DOCSLIB.ORG

Endoperspectives, Volume 3, Issue 1

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Endoperspectives, Volume 3, Issue 1 Department of Endocrine Neoplasia and Hormonal Disorders NEWSLETTER Volume 3, Issue 1, 2010 Pediatric Endocrine Tumors ® Steven G. Waguespack, M.D., FAAP, FACE Associate Professor Department of Endocrine Neoplasia and Hormonal Disor- ders; Division of Pediatrics Endocrine tumors Pediatric Endocrine Tumors, An Overview represent a minority of Pituitary Tumors all neoplasms observed Pituitary adenomas (PA) represent less than in the pediatric popu- 3% of all supratentorial tumors diagnosed dur- lation and are gener- ing childhood. Over 75% of PAs occur in children ally clinically benign older than 12 years, and females are more com- or low-grade cancers, monly affected than males. Pituitary tumors are although a small percentage of these tumors are usually diagnosed secondary to symptoms of high-grade malignancies requiring multimodal- hormone excess or mass effect, such as visual ity therapies. Although rare, pediatric endocrine disturbance (classically a bitemporal hemiano- tumors are important clinical entities that can psia), headache, or ophthalmoplegia. Patients have unique clinical presentations and genet- may also present with delayed growth and pu- ic causes. As with any rare childhood disease, bertal development due to pituitary hormone treatment is best provided at referral centers hypersecretion, as seen with hyperprolactine- with multidisciplinary expertise in the manage- mia, or hyposecretion, as seen with large PAs ment of such tumors. The Pediatric Endocrine that compress the normal adenohypophyseal Tumor Program at the Children’s Cancer Hos- tissue. The initial workup of a PA is similar to pital of the University of Texas M. D. Anderson adults and includes imaging of the sella turcica, Cancer Center is a multidisciplinary program a comprehensive hormonal evaluation to assess that provides primary evaluation and treat- for hyper- and hypopituitarism, and visual field ment to children and young adults suspected testing for children with large tumors that ap- PERSPECTIVES to have or who are already diagnosed with thy- proach the optic chiasm. Prolactinomas are the roid neoplasms, adrenal tumors, pituitary ad- most common PA in childhood, representing enomas, and/or hereditary tumor syndromes. about 50% of cases, followed by ACTH-secret- Our team currently consists of two board-cer- ing adenomas (Cushing disease), somatotroph tified adult and pediatric endocrinologists, Ste- adenomas (gigantism/acromegaly), and lastly, ven Waguespack, M.D. and Anita Ying, M.D., and clinically nonfunctioning PAs. In contradistinc- Sarah Bottomley, RN, MSN, CPNP, a pediatric tion to adults, (continued on Page 2) nurse practitioner who works closely with both Drs. Waguespack and Ying. Table of Contents This issue of EndoPerspectives is focused on pediatric endocrine tumors. We start with a general overview of the most common endo- Pediatric Endocrine Tumors Page 1 crine neoplasms encountered in the pediatric Upcoming Events Page 2 population. Dr. Ying and Ms. Bottomley review Notes from the Endocrine Faculty Page 4 late effects of endocrine tumors and their treat- Team ment. Thereasa Rich, M.S., C.G.C., reviews the MEN Patient Education Conference Page 4 genetic syndromes most commonly associated ENDO Late Effects of Endocrine Tumors Page 5 with pediatric endocrine tumors and emphasiz- Genetic Counseling and Genetic As- es the role of genetic counseling when taking care of these children. pects of Pediatric Endocrine Tumors Page 6 The Thyroid Nodule Clinic Page 8 (Waguespack, continued from Page 1) oncogene: multiple endocrine neoplasia type nonfunctioning PAs represent only <5% of 2a (MEN2A) and type 2b (MEN2B). In addition Upcoming Events pediatric pituitary tumors. Although the clini- to the almost complete penetrance of MTC, cal presentation of PAs during childhood is 50% of patients with MEN2A and MEN2B de- AACE 19th Annual Meet- often different compared with adults, the ap- velop pheochromocytomas and up to 30% of ing and Clinical Congress proach to diagnosis and treatment of these MEN2A patients have parathyroid adenomas, April 21-25, 2010 neoplasms is similar. although these tumors are rarely diagnosed Sheraton Boston Hotel and during childhood. Patients with MEN2B have the John B. Hynes Veterans a distinct phenotype with a characteristic fa- Memorial Convention Center cial appearance, Marfanoid body habitus, and Boston, MA (http://www. a generalized ganglioneuromatosis, mani- aace.com/meetings/cal- fested most obviously by the presence of oral endar/calendar.php) mucosa neuromas. LWPES 2010 Annual Meet- Children with thyroid cancer usually pres- ing May 1-3, 2010. Vancou- ent with an asymptomatic thyroid mass and/ ver, Canada. (http://www. or cervical lymphadenopathy. Lymph node lwpes.org/meetingsEvents/ metastases are present in the majority of PTC pdf/2010LWPESschedule. cases and lung metastases are identified in pdf) up to 20% of cases; metastases to other sites occur but are rare. ATA Spring Meeting of the American Thyroid Association May 13- 16, 2010. Minneapolis, MN (www.thyroid.org) PTC 8-year-old with Cushing disease and a cys- The Endocrine Society tic pituitary macroadenoma (arrows). LN ENDO 2010, June 19-22, LN 2010. San Diego, CA. Thyroid Tumors (www.endo-society.org) Benign Thyroid Tumors Benign thyroid tumors represent up to 80% 14th International Thy- of all thyroid nodules presenting in the pedi- roid Congress Sept. 11- atric population. The younger a child is at the 16, 2010. Paris, France. (www.itc2010.com) time of presentation, the more likely it is for a solitary nodule to be malignant. The work North American Neuro- up of a thyroid nodule in a child includes the Endocrine Tumor Society laboratory assessment of thyroid function, Annual Conference ultrasound to assess the nodule and regional CT scan of a 6-year-old with papillary thyroid October 29-30, 2010. lymph node characteristics, and fine needle cancer (PTC) and bulky bilateral lymph node Santa Fe, New Mexico aspiration (usually under ultrasound guid- (LN) metastases. (www.nanets.net) ance) for definitive diagnosis. Solid lesions with increased blood flow and the presence Despite a more advanced presentation, 14th Asia-Oceania Con- of microcalcifications are more likely to be children with thyroid cancer usually have an gress of Endocrinology malignant whereas pure cystic lesions are excellent prognosis with anticipated survival December 2-5, 2010. over decades. Part of this clinical phenom- Kuala Lumpur, Malaysia almost always benign. Nuclear scintigraphy (www.aoce2010.com) with 123I or Tc-99m pertechnetate is gener- enon may be due to mutational differences ally not useful in the diagnostic evaluation, between children and adults, in addition to MEN 2010 12th Interna- except in the event of a suppressed TSH. the fact that pediatric PTC is usually very io- tional Workshop on Mul- Malignant Thyroid Tumors dine avid, typically leading to successful treat- tiple Endocrine Neoplasia Malignant neoplasms of the thyroid are ment of distantly metastatic disease after one Sept. 16-18, 2010. Gubbio, Italy (www.men2010.com) rare in the pediatric population, with an in- or more courses of radioactive iodine (RAI) cidence of ≤1 case/million/year in children therapy. under ten years of age to 15.4 cases/million/ Total thyroidectomy and a compartment-ori- year in adolescents ages 15-19, which is the ented lymph node dissection, as indicated, are most commonly affected pediatric age group. best accomplished by surgeons with extensive Over 90% of thyroid cancers occurring in experience in the management of thyroid ma- childhood are papillary thyroid cancer (PTC). lignancies. In DTC, radioiodine (using 131I) is Medullary thyroid carcinoma (MTC) is a very often administered a few weeks after surgery uncommon disease in childhood that almost to ablate any residual normal thyroid tissue always occurs in the context of one of two and treat any residual thyroid cancer that is hereditary endocrine tumor syndromes that iodine-avid; children with MTC do not require arise secondary to activating mutations of the radioiodine ablation. The thyroid stimulating RET (REarranged during Transfection) proto- hormone (TSH) level is suppressed by giving Continued on Page 3 2 (Waguespack, continued from Page 1) to present at an age <5 years. They have a female predomi- supraphysiologic levothyroxine doses in DTC but the TSH is nance and are functional tumors (producing androgens and/ kept normal in MTC. Long-term follow up involves monitor- or glucocorticoids, typically) in >90% of cases. ACT may also ing of tumor markers (thyroglobulin in DTC and calcitonin/ present as an abdominal mass or pain. In children, ACT are as- carcinogenic embryonic antigen in MTC) as well as routine sociated with the Li-Fraumeni syndrome (germline inactivat- imaging based upon the initial extent of disease presenta- ing mutations in the p53 tumor suppressor gene), the Beck- tion. Neck US is generally the most useful imaging modality. with-Wiedemann syndrome (BWS), hemihypertrophy other Traditional chemotherapy has generally not been shown to than that seen as part of BWS, and rarely congenital adrenal be effective in thyroid cancer, but newer anti-cancer agents, hyperplasia. Other rare causes of nodular adrenocortical dis- the oral tyrosine kinase inhibitors, are showing promise in the ease, which usually present with Cushing syndrome, include treatment of children with disease not amenable or respon- Carney’s complex and macronodular adrenal hyperplasia. sive to standard therapeutic approaches. PHEO
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]
  • 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]
  • 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]
  • 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]
  • Pathology Clinic Medullary Thyroid Carcinoma
    Pathology Clinic Medullary thyroid carcinoma. by Lester D. R. Thompson, MD Medullary thyroid carcinoma (MTC) is a malignant epithelial tumor of the thyroid gland that exhibits C-cell differentiation. C cells arise from the ultimobranchial body, which is derived from the fourth pharyngeal pouch, and they are found in the upper and middle areas of the thyroid lobes. These cells produce calcitonin, a hormone involved in calcium homeostasis. While a number (20%) of MTCs are associated with the autosomal-dominant inherited multiple endocrine neoplasia (MEN) syndromes (specifically MEN2A and MEN2B), most (80%) cases are sporadic. Germline or somatic mutations of the RET gene are characteristic of this tumor. They usually involve an activating point mutation of 10q11.2. Specifically, codon 634 in exon 11 is most common in MEN2A, while codon 918 in exon 16 is most common in MEN2B. MTC accounts for approximately 5 to 8% of all thyroid malignancies. Its affects patients of a wide age range; familial MTC tends to occur at an earlier age and sporadic MTC at a later age. Patients typically present with a painless thyroid mass-usually bilateral or multicentric in familial cases and unilateral in sporadic cases. Cervical lymph node enlargement is seen in as many as 50% of patients at presentation. In familial cases, it is important to exclude extrathyroidal findings, such as hyperparathyroidism; symptoms referable to pheochromocytoma; pituitary and pancreatic dysfunction; and mucosal neuromas. Serum calcitonin levels are almost invariably increased, as are carcinoembryonic antigen (CEA) levels. Prophylactic thyroidectomy is recommended for patients with germline RET mutations, and thyroidectomy with or without neck dissection is recommended for other patients.
    [Show full text]
  • Thyroid Paraganglioma: an Extremely Rare Tumor of the Thyroid
    Case Report Annals of Clinical Case Reports Published: 24 Nov, 2016 Thyroid Paraganglioma: An Extremely Rare Tumor of the Thyroid Adnan Özpek1*, Gözde Kir2, Hüseyin Kerem Tolan1, Metin Yucel1, Kemal Tekesin3, Gürhan Bas1 and Orhan Alimoglu4 1Department of General Surgery, Umraniye Training and Research Hospital, Turkey 2Department of Pathology, Umraniye Training and Research Hospital, Turkey 3Department of General Surgery, Usküdar State Hospital, Turkey 4Department of General Surgery, Istanbul Medeniyet University, Turkey Abstract Background: Paraganglioma originates from the para-ganglionic system which is mostly located in the adrenal gland medulla is a rare condition seen in the thyroid gland accounting for the 0.1% of all the thyroid malignancies. Case Presentation: A 67 years old female patient was admitted to our out-patient clinic with a swelling around the neck and an unexplained chronic cough. The ultrasound (US) revealed a 33 x 20 mm hypoechoic nodule on the right lobe of the thyroid gland without any cervical lymph node enlargement. Fine Needle Aspiration Biopsy (FNAB) was performed from the dominant nodule with the guidance of US. In the cytopathology report there was a suspicion of neuro-endocrine tumor. In the operation the nodule was found to be fixed to the adjacent trachea and a Total Thyroidectomy was performed to the patient. Finally, in the histopathology report the diagnosis was Thyroid Paraganglioma (TP). Screening of the patient 29 months after the operation and no metastasis or local recurrences were seen in the ultrasound and PET scan (F-18 FDG). Tests performed for the detection of catecholamine secretion were found to be in normal limits.
    [Show 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.
    [Show full text]
  • Medullary Thyroid Cancer Handbook
    Medullary Thyroid Cancer www.thyca.org Medullary2 Thyroid Cancer This handbook provides an overview of medullary thyroid cancer, its diagnosis, and typical treatment options, advances in research, and how to find a specialist experienced with this rare type of thyroid cancer. We also tell you about free support services, educational events, and more resources. Our goal is help patients and caregivers cope with the emotional and practical impacts of this disease. While this handbook contains important information about medullary thyroid cancer, your individual course of testing, treatment, and follow- up may vary for many reasons. This free handbook is one in a series, for people with all types of thyroid cancer. Others now available from ThyCa are Thyroid Cancer Basics (about all types of thyroid cancer) and Anaplastic Thyroid Cancer. More handbooks are in development. Thank you to our physician and thyroid scientist reviewers, our publication volunteers, and our donors. Thank you to the physicians on our Medical Advisory Council and to the many other thyroid cancer specialist physicians and thyroid researchers who reviewed and provided content for this publication. Thank you to our generous donors for their support, to the volunteers in ThyCa’s Medullary Thyroid Cancer E-Mail Support Group, and to our publications committee, who all contribute time and input. We greatly appreciate everyone’s efforts. ThyCa's free support services and publications, including this handbook, are made possible by the generous support of our volunteers, members and individual contributors, and by unrestricted educational grants from AstraZeneca, Bayer HealthCare, Eisai, Exelixis, Inc., Genzyme, Onyx Pharmaceuticals, OXiGENE, and Veracyte.
    [Show full text]
  • About Thyroid Cancer Overview and Types
    cancer.org | 1.800.227.2345 About Thyroid Cancer Overview and Types If you have been diagnosed with thyroid cancer or are worried about it, you likely have a lot of questions. Learning some basics is a good place to start. ● What Is Thyroid Cancer? Research and Statistics See the latest estimates for new cases of thyroid cancer and deaths in the US and what research is currently being done. ● Key Statistics for Thyroid Cancer ● What’s New in Thyroid Cancer Research? What Is Thyroid Cancer? Thyroid cancer is a type of cancer that starts in the thyroid gland. Cancer starts when cells begin to grow out of control. (To learn more about how cancers start and spread, see What Is Cancer?1) The thyroid gland makes hormones that help regulate your metabolism, heart rate, blood pressure, and body temperature. 1 ____________________________________________________________________________________American Cancer Society cancer.org | 1.800.227.2345 Where thyroid cancer starts The thyroid gland is in the front part of the neck, below the thyroid cartilage (Adam’s apple). In most people, the thyroid cannot be seen or felt. It is shaped like a butterfly, with 2 lobes — the right lobe and the left lobe — joined by a narrow piece of gland called the isthmus(see picture below). The thyroid gland has 2 main types of cells: ● Follicular cells use iodine from the blood to make thyroid hormones, which help regulate a person’s metabolism. Having too much thyroid hormone (hyperthyroidism) can cause a fast or irregular heartbeat, trouble sleeping, nervousness, hunger, weight loss, and a feeling of being too warm.
    [Show full text]