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- Herausgeber: John Wiley & Sons
- Kategorie: Fachliteratur
- Sprache: Englisch
Imaging in Endocrinology will provide endocrinologists and radiologists of all levels with an outstanding diagnostic imaging atlas to aid them in the diagnosis and management of all the major endocrine diseases they are likely to encounter.
In full colour throughout, the 300 high-quality images consist of CT scans, MRI, NMR and histopathology slides, and are arranged by each specific endocrine condition, resulting in a visually outstanding and easily accessible tool that guides the user through exactly what to look out for and provides a practical and extremely useful aid in helping them formulate a diagnosis.
Every major endocrine condition is covered in a specific section, including diseases of the thyroid, pituitary, reproductive and adrenal glands, the pancreas, bone metabolism problems, and the various forms of endocrine cancers. Each disease covered will offer a comparison of the normal findings so as to further assist in diagnosis. An accompanying website contains an online slide-atlas of all the figures in the book, to allow users to download all figures for use in presentations.
Led by Paolo Pozzilli, an internationally-recognised expert in this field, the authors have assembled a wonderful collection of images that will be greatly valued by endocrinologists and radiologists alike, ensuring this is the perfect tool to consult when assessing patients with endocrine disease.
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Seitenzahl: 342
Veröffentlichungsjahr: 2013
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Contents
Cover Page
Title Page
Copyright Page
About the Companion Website
Preface
Collaborators
Chapter 1: Thyroid
Hashimoto’s thyroiditis (chronic autoimmune thyroiditis)
Graves’ disease (Basedow’s disease)
Subacute thyroiditis (de Quervain’s thyroiditis)
Benign thyroid nodules
Thyroid cancer
Chapter 2: Pituitary Gland
Craniopharyngioma
Hypothalamic dysgerminoma
Growth hormone-secreting pituitary tumor
Prolactin-secreting pituitary tumor
Corticotropin-secreting pituitary tumor (Cushing syndrome)
Nelson syndrome
Clinically nonfunctioning pituitary tumors
Primary pituitary carcinoma
Pituitary cyst
Nontumorous lesions of the pituitary gland and pituitary stalk
Pituitary apoplexy
Tumors metastatic to the pituitary
Chapter 3: Adrenal Gland
Adrenal incidentaloma
Adrenocortical carcinoma
Adrenal-dependent Cushing syndrome
Classic congenital adrenal hyperplasia
Primary aldosteronism
Adrenal venous sampling
Primary adrenal failure (Addison’s disease)
Pheochromocytoma
Paraganglioma
Adrenal infarction
Tumors metastatic to the adrenal glands
Chapter 4: Pancreas
Diabetes
Insulinoma
Acute pancreatitis
Chronic pancreatitis
Chapter 5: Bone and Mineral Metabolism
Osteoporosis
Primary hyperparathyroidism
Hypoparathyroidism
Pseudohypoparathyroidism
Paget’s disease of bone
Osteomalacia
Chronic kidney disease
Fibrous dysplasia
Selected sclerosing bone disorders
Tumoral calcinosis
Osteogenesis imperfecta
Other skeletal disorders
Chapter 6: Gonads
Male gonads
Female gonads
Chapter 7: Mucocutaneous Manifestations of Endocrine Disorders
Acanthosis nigricans
Acne
Alopecia
Café-au-lait macules
Mucocutaneous neuromas
Necrolytic migratory erythema
Neurofibromas
Vitiligo
Index
This edition first published 2014 © 2014 by John Wiley & Sons, Ltd
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Library of Congress Cataloging-in-Publication Data
Pozzilli, Paolo, author. Imaging in endocrinology / Paolo Pozzilli, Andrea Lenzi, Bart L. Clarke, William F. Young Jr. p. ; cm. Includes bibliographical references and index. ISBN 978-0-470-65627-3 (cloth : alk. paper) – ISBN 978-1-118-74907-4 (epub) – ISBN 978-1-118-74908-1 – ISBN 978-1-118-74930-2 (emobi) – ISBN 978-1-118-74931-9 (epdf) I. Lenzi, Andrea, author. II. Clarke, Bart, author. III. Young, William F., Jr., 1951- author. IV. Title. [DNLM: 1. Endocrine System Diseases–Atlases. 2. Diagnostic Imaging–methods–Atlases. 3. Metabolic Diseases–Atlases. WK 17] RC649 616.40022′3–dc23 2013029209
A catalogue record for this book is available from the British Library.
Wiley also publishes its books in a variety of electronic formats. Some content that appears in print may not be available in electronic books.
Cover images: © the authors Cover designer: Visual Philosophy, Ltd., Oxford
About the Companion Website
This book is accompanied by a companion website: www.wiley.com\go\Pozzilli\endocrinemetabolicdisease
The website includes:
Powerpoints of all figures from the book for downloadingPreface
No medical discipline requires such a precise phenotypic classification and careful consideration of the “image” as does endocrinology. Indeed, it is from “observation” that the endocrinologist extrapolates the elements upon which he or she bases clinical reasoning in the identification of a medical condition.
This atlas aims to be a valuable guide in endocrine diagnosis – suitable for both specialists and physicians in training, as well as physicians in other disciplines with an interest in endocrine disorders. Using the image as a unifying theme, we address the most salient themes of the science of endocrinology – including thyroid, pituitary, adrenal, endocrine pancreas, bone and mineral metabolism, and gonads. Each section provides iconographic support for the pathologies examined.
The universal character of this atlas guarantees a high standard of quality. The work was carried out in both Italy and the USA and has the added benefit of combining the rigor and scientific integrity belonging to the cradle of modern endocrinology (Rome) and the clinical resources of a major quaternary endocrine referral center (Mayo Clinic).
We would like to express our gratitude to all those who collaborated on this project. Their passion and enthusiasm toward the completion of this work has been exceptional. Without their hard work and dedication, this publication would have not seen the light.
We would also like to thank Wiley and its editors, who have demonstrated, once again, the high level of professionalism and special attention to detail needed to successfully bring to fruition this type of publication. We hope you enjoy consulting the Atlas.
Paolo Pozzilli, Andrea Lenzi, Bart L Clarkeand William F Young JrJuly 2013
Collaborators
Giusy Beretta
(collaborator for Thyroid and Pancreas chapters) Senior Investigator Dept of Endocrinology and Diabetes University Campus Bio-Medico Rome, Italy
Daniele Gianfrilli
(collaborator for Gonads and Mucocutaneous Manifestations of Endocrine Disorders chapters) Senior Investigator in Endocrinology Dept of Experimental Medicine Section of Medical Pathophysiology, Food Science, and Endocrinology Sapienza University of Rome Rome, Italy
Elisa Giannetta
(collaborator for Gonads and Mucocutaneous Manifestations of Endocrine Disorders chapters) Senior Investigator in Endocrinology Dept of Experimental Medicine Section of Medical Pathophysiology, Food Science, and Endocrinology Sapienza University of Rome Rome, Italy
Andrea M. Isidori
(collaborator for Gonads and Mucocutaneous Manifestations of Endocrine Disorders chapters) Assistant Professor of Endocrinology Dept of Experimental Medicine Section of Medical Pathophysiology, Food Science, and Endocrinology Sapienza University of Rome Rome, Italy
Angelo Lauria
(collaborator for Thyroid and Pancreas chapters) Senior Investigator Dept of Endocrinology and Diabetes University Campus Bio-Medico Rome, Italy
Andrea Palermo
(collaborator for Thyroid and Pancreas chapters) Senior Investigator Dept of Endocrinology and Diabetes University Campus Bio-Medico Rome, Italy
Alberto Signore
(collaborator for Thyroid and Pancreas chapters) Professor, Dept of Nuclear Medicine Sapienza University II Medical Faculty Rome, Italy; and Dept of Nuclear Medicine University of Groningen, The Netherlands
1
Thyroid
Hashimoto’s thyroiditis (chronic autoimmune thyroiditis)
Definition and epidemiology
Hashimoto’s thyroiditis (HT), or chronic lymphocytic thyroiditis, is an autoimmune disease in which the thyroid gland is attacked by a variety of cell and antibody-mediated immune processes. The name “Hashimoto’s thyroiditis” is derived from the 1912 original report by Hashimoto describing patients with both goiter and intense lymphocytic infiltration of the thyroid (Figs 1.1 & 1.2) as “struma lymphomatosa.”
Hashimoto’s thyroiditis is the most common cause of primary hypothyroidism in iodine-sufficient areas of the world; it is among the most common causes of nonendemic goiter. On average 1.0–1.5/1000 people suffer from this disease. It occurs far more often in women than in men (incidence of 10 : 1 to 20 : 1, respectively), and it is most prevalent between 45 and 65 years of age. Occurrence in children is also uncommon, especially in populations where iodine is not a dietary scarcity.
Etiology and pathogenesis
Autoantibodies may be present against thyroid peroxidase, thyroglobulin, and thyroid-stimulating hormone (TSH) receptors, although a small percentage of patients may have none of these antibodies present. Antibody-dependent cell-mediated cytotoxicity is a substantial factor behind the apoptotic fallout of HT. Activation of cytotoxic T lymphocytes (CD8+ T cells) in response to cell-mediated immune response affected by helper T lymphocytes (CD4+ T cells) is central to thyrocyte destruction. Recruitment of macrophages is another effect of helper T-lymphocyte activation, with Th1-axis lymphocytes producing inflammatory cytokines within the thyroid tissue to further macrophage activation and migration into the thyroid gland for a direct effect. Infection, stress, sex steroids, pregnancy, iodine intake, and radiation exposure are known possible precipitating factors for HT. Fetal microchimerism within the maternal thyroid is also a possibility.
Signs and symptoms
Hashimoto’s thyroiditis very often results in hypothyroidism with bouts of hyperthyroidism. Symptoms of HT include weight gain, depression, mania, sensitivity to heat and cold, paresthesia, fatigue, panic attacks, bradycardia, tachycardia, high cholesterol, reactive hypoglycemia, constipation, migraine, muscle weakness, cramps, memory loss, infertility, hair loss, and myxedematous psychosis.
Diagnosis
Laboratory findings
Laboratory tests for HT include:
Antithyroid peroxidase antibodies (TPOAbs) and thyroglobulin antibodies (TgAbs)TSH, free thyroxine (FT4)Total cholesterol, high density lipoprotein (HDL), and triglyceridesImaging tests
Imaging tests for HT include:
Neck ultrasound (Fig. 1.3)Computed tomography (CT) scan (rare)99mTcO4 thyroid scintigraphy (Fig. 1.4)Treatment
In patients with primary hypothyroidism, the main treatment is levothyroxine.
Illustrations (Figs 1.1–1.4)
Figure 1.1 Cytology of thyroiditis. This figure shows rare and normal thyrocytes associated with numerous lymphocytes (Papanicolau, 10×).
Figure 1.2 Histology of thyroiditis. Hashimoto thyroiditis is characterized by Hürthle cells associated with follicular lymphoid structures (HE, 10×).
Figure 1.3 A 46-year-old woman with a recent episode of cervical tenderness and a familiar history of thyroid disease. The patient complained of fatigue and reported a weight gain of about 10 kg in the last 2 months. (a) Thyroid ultrasound – cross section. This ultrasound shows a thyroid with a slight increase in volume, globular shape, and homogeneous structure, and less echogenic than normal. (b) Thyroid ultrasound – longitudinal section. This ultrasound shows diffuse patchy hypoechoic lesions throughout the gland. This sonographic appearance is called a “leopard skin” pattern and is seen in lymphocytic infiltration of the thyroid in Hashimoto’s thyroiditis. The hypoechoic lesions within the thyroid are areas of lymphocytic infiltration of the thyroid tissue. C, carotid artery; H, hypoechoic lesions; P, thyroid parenchyma; T, trachea.
Figure 1.4 The same patient as in Fig. 1.3: 99mTcO4 thyroid scintigraphy with iodine uptake curve. Iodine uptake was 2% at 4 hours (a) and 2% at 24 hours (b). The scan showed no uptake in the thyroid bed. The free triiodothyronine (FT3) and free thyroxine (FT4) levels were low with elevated thyroid stimulating hormone (TSH) and antibodies against thyroperoxidase (TPOAb) values. The patient started levothyroxine treatment.
Graves’ disease (Basedow’s disease)
Definition and epidemiology
Graves’ disease (GD) is an autoimmune disease representing the most common cause of hyperthyroidism (60–90% of all cases).
Graves’ disease has a powerful hereditary component, affecting up to 2% of the female population, and is between five and ten times more common in females than in males (incidence of 5 : 1 to 10 : 1, respectively). It is also the most common cause of severe hyperthyroidism, which is accompanied by extended clinical signs and symptoms and laboratory abnormalities compared with milder forms of hyperthyroidism. About 30–50% of patients with GD will also suffer from Graves’ ophthalmopathy, which is caused by inflammation of the eye muscles mediated by an inflammatory immune process.
Etiology and pathogenesis
Graves’ disease is an autoimmune disorder in which the body produces antibodies to the receptor for thyroid stimulating hormone (TSHrAb). (Antibodies to thyroglobulin and thyroperoxidase may also be produced.) TSHrAb bind to the thyroid stimulating hormone (TSH) receptors, which are located on cells producing thyroid hormone in the thyroid gland (follicular cells), and chronically stimulate them, resulting in an abnormally high production of triiodothyronine (T3) and thyroxine (T4). There are several factors that predispose to GD and Graves’ ophthalmopathy; in particular, genetic susceptibility, infection, smoking, pregnancy, iodine, and iodine-containing drugs.
Signs and symptoms
Signs and symptoms of GD all result from the direct and indirect effects of hyperthyroidism, with the main exceptions being Graves’ ophthalmopathy, goiter, and pretibial myxedema.
Diagnosis
Laboratory findings
Laboratory tests for GD include:
Thyroid stimulating hormone (TSH), free triiodothyronine (FT3), and free thyroxine (FT4)TSHrAbTotal cholesterol, high density lipoprotein (HDL), triglyceridesImaging tests
Imaging tests for GD include:
Thyroid ultrasound (Fig. 1.5)99mTcO4 thyroid scintigraphy (Fig. 1.6)Computed tomography (CT) neck scanOrbital nuclear magnetic resonance (NMR)Treatment
Treatment options for GD are:
Beta blockers (rapid amelioration of symptoms)ThionamideRadioiodine ablationSurgeryGlucocorticoid (for Graves’ ophthalmopathy)Orbital irradiation (for Graves’ ophthalmopathy)Orbital decompression surgery (for Graves’ ophthalmopathy)Illustrations (Figs 1.5 & 1.6)
Figure 1.5 A 32-year-old man presented with an unintentional 15 kg weight loss but with an otherwise normal physical examination. Laboratory studies revealed a suppressed thyroid stimulating hormone (TSH) concentration and an elevated thyroxine level, which are consistent with hyperthyroidism. Thyroid ultrasound – (a) cross section and (b) longitudinal section. These ultrasound/color Doppler images reveal markedly increased vascularity throughout the thyroid gland (“thyroid hell”). P, thyroid parenchyma; T, trachea.
Figure 1.6 Thyroid scan of the same patient from Figure 1.5 with 99mTcO4. The thyroid scan with 99mTcO4 before (a) and after (b) treatment with methimazole. Intense and homogeneous uptake of the radiopharmaceutical in both lobes of thyroid gland is seen before therapy. The post-therapy scan was performed 6 months after therapy and shows a reduction of thyroid size and uptake.
Subacute thyroiditis (de Quervain’s thyroiditis)
Definition and epidemiology
Subacute thyroiditis (ST) is a subacute granulomatous thyroiditis that belongs to a group of thyroiditis conditions known as resolving thyroiditis. Other names for this disorder are de Quervain’s thyroiditis, subacute nonsuppurative thyroiditis, giant cell thyroiditis, and painful thyroiditis. It has an incidence of 12.1/100 000 per year with a higher incidence in females than in males (19.1 and 4.1/100 000 per year, respectively). It is most common in young adulthood (24/100 000 per year) and middle age (35/100 000 per year), and decreases with increased age.
Etiology and pathogenesis
Subacute thyroiditis is presumed to be caused by a viral infection or a postviral inflammatory process. The majority of patients have a history of an upper respiratory infection prior to the onset of thyroiditis (typically 2–8 weeks beforehand). The disease was thought to have a seasonal incidence (higher in the summer), and clusters of cases have been reported in association with Coxsackievirus, mumps, measles, adenovirus, and other viral infections. Thyroid autoimmunity does not appear to play a primary role in the disorder, but it is strongly associated with HLA-B35 in many ethnic groups. A unifying hypothesis might be that the disorder results from a common subclinical viral infection that provides an antigen, either of viral origin or resulting from virus-induced host tissue damage, that uniquely binds to HLA-B35 molecules on macrophages. The resulting antigen-HLA-B35 complex activates cytotoxic T lymphocytes that then damage thyroid follicular cells, since the cells have partial structural similarity with the infection-related antigen. Unlike autoimmune thyroid disease, however, the immune reaction is not self-perpetuating, so the process is limited. The resulting thyroid inflammation damages thyroid follicles and activates proteolysis of the thyroglobulin stored within the follicles. The result is an unregulated release of large amounts of thyroxine (T4) and triiodothyronine (T3) into the circulation resulting in clinical and biochemical hyperthyroidism.
Signs and symptoms
Subacute thyroiditis is a self-limiting thyroid condition associated with a triphasic clinical course of hyperthyroidism, hypothyroidism, and return to normal thyroid function. In particular, ST may be responsible for 15–20% of patients with thyrotoxicosis and 10% of patients presenting with hypothyroidism. Pain is the main symptom and it may be limited to the thyroid region or it may radiate to the upper neck, jaw, throat, upper chest, or ears. It can be exacerbated by coughing or turning the head. Fever, fatigue, malaise, anorexia, and myalgia are common.
Diagnosis
Laboratory findings
Laboratory tests for ST include:
Thyroid stimulating hormone (TSH), free triiodothyronine (FT3), and free thyroxine (FT4)Erythrocyte sedimentation rate (ESR)Polymerase chain reaction (PCR) for C-reactive proteinHemochromeImaging tests
A neck ultrasound is needed (Fig. 1.7).
Treatment
Subacute thyroiditis is a self-limiting condition and so in most patients no specific therapy, such as antithyroid or thyroid hormone replacement therapy, is necessary. Treatment of patients with ST should be directed at providing relief for thyroid pain (e.g. prednisone) and tenderness, and ameliorating symptoms of hyperthyroidism (e.g. with a beta blocker such as propranolol).
Illustration (Fig. 1.7)
Figure 1.7 A 47-year-old woman presents with pain and tenderness on her right side due to a chronic goiter. Her erythrocyte sedimentation rate was elevated and her thyroid laboratory tests suggested subclinical hypothyroidism. Two weeks previously, she had a fever and now her 99mTc pertechnetate uptake is markedly decreased. (a) Thyroid ultrasound – cross section (before treatment). Focal hypoechogenicity in the painful area with decreased vascular flow by Doppler scan. C, carotid artery; P, thyroid parenchyma. The patient’s clinical symptoms showed a dramatic response to glucocorticoid treatment. She became hypothyroid and began levothyroxine therapy. (b) Thyroid ultrasound – cross section (after treatment). The focal hypoechogenicity is reduced and the thyroid parenchyma has become more homogeneous. C, carotid artery; P, thyroid parenchyma.
Benign thyroid nodules
Definition and epidemiology
Thyroid nodules are the most common of thyroid diseases. They affect up to 5% of the general population and are more frequent in iodine deficient areas and in women (female to male ratio, 5 : 1). Thyroid nodules are mostly benign (adenoma, cysti, focal hyperplasia) and the incidence of malignant neoplasia is very low (4/100 000 per year).
Thyroid nodules are abnormal cell growths in the thyroid gland. The thyroid can be uninodular when a single nodule is present or multinodular when multiple nodules are present.
Thyroid nodules are mostly nonfunctioning but can be hyperfunctioning (toxic multinodular goiter, Plummer’s disease) leading to symptoms of hyperthyroidism.
Etiology and pathogenesis
The etiology of thyroid nodules is unknown. There are several factors that predispose to these nodules; in particular, genetic susceptibility, iodine deficiency, neck irradiation, and unknown environmental agents.
Signs and symptoms
Usually thyroid nodules are asymptomatic and they are occasionally discovered during physical examination or an ultrasound neck scan.
Signs and symptoms of large nodules or multinodular goiter mainly result from thyroid increased volume and neck compression. The signs and symptoms include:
Neck lumpNeck pain, dyspnea, dysphagia, dysphoniaSymptoms due to hyperthyroidism (in toxic multinodular goiter or Plummer’s adenoma)Diagnosis
The gold standard for diagnosing thyroid nodules consists of both a neck ultrasound scan (evaluating nodules size and eventually suspicious features) and fine needle cytology (FNC) to diagnose malignant neoplasia.
Laboratory and cytology tests
The laboratory and cytology tests for thyroid nodules include:
Calcitonin (in nodules suspicious for medullary carcinoma)Thyroid stimulating hormone (TSH), free triiodothyronine (FT3), free thyroxine (FT4)Cytology (fine needle cytology)Imaging tests
Imaging tests for thyroid nodules include:
Thyroid ultrasounds (Figs 1.8a, 1.9a & 1.10a): Relevant ultrasound scan features of thyroid nodules are: echostructure (solid, cystic, or mist nodules), echogenicity (ipo-, iso-, or anechogen nodules), vascular pattern, presence of microcalcifications (regular or irregular), and defined or undefined marginsComputed tomography (CT) neck scan (Fig. 1.11)Neck X-rayScintigraphy thyroid scans (Figs 1.8b, 1.9b & 1.10b)Treatment
Treatment options for thyroid nodules are:
Clinical and ultrasound scan follow-upSurgery (for compressive symptoms, tracheal or neck vessel compression or dislocation, mediastinal thyroid)Treatment of hyperthyroidism (toxic multinodular goiter, Plummer’s adenoma)Illustrations (Figs 1.8–1.11)
Figure 1.8 A case of thyroid toxic adenoma. A 56-year-old female patient with symptoms of hyperthyroidism. Hormonal blood levels showed increased free triiodothyronine (FT3), free thyroxine (FT4), and suppressed thyroid stimulating hormone (TSH). (a) Thyroid ultrasound showed a hypoechoic solid nodule of 14 × 15 mm with intra- and perinodular vascularization in the lower third of the right thyroid lobe. (b) The thyroid morpho-functional study was performed with 50 μCi of 131I orally and 3 mCi of 99mTcO4 intravenously to evaluate thyroid uptake of iodine and scintigraphic distribution of Tc, respectively. Thyroid uptake was 17% at 6 hours, 29% at 24 hours, and 22% at 48 hours (data relevant for dosimetric calculations). The thyroid scan confirmed the clinical suspicion of Plummer’s adenoma and showed complete functional inhibition of extranodular glandular tissue (inhibiting adenoma), which is the ideal condition for performing 131I therapy.
Figure 1.9 A case of single thyroid nodule. A 25-year-old patient with incidental ultrasound finding of a thyroid nodule in the left lobe. (a) Thyroid ultrasound shows a solid hypoechoic nodule, with microcalcifications. (b) Thyroid scintigraphy shows the “cold” nodule with no detectable 99mTcO4 uptake. The patient underwent fine needle cytology and the cytology was suspicious for papillary carcinoma.
Figure 1.10 A case of multinodular toxic thyroid. A hyperthyroid 46-year-old woman with a palpable multinodular thyroid. (a) Ultrasound scan shows an enlarged thyroid with multiple nodules in both right and left lobe. The gland seems to extend in the mediastinum. (b) Thyroid scan with 99mTcO4. The scan shows intense uptake in the glandular parenchima with multiple “cold” areas in correspondence to the major nodules seen at ultrasound. This finding is consistent with the diagnosis of a “multinodular toxic thyroid.” The patient underwent surgery.
Figure 1.11 A case of goiter. A 70-year-old man with a palpable multinodular goiter. The axial contrast-enhanced computed tomography (CT) image shows increased thyroid volume compressing the tracheal and esophageal lumen.
Thyroid cancer
Definition and epidemiology
Carcinoma of the thyroid is an uncommon cancer but it is the most common malignancy of the endocrine system (Figs 1.12–1.25). Differentiated tumors (papillary [Figs 1.14–1.17] or follicular [Fig. 1.20]) are highly treatable and are usually curable. Poorly differentiated tumors (medullary [Fig. 1.23] or anaplastic) are much less common, are aggressive, metastasize early, and have a much poorer prognosis. Thyroid cancer affects women more often than men and usually occurs in people between the ages of 25 and 65. The incidence of this malignancy has been increasing over the last decade.
The World Health Organization 2004 classification is shown in Table 1.1. The prognosis for differentiated carcinoma is better for patients aged below 40 without extracapsular extension or vascular invasion. Age appears to be the single most important prognostic factor. The prognostic significance of lymph node status is controversial. Adverse factors include: Older than 45 years; follicular histology; primary tumor > 4 cm (T2–T3); extrathyroid extension (T4); and distant metastases.
Table 1.1 World Health Organization 2004 classification of neoplasms of the thyroid
Etiology and pathogenesis
Risk factors for thyroid cancer
The risk factors for thyroid cancer are:
External radiation and thyroid cancerHistory of goiterFamily history of thyroid diseaseFemale genderAsian ethnicityWhile an increased incidence of thyroid cancer in patients with Hashimoto’s thyroiditis has been reported, clinical experience does not suggest a strong relationship between this relatively common disease and thyroid cancer.
Signs and symptoms
Most frequently the tumor is discovered accidentally as a finding during an ultrasound of the neck. It may appear as a gradually enlarging, painful mass with associated symptoms of hoarseness, dysphagia, or dysphonia, or there may be difficulty breathing. Occasionally a patient arrives with metastatic nodules in the neck, pulmonary symptoms from metastases, or a pathologic fracture of the spine or hip. Usually there are no symptoms of hyper- or hypothyroidism.
Upon examination of the neck, carcinoma of the thyroid characteristically appears as an asymmetrical lump in the gland. If it is still within the confines of the gland, it will move with the gland when the patient swallows and may be moveable within the gland. If it has invaded the trachea or neighboring structures, it may be fixed in place. Lymph nodes containing metastases may be found in the supraclavicular triangles, in the carotid chain, along the thyroid isthmus, and rarely in the axillary nodes. Although carcinoma of the thyroid is typically firm or hard, rapidly growing lesions may sometimes be soft or even fluctuant.
Diagnosis
Most patients with thyroid carcinoma are recognized because of the observation of a neck mass and the result of fine needle aspiration cytology.
Laboratory findings
Laboratory tests for thyroid carcinoma:
Thyroid stimulating hormone (TSH), and free thyroxine (FT4) are usually measured to verify metabolic status.Antithyroid peroxidase antibodies (TPOAbs) and thyroglobulin antibodies (TgAbs).Thyroglobulin (Tg) assay: Although Tg assay has been suggested as an important marker for thyroid cancer, practice shows that elevated Tg levels can be caused by adenoma, multinodular goiter, and other diseases; thus, the determination is of little value before operating.Calcitonin immunoassay: Some groups recommend this on all patients with nodules to allow preoperative diagnosis of medullary thyroid carcinoma.Imaging tests
Imaging tests for thyroid carcinoma include:
Ultrasound studies, which are the most basic and useful test.Isotope scans (Figs 1.18a&f, 1.19, 1.21 & 1.24) have a limited role in the initial diagnosis.Chest X-rays may be informative but are often omitted.Computed tomography (CT) (Figs 1.12, 1.13, 1.22–1.25) and TC-18F-FDG positron emission tomography (PET) (Fig. 1.18d&g) scanning of the lungs and magnetic resonance imaging (MRI) of the neck can provide useful information prior to surgery in lesions that extend outside the thyroid or have metastasis.Treatment
In differentiated cancers contemporary medical and surgical practice depends mainly on the clinical stage of the disease rather than on the exact histologic status. The classification can be conveniently reduced to four categories that have prognostic significance and clear therapeutic relevance (Table 1.2).
Thyroid cancer may require surgery. The possible surgical approaches range from a simple removal of the nodule to total thyroidectomy with bilateral radical neck dissection.Radioactive iodine-131 is used in patients with papillary or follicular thyroid cancer for ablation of residual thyroid tissue after surgery and for the treatment of thyroid cancer. Patients with medullary, anaplastic, and most Hürthle cell cancers do not benefit from this therapy.Table 1.2 Thyroid cancer: Classification
Clinical stageComparable TNM classificationI. IntrathyroidalT0, T1, T2, N0, M0II. Cervical adenopathyT0–T2, N0, N1a, N1b, M0III. Locally invasive diseaseT3, T4a, T4b, M0IV. Distant metastasesM1Courtesy of Edge SB, Byrd DR, Compton CC, et al. (eds) (2010) AJCC Cancer Staging Manual, 7th edn. Springer, New York, NY.External irradiation may be used when the cancer is unresectable, when it recurs after resection, or to relieve pain from bone metastasis.
Sorafenib and sunitinib, approved for other indications, show promise as treatments for thyroid cancer and are being used by some patients who do not qualify for clinical trials.
Survival rate for thyroid cancer is related to both type of cancer and stage at time of diagnosis (Table 1.3).
Table 1.3 Thyroid cancer: 5-year survival rates
Illustrations (Figs 1.12–1.25)
Figure 1.12 Papillary thyroid carcinoma and cystic lymph node metastasis in a 57-year-old man. (a) Transverse sonogram of the right lobe of the thyroid shows a solitary, isoechoic, inhomogenous nodule with irregular margin. (b) Longitudinal sonogram of the same thyroid nodule.
Figure 1.13 Transverse sonogram of the right neck shows a cystic level 4 nodal (red arrow). C: carotid artery; J, jugular vein.
Figure 1.14 Cytology of papillary carcinoma (Papanicolau, 20×). Thyrocytes are arranged in a pseudopapillary structure with nuclear irregularity.
Figure 1.15 Cytology of papillary carcinoma (Papanicolau, 40×). Thyrocytes with evidence of little nucleus, nuclear pseudoinclusion, and nuclear incision.
Figure 1.16 Histology of papillary carcinoma. Papillary carcinoma with ground glass nuclei (HE, 10×).
Figure 1.17 Histology of papillary carcinoma. Metastasis in neck node (HE, 10×).
Figure 1.18 A case of differentiated thyroid cancer: Papillary thyroid cancer infiltrating the periglandular soft tissues with lymph node metastases (pT4 N1a Mx). After several cycles of 131I therapy (a–c) the patient still had residual disease in the paratracheal lymph nodes unaffected by the iodine therapy and clearly detected by 18F-FDG positron emission tomography (PET) performed in 2008 (d) during 131I therapy and in 2009 (e) after the last 131I treatment. An increase in the size of the metastases was observed despite the iodine therapy. Iodine therapy was therefore discontinued and the patient performed an 111In-Octreoscan to verify if metastases had somatostatin receptors. The scan showed high density of somatostatin receptors (f). The patient started treatment with long-acting somatostatin analogs, with stable disease after 2 years as shown by the 18F-FDG PET scan performed for restaging 2 years after (g).
Figure 1.19 A case of follicular thyroid cancer with bone metastases. Bone metastases is rare in differentiated thyroid cancer but may occur with or without 131I uptake and variable thyroglobulin (Tg) production. These metastases can be detected with 18F-FDG positron emission tomography (PET) when metabolically active, but the diagnostic exam of choice is a bone scan with 99mTc-hydroxymethylene-diphosphonate (HDP) or 99mTc-methyl-diphosphate (MDP) as shown in this figure. An avid bone uptake offers the treatment option with a beta-emitting isotope (188Re-HEDP, 153Sm-EDTMP, 89Sr-Chloride, etc.).
Figure 1.20 Cytology of follicular lesion (Papanicolau, 10×). Thyrocytes arranged in microfollicular structure, with scant colloid.
Figure 1.21 A case of undifferentiated thyroid cancer: These images show the case of a 64-year-old woman who underwent several surgeries for primary and recurrent undifferentiated thyroid cancer. She also performed 131I therapy and the whole body scan post-therapy showed disease recurrence in the neck and lungs (ai & aii). At the same time an 18F-FDG positron emission tomography (PET) scan showed high glucose metabolism in the neck metastasis (a negative prognostic factor) but not in the other metastases (b). The patient therefore performed a salvage radiotherapy with external beam, but the following 18F-FDG PET scan showed progression of the disease with multiple focal areas of increased uptake in the laterocervical lymph nodes, the mediastinum, the pulmonary parenchima, and the hilum (c).
Figure 1.22 Medullary thyroid carcinoma in a 52-year-old woman with an elevated calcitonin value. (a) Transverse sonogram of the right lobe of the thyroid shows a solitary, hypoechoic, nodule with an irregular margin. (b) Longitudinal sonogram of the same thyroid nodule.
Figure 1.23 Histology of medullary carcinoma: Solid nests of polygonal cells associated to amyloid deposits within the stroma (HE, 20×).
Figure 1.24 A case of medullary thyroid cancer (MTC): Gamma camera images of the chest of a patient with metastatic MTC. Images were acquired 1 hour after the intravenous administration of 15 mCi of 99mTc-HYNICTOC (a somatostatin analog that binds to type 3 receptors) before (a) and after (b) therapy with 60 mCi of 90Y-DOTATOC (the same somatostatin analog radiolabeled with a beta-emitting isotope). In MTC primary and metastatic lesions can express somatostatin receptors (SSTRs). The scintigraphic demonstration of SSTRs is therefore mandatory for both correct staging and therapy decision making. In this patient the treatment of choice was with radiolabeled somatostatin analogs, and after just one cycle of therapy the scan (b) shows large necrosis and reduction in size of all metastasis.
Figure 1.25 B-cell non-Hodgkin lymphoma of the thyroid in a 77-year-old woman with Hashimoto thyroiditis. (a) Transverse sonogram of the left lobe of the thyroid shows a large heterogeneous mass with marked hypoechogenicity when compared with the strap muscles (SM). (b) Axial contrast-enhanced computed tomography (CT) imaging shows widespread morphostructural disruption of the thyroid left lobe, replaced by hypodense solid tissue, invading adjacent muscle planes and extending to the posterior mediastinum, compressing the tracheal and esophageal lumen, and to the origin of neck vessels, significantly reducing the size of the internal jugular vein. Lymphadenopathies in the right side of the neck and in the superior mediastinum are also evident.
2
Pituitary Gland
Craniopharyngioma
Definition
Craniopharyngiomas may be large and invade the third ventricle and associated brain structures. This tumorous process is usually located above the sella turcica, depressing the optic chiasm and extending up into the third ventricle (Fig. 2.1).
Etiology
Craniopharyngioma is a benign epithelioid tumor arising from embryonic squamous remnants of Rathke’s pouch and it is the most common tumor found in the region of the pituitary gland in children and adolescents – constituting about 3% of all intracranial tumors and up to 10% of all childhood brain tumors.
Signs and symptoms
Signs and symptoms are primarily due to mass effect and typically occur in the adolescent years and rarely after age 40. The mass effect symptoms include: vision loss by compression of the optic chiasm; diabetes insipidus by invasion or disruption of the hypothalamus or pituitary stalk; hypothalamic dysfunction (e.g. obesity with hyperphagia, hypersomnolence, disturbance in temperature regulation); various degrees of anterior pituitary insufficiency (e.g. growth hormone deficiency with short stature in childhood, hypogonadism, adrenal insufficiency, hypothyroidism); hyperprolactinemia due to compression of the pituitary stalk or damage to the dopaminergic neurons in the hypothalamus; signs and symptoms of increased intracranial pressure (e.g. headache, projectile emesis, papilledema, optic atrophy); symptoms of hydrocephalus (e.g. mental dullness and confusion); and cranial nerve palsies caused by cavernous sinus invasion.
Diagnosis
Imaging tests
The findings on radiologic imaging are quite characteristic. Plain skull roentgenograms and computed tomography (CT) show irregular calcification in the suprasellar region. Magnetic resonance imaging (MRI) typically shows a multilobulated cystic structure – usually suprasellar in location, but it may also appear to arise from the sella (Fig. 2.2). The cystic regions are usually filled with a turbid, cholesterol-rich, viscous fluid (Fig. 2.3).
Laboratory findings
There is no specific laboratory test for craniopharyngioma. Hypothalamic–pituitary function may be intact, or testing may demonstrate varying degrees of hormonal deficiencies.
Treatment
Treatment options for patients with craniopharyngiomas include observation, transsphenoidal surgery for smaller intrasellar tumors, craniotomy for larger suprasellar tumors, stereotactic radiotherapy, or a combination of these modalities.
Illustrations (Figs 2.1–2.3)
Figure 2.1 Magnetic resonance imaging (MRI) (sagittal view) showing cystic suprasellar craniopharyngioma.
Figure 2.2 Magnetic resonance imaging (MRI) (coronal view) showing suprasellar craniopharyngioma.
Figure 2.3 Turbid, cholesterol-rich, viscous fluid from a craniopharyngioma.
Hypothalamic dysgerminoma
Definition
Hypothalamic dysgerminomas are germ cell tumors.
Etiology
The locations of central nervous system germ cell tumors include the diencephalic area, pineal region, and hypothalamus. Germ cell tumors primarily affect young people and represent 5% of intracranial neoplasms in children.
Signs and symptoms
Due to the proximity to the optic chiasm, hypothalamic lesions are frequently associated with vision loss. An enlarging hypothalamic mass may also cause headaches and recurrent emesis. The hypothalamus is responsible for many homeostatic functions such as appetite control, the sleep–wake cycle, water metabolism, temperature regulation, anterior pituitary function, circadian rhythms, and inputs to the parasympathetic and sympathetic nervous systems. The clinical presentation is dependent on the location of the dysgerminoma within the hypothalamus and patients may present with a constellation of symptoms including: hypersomnolence; hyperactivity and insomnia; alterations in the sleep–wake cycle (e.g. nighttime hyperactivity and daytime sleepiness); dysthermia (acute hyperthermia or chronic hypothermia); hyperphagia and obesity; hypophagia with weight loss and cachexia; central diabetes insipidus; polydipsia or hypodipsia; and varying degrees of anterior pituitary dysfunction.
Hypothalamic germ cell tumors may produce peptides normally secreted by the hypothalamus. For example, children may present with precocious puberty when beta-human chorionic gonadotropin (β-hCG) is hypersecreted by suprasellar germ cell tumors.
Diagnosis
Although hypothalamic dysgerminoma may be diagnosed based on measurement of cerebral spinal fluid β-hCG, α-fetoprotein, or cytology, the diagnosis typically requires tissue confirmation with a biopsy.
Magnetic resonance imaging (MRI) typically reveals a gadolinium-enhancing hypothalamic mass extending into the pituitary stalk in a “V-shape” (Figs 2.4 & 2.5). Occasionally the dysgerminoma may appear to be primarily located in the pituitary stalk or it may extend into the sella.
In patients with disease that extends beyond the hypothalamus, the cerebrospinal fluid analysis may show increased concentrations of β-hCG, α-fetoprotein, or abnormal cytology.
Treatment
Treatment typically involves radiation therapy to the hypothalamic region and also to the spine if there is evidence of drop metastases. Systemic chemotherapy may be indicated in patients with more primitive tumor types.
Illustrations (Figs 2.4 & 2.5)
Figure 2.4 Magnetic resonance imaging (MRI) (sagittal view) showing suprasellar dysgerminoma.
Figure 2.5 Magnetic resonance imaging (MRI) (coronal view) showing suprasellar dysgerminoma.
Growth hormone-secreting pituitary tumor
Definition
Chronic growth hormone (GH) excess from a GH-producing pituitary tumor results in the clinical syndrome of acromegaly in adults and gigantism in children or adolescents (before the epiphyses fuse).
Etiology
Growth hormone-secreting pituitary tumors are monoclonal somatotroph cell adenomas that appear to result from sporadic mutations. Although the annual incidence is estimated to be only 3 per 1 million persons in the general population, a GH-secreting pituitary adenoma is the second most common hormone-secreting pituitary tumor.
Signs and symptoms
The effects of the chronic GH excess include acral and soft-tissue overgrowth, expansion of the frontal sinuses – termed “frontal bossing” (Fig. 2.6), increased tissue on the plantar surfaces of the feet leading to plantar grooves (Fig. 2.7), progressive dental malocclusion (underbite), widening of the nose and thick lips (Fig. 2.8), degenerative arthritis related to chondral and synovial tissue overgrowth within joints, low-pitched sonorous voice, headaches, malodorous hyperhidrosis, oily skin, increased numbers of skin tags (Fig. 2.9), perineural hypertrophy leading to nerve entrapment (e.g. carpal tunnel syndrome), proximal muscle weakness, carbohydrate intolerance (initial presentation may be diabetes mellitus), hypertension, colonic neoplasia, obstructive sleep apnea, and cardiac dysfunction. The mass effects of GH-producing pituitary macroadenomas (> 10 mm in diameter) are similar to those of other pituitary macroadenomas – they include visual field defects, oculomotor pareses, headaches, and pituitary insufficiency.
The patient with acromegaly has a characteristic appearance with coarsening of facial features (Fig. 2.8), prognathism, frontal bossing (Fig. 2.6), spade-like hands, and wide feet. Often there is a history of progressive increase in shoe, glove, ring, or hat size. These changes may occur slowly and may go unrecognized by the patient, family, and physician. The average delay in diagnosis from the onset of the first symptoms to the eventual diagnosis is 8.5 years. Comparison with earlier photographs of the patient is helpful in confirming the clinical suspicion of acromegaly.
When a GH-secreting tumor develops in infancy, it may lead to exceptional height. The tallest well-documented person with pituitary gigantism measured 8 ft 11 in (2.72 m).
Diagnosis
Laboratory findings
