January nodal metastasis and BRAF V600E mutation in compari- 1, 2007, to August 1, 2010, were reviewed.
    [Show full text]
  • Follicular Variant of Papillary Thyroid Carcinoma Arising from a Dermoid Cyst: a Rare Malignancy in Young Women and Review of the Literature
    View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Elsevier - Publisher Connector Available online at www.sciencedirect.com Taiwanese Journal of Obstetrics & Gynecology 51 (2012) 421e425 www.tjog-online.com Case Report Follicular variant of papillary thyroid carcinoma arising from a dermoid cyst: A rare malignancy in young women and review of the literature Cem Dane a,*, Murat Ekmez a, Aysegul Karaca b, Aysegul Ak c, Banu Dane d a Department of Gynecology and Obstetrics, Haseki Training and Research Hospital, Istanbul, Turkey b Department of Family Medicine, Haseki Training and Research Hospital, Istanbul, Turkey c Department of Pathology, Haseki Training and Research Hospital, Istanbul, Turkey d Department of Gynecology and Obstetrics, Bezmialem Vakif University, Faculty of Medicine, Istanbul, Turkey Accepted 3 November 2011 Abstract Objective: Benign or mature cystic teratomas, also known as dermoid cysts, are composed of mature tissues, which can contain elements of all three germ cell layers. Malignant transformation of a mature cystic teratoma is more common in postmenopausal women, however, it can also, rarely, be identified in younger women. We present a case of a 19-year-old woman with malignant transformation of an ovarian mature cystic teratoma. Case Report: Our case was a 19-year-old woman, who was diagnosed postoperatively with follicular variant of papillary thyroid carcinoma in a mature cystic teratoma. She underwent right cystectomy for adnexal mass. Postoperative metastatic workup revealed a non-metastatic disease and the patient did not undergo any further treatment. After 2 months, a near-total thyroidectomy was performed. Serum thyroglobulin levels were monitored on follow-up and the patient is asymptomatic.
    [Show full text]
  • Multiple Endocrine Neoplasia Type 2: an Overview Jessica Moline, MS1, and Charis Eng, MD, Phd1,2,3,4
    GENETEST REVIEW Genetics in Medicine Multiple endocrine neoplasia type 2: An overview Jessica Moline, MS1, and Charis Eng, MD, PhD1,2,3,4 TABLE OF CONTENTS Clinical Description of MEN 2 .......................................................................755 Surveillance...................................................................................................760 Multiple endocrine neoplasia type 2A (OMIM# 171400) ....................756 Medullary thyroid carcinoma ................................................................760 Familial medullary thyroid carcinoma (OMIM# 155240).....................756 Pheochromocytoma ................................................................................760 Multiple endocrine neoplasia type 2B (OMIM# 162300) ....................756 Parathyroid adenoma or hyperplasia ...................................................761 Diagnosis and testing......................................................................................756 Hypoparathyroidism................................................................................761 Clinical diagnosis: MEN 2A........................................................................756 Agents/circumstances to avoid .................................................................761 Clinical diagnosis: FMTC ............................................................................756 Testing of relatives at risk...........................................................................761 Clinical diagnosis: MEN 2B ........................................................................756
    [Show full text]
  • Cancer Mortality in Women with Thyroid Disease1
    (CANCER RESEARCH 50. 228.1-2289. April 15. 1990| Cancer Mortality in Women with Thyroid Disease1 Marlene B. Goldman,2 Richard R. Monson, and 1aralio Maloof Department of Epidemiology, Harvard School of Public Health, Boston 02115 [M. B. 6"..R. K. M.J, and Thyroid I'nil, Massachusetts Ornerai Hospital, Boston 02114 /F. M.I, Massachusetts ABSTRACT in a study of American women (4). A mechanism for a causal relationship between the two diseases is not established, al A retrospective follow-up study of 7338 women with either nontoxic though thyroid hormones are known to influence the breast nodular goiter, thyroid adenoma, hyperthyroidism, hypothyroidism, Hashimoto's thyroiditis, or no thyroid disease was conducted. All women either directly or through effects on thyroid-stimulating hor patients at the Massachusetts General Hospital Thyroid Clinic who were mone, prolactin, estrogens, or androgens. Researchers sug seen between 1925 and 1974 and who were treated for a minimum of 1 gested that a deficit of thyroid hormone altered the hormonal year were traced. A total of 2231 women (30.4%) were dead and 2012 milieu in a way that permitted the growth of malignant cells (1, women (27.4%) were alive as of December 31, 1978. Partial follow-up 5, 6). It is just as reasonable to hypothesize, however, that an information was available for the remaining 3095 women (42.2%). The excess of thyroid hormone may promote tumor growth. A average length of follow-up was 15.2 years. When losses to follow-up number of biochemical and clinical studies have been con were withdrawn at the time of their loss, the standardized mortality ratios ducted, but no consensus has been reached as to the role of (SMR) for all causes of death were 1.2 |95% confidence interval (CI), thyroid hormones in the initiation or promotion of cancer.
    [Show full text]
  • Classification and General Considerations of Thyroid Cancer
    Central Annals of Clinical Pathology Review Article *Corresponding author Hiroshi Katoh, Department of Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Classification and General Sagamihara, 252-0374, Japan, Tel: 81-42-778-8735; Fax:81-42-778-9556; Email: Submitted: 22 December 2014 Considerations of Thyroid Accepted: 12 March 2015 Published: 13 March 2015 Cancer ISSN: 2373-9282 Copyright Hiroshi Katoh*, Keishi Yamashita, Takumo Enomoto and © 2015 Katoh et al. Masahiko Watanabe OPEN ACCESS Department of Surgery, Kitasato University School of Medicine, Japan Keywords Abstract • Thyroid cancer • Pathological classification Thyroid cancer is the most common malignancy in endocrine system, composed of • Genetic alteration four major types; papillary thyroid carcinoma, follicular thyroid carcinoma, anaplastic thyroid carcinoma, and medullary thyroid carcinoma. The incidence of thyroid cancer, especially differentiated thyroid cancer, is increasing in developed countries. Growing body of studies on molecular pathogenesis in thyroid cancer provide clues for further research and diagnostic/therapeutic targets. The general pathological classifications and clinical features of follicular cell derived thyroid carcinomas are overviewed, and recent advances of genetic alterations are discussed in this review. ABBREVIATIONS growth. PTC consists of 85-90% of all thyroid cancer cases, followed by FTC (5-10%) and MTC (about 2%). ATC accounts for PTC: Papillary Thyroid Cancer; FTC: Follicular Thyroid less than 2% of thyroid cancers and typically arises in the elder Cancer; ATC: Anaplastic Thyroid Cancer; MTC: Medullary patients. Its incidence continues to rise with age. The mechanism Thyroid Cancer; PDTC: Poorly Differentiated Thyroid Cancer; of MTC carcinogenesis is the activation of RET signaling caused DTC: Differentiated Thyroid Cancer by RET mutations [6], which are not observed in follicular INTRODUCTION thyroid cell derived cancers.
    [Show full text]