Riedel's thyroiditis is a rare form of thyroiditis characterized by replacement of thyroid tissue with fibrous tissue that invades surrounding areas. It typically presents as a painless hard neck mass that progresses over weeks to years, causing compression symptoms. Surgery aims to decompress the trachea by wedge resection but extensive resection is not advised due to the infiltrative nature of the fibrosis. The cause is controversial but it may be an autoimmune or fibrotic disorder associated with other focal sclerosing syndromes.
Riedel's thyroiditis is a rare form of thyroiditis characterized by replacement of thyroid tissue with fibrous tissue that invades surrounding areas. It typically presents as a painless hard neck mass that progresses over weeks to years, causing compression symptoms. Surgery aims to decompress the trachea by wedge resection but extensive resection is not advised due to the infiltrative nature of the fibrosis. The cause is controversial but it may be an autoimmune or fibrotic disorder associated with other focal sclerosing syndromes.
Riedel's thyroiditis is a rare form of thyroiditis characterized by replacement of thyroid tissue with fibrous tissue that invades surrounding areas. It typically presents as a painless hard neck mass that progresses over weeks to years, causing compression symptoms. Surgery aims to decompress the trachea by wedge resection but extensive resection is not advised due to the infiltrative nature of the fibrosis. The cause is controversial but it may be an autoimmune or fibrotic disorder associated with other focal sclerosing syndromes.
Riedel's thyroiditis is a rare form of thyroiditis characterized by replacement of thyroid tissue with fibrous tissue that invades surrounding areas. It typically presents as a painless hard neck mass that progresses over weeks to years, causing compression symptoms. Surgery aims to decompress the trachea by wedge resection but extensive resection is not advised due to the infiltrative nature of the fibrosis. The cause is controversial but it may be an autoimmune or fibrotic disorder associated with other focal sclerosing syndromes.
Riedel’s Thyroiditis Riedel’s thyroiditis is a Hypothyroid patients are treated with
rare variant of thyroid hormone
thyroiditis also known as Riedel’s struma or replacement. Some patients who remain invasive fibrous symptomatic have been thyroiditis that is characterized by the reported to experience dramatic improvement replacement of all or part after treatment of the thyroid parenchyma by fibrous with corticosteroids and tamoxifen. More tissue, which also invades recently mycophenolate into adjacent tissues. The etiology of this mofetil has been used to attenuate the disorder is controversial, inflammatory process and it has been reported to occur in and led to dramatic symptom improvements in patients with other some patients.14 autoimmune diseases. This association, Goiter. Any enlargement of the thyroid gland coupled with the presence is referred to as of lymphoid infiltration and response to a goiter. The causes of nontoxic goiters steroid therapy, are listed in Table 38-3. suggests a primary autoimmune etiology. Goiters may be diffuse, uninodular, or Riedel’s thyroiditis multinodular. Most nontoxic also is associated with other focal goiters are thought to result from TSH sclerosing syndromes including stimulation secondary mediastinal, retroperitoneal, periorbital, to inadequate thyroid hormone synthesis and and retro-orbital other paracrine fibrosis and sclerosing cholangitis, growth factors.15 Elevated TSH levels induce suggesting that it may, in diffuse thyroid fact, be a primary fibrotic disorder. The hyperplasia, followed by focal hyperplasia, disease occurs predominantly resulting in nodules in women between the ages of 30 and 60 that may or may not concentrate iodine, years old. It colloid nodules, or typically presents as a painless, hard microfollicular nodules. The TSH-dependent anterior neck mass, which nodules progress progresses over weeks to years to produce to become autonomous. Familial goiters symptoms of compression, resulting from inherited including dysphagia, dyspnea, choking, and deficiencies in enzymes necessary for hoarseness. thyroid hormone Patients may present with symptoms of synthesis may be complete or partial. The hypothyroidism term endemic goiter refers to the occurrence and hypoparathyroidism as the gland is of a goiter in a significant proportion of replaced by fibrous individuals in a particular geographic tissue. Physical examination reveals a region. In the past, dietary hard, “woody” thyroid iodine deficiency was the most common cause gland with fixation to surrounding tissues. of endemic goiter. The diagnosis needs This condition has largely disappeared in to be confirmed by open thyroid biopsy, North America because the firm and due to routine use of iodized salt and fibrous nature of the gland renders FNAB iodination of fertilizers, inadequate. animal feeds, and preservatives. However, Surgery is the mainstay of the treatment. in areas of iodine The chief goal deficiency, such as Central Asia, South of operation is to decompress the trachea America, and Indonesia, by wedge excision up to 90% of the population have goiters. of the thyroid isthmus and to make a tissue Other dietary goitrogens diagnosis. More that may participate in endemic goiter extensive resections are not advised due to formation include the infiltrative nature kelp, cassava, and cabbage. In many of the fibrotic process that obscures usual sporadic goiters, no obvious landmarks and structures. cause can be identified. Clinical Features Most patients with nontoxic decrease and/or stabilization of goiter goiters are size and is most effective asymptomatic, although patients often for small diffuse goiters. Endemic goiters complain of a pressure are treated by iodine sensation in the neck. As the goiters administration. Surgical resection is become very large, compressive reserved for goiters that (a) symptoms such as dyspnea and dysphagia continue to increase despite T4 suppression, ensue. Patients (b) cause obstructive also describe having to clear their throats symptoms, (c) have substernal extension, frequently (catarrh). (d) have malignancy Dysphonia from RLN injury is rare, except suspected or proven by FNAB, and (e) are when malignancy is cosmetically present. Obstruction of venous return at unacceptable. Near-total or total the thoracic inlet from a thyroidectomy is the treatment substernal goiter results in a positive of choice, and patients require lifelong T 4 Pemberton’s sign—facial therapy. flushing and dilatation of cervical veins Solitary Thyroid Nodule upon raising the arms Solitary thyroid nodules are present in above the head (Fig. 38-13A). Sudden approximately 4% of enlargement of nodules individuals in the United States, whereas or cysts due to hemorrhage may cause acute thyroid cancer has a pain. Physical much lower incidence of 40 new cases per 1 examination may reveal a soft, diffusely million. Therefore, enlarged gland (simple it is of utmost importance to determine goiter) or nodules of various size and which patients with solitary consistency in case of a thyroid nodule would benefit from surgery. multinodular goiter. Deviation or History. Details regarding the nodule, such compression of the trachea as time of onset, may be apparent. change in size, and associated symptoms Diagnostic Tests Patients usually are such as pain, dysphagia, euthyroid with normal dyspnea, or choking, should be elicited. TSH and low-normal or normal free T4 levels. Pain is an If some nodules unusual symptom and, when present, should develop autonomy, patients have suppressed raise suspicion TSH levels or for intrathyroidal hemorrhage in a benign become hyperthyroid. RAI uptake often shows nodule, thyroiditis, or patchy uptake malignancy. Patients with MTC may complain with areas of hot and cold nodules. FNAB is of a dull, aching recommended in sensation. A history of hoarseness is patients who have a dominant nodule or one worrisome, as it may be that is painful or secondary to malignant involvement of the enlarging, as carcinomas have been reported RLNs. Most importantly, in 5% to 10% of patients should be questioned regarding multinodular goiters. CT scans are helpful risk factors for to evaluate the extent malignancy, such as exposure to ionizing of retrosternal extension and airway radiation and family compression (Fig. 38-13B). history of thyroid and other malignancies Treatment Most euthyroid patients with associated with small, diffuse goiters thyroid cancer. do not require treatment. Some physicians External-Beam Radiation Low-dose therapeutic give patients radiation has with large goiters exogenous thyroid been used to treat conditions such as tinea hormone to reduce the capitis (6.5 cGy), thymic TSH stimulation of gland growth; this enlargement (100 to 400 cGy), enlarged treatment may result in tonsils and adenoids (750 cGy), acne vulgaris (200 to 1500 cGy), such as Cowden’s syndrome, Werner’s and other conditions syndrome (adult such as hemangioma and scrofula. Radiation progeroid syndrome), and familial (approximately adenomatous polyposis 4000 cGy) is also an integral part of the (Table 38-4). Nonmedullary thyroid cancers management of can also occur patients with Hodgkin’s disease. It is now independently of these syndromes as the known that a history predominant tumors in of exposure to low-dose ionizing radiation the families. The definition of familial to the thyroid gland nonmedullary thyroid places the patient at increased risk for cancer (FNMTC) is variable across the developing thyroid cancer. literature; however, in The risk increases linearly from 6.5 to most studies, it is defined by the presence 2000 cGy, beyond of two or more firstdegree which the incidence declines as the relatives with follicular cell–derived radiation causes destruction cancers. FNMTC is of the thyroid tissue. The risk is maximum now recognized as a distinct clinical 20 to 30 years entity associated with a after exposure, but these patients require high incidence of multifocal tumors and lifelong monitoring. benign thyroid nodules. During the nuclear fallout from Chernobyl Some studies report that these patients in 1986, 131I release have higher locoregional was accompanied by a marked increase in the recurrence rates and consequent shorter incidence of both disease-free benign and malignant thyroid lesions noted survival. Several candidate loci that within 4 years of predispose to these tumors exposure, particularly in children.16 Most have been identified, including MNG1 thyroid carcinomas (14q32), thyroid carcinoma following radiation exposure are papillary, with oxphilia (TCO, on 19p13.2), and some of these fPTC/papillary renal cancers with a solid type of histology and neoplasia (PRN, on 1q21), NMTC (2q21), FTEN presence of RET/PTC (8p23.1-p22), translocations appear to be more and the telomere-telomerase complex.17 aggressive. In general, there Physical Examination. The thyroid gland is is a 40% chance that patients presenting best palpated with a thyroid nodule from behind the patient and with the neck and a history of radiation have thyroid in mild extension. cancer. Of those patients The cricoid cartilage is an important who have thyroid cancer, the cancer is landmark, as the isthmus located in the dominant is situated just below it. Nodules that are nodule in 60% of patients, but in the hard, gritty, or fixed remaining 40% of patients, to surrounding structures such as the the cancer is in another nodule in the trachea or strap muscles thyroid gland. Family History A family history are more likely to be malignant. The of thyroid cancer is a risk factor cervical chain of lymph for the development of both medullary and nodes should be assessed as well as the nonmedullary nodes in the posterior thyroid cancer. Familial MTCs occur in triangle. Diagnostic Investigations. An isolation or in association algorithm for the workup of with other tumors as part of multiple a solitary thyroid nodule is shown in Fig. endocrine neoplasia 38-14. type 2 (MEN2) syndromes. Nonmedullary Fine-Needle Aspiration Biopsy FNAB has become thyroid cancers can the single occur in association with other known most important test in the evaluation of familial cancer syndromes thyroid masses and can be performed with or without ultrasound The most common lesion in this setting is a guidance.18 Ultrasound follicular nodule guidance is recommended for nodules that (includes adenomatoid nodule, colloid are difficult to palpate, nodule, and follicular for cystic or solid-cystic nodules that adenoma). Other diagnoses include recur after the initial lymphocytic (Hashimoto’s) aspiration, and for multinodular goiters. A thyroiditis and granulomatous thyroiditis. 23-gauge needle is False-negative results are reported in up inserted into the thyroid mass, and several to 3% of cases, and follow-up is passes are made while recommended. aspirating the syringe. After releasing the A result of “atypia of unknown suction on the syringe, significance (AUS) or the needle is withdrawn and the cells are follicular lesion of unknown significance immediately placed on prelabeled dry glass (FLUS)” is obtained slides; some are immersed in a 70% alcohol in 3% to 6% of biopsies. The risk of solution while others are air dried. A malignancy in this scenario sample of the aspirate is is difficult to determine; however, it is also placed in a 90% alcohol solution for thought to be in the range cytospin or cell pellet. of 10% to 35% for FLUS and 60% to 75% for The slides are stained by Papanicolaou’s AUS. Clinical or Wright’s stains and correlation and a repeat FNA are examined under the microscope. If a bloody recommended for AUS lesions aspirate is obtained, (which often results in a more definitive the patient should be repositioned in a interpretation), although more upright position and clinical observation or surgery may be the biopsy repeated with a finer (25- to appropriate because of 30-gauge) needle. worrisome clinical or ultrasound findings. After FNAB, the majority of nodules can be The category of classified into “follicular neoplasm” is intended to several categories that determine further identify nodules that might management. To be follicular carcinomas. The term suspicious address the issue of variability in the for a follicular terminology of fine-needle neoplasm is preferred by some laboratories aspiration (FNA), the National Cancer for this category Institute (NCI) hosted the because up to 35% of cases turn out not to “NCI Thyroid Fine Needle Aspiration State be neoplasms but of the Science hyperplastic proliferations of follicular Conference,” which then defined the cells, most commonly Bethesda criteria for those of multinodular goiter. Lobectomy is thyroid FNA.19 Accordingly, optimum cytology the preferred treatment specimens for this result, and approximately 15% to should have at least six follicles each 35% of lesions containing at least 10 to 15 placed in this category prove to be cells from at least two aspirates. malignant. Hurthle cell neoplasms The FNA is classified as “nondiagnostic or are also included in this category. Most unsatisfactory” papillary and other in 2% to 20% of cases and typically results carcinomas can be diagnosed by FNA, but the from a virtually acellular features are subtle specimen, cyst fluid, or the presence of at times, such as in follicular variant of blood or clotting papillary carcinomas. If the artifact. The risk of malignancy in this diagnosis is uncertain, the lesions are setting ranges from 1% to classified as “suspicious for 4%, and reaspiration under ultrasound malignancy.” Lobectomy or near-total guidance is recommended. thyroidectomy is recommended A “benign” result is obtained in 60% to because more than 60% turn out to be 70% of thyroid FNAs. malignant. This category also includes lesions suspicious of malignancy, such as fine stippled for medullary carcinoma calcification and enlarged and lymphoma, and ancillary testing such as regional nodes; however, a tissue diagnosis immunohistochemical is strongly recommended analysis and flow cytometry may be helpful. before thyroidectomy.20 Ultrasound also The risk of provides a malignancy in lesions classified as “ noninvasive and inexpensive method of malignant” by FNA is 97% to following the size of 99%, and near-total/total thyroidectomy is suspected benign nodules diagnosed by FNAB recommended. and for identifying Laboratory Studies Most patients with thyroid enlarged lymph nodes. Ultrasound nodules are elastography is used euthyroid. Determining the blood TSH level to evaluate tissue stiffness noninvasively. is helpful. If a This technique takes patient with a nodule is found to be advantage of the fact that malignant hyperthyroid, the risk of nodules tend to be harder malignancy is approximately 1%. Serum Tg than benign nodules and thus deform less levels cannot differentiate compared with the benign from malignant thyroid nodules surrounding normal thyroid parenchyma. 21 unless the levels Larger studies are are extremely high, in which case warranted before elastography can be metastatic thyroid cancer routinely included in the should be suspected. Tg levels are, evaluation of thyroid nodules. CT and MRI however, useful in following are unnecessary in patients who have undergone total the routine evaluation of thyroid tumors thyroidectomy for thyroid except for large, fixed, cancer and also for serial evaluation of or substernal lesions. Scanning the thyroid patients undergoing with 123I or 99mTc nonoperative management of thyroid nodules. is rarely necessary, and thyroid scanning Serum calcitonin currently is recommended levels should be obtained in patients with in the assessment of thyroid nodules only MTC or a family history in patients of MTC or MEN2. There is insufficient who have follicular thyroid nodules on FNAB evidence to recommend and a suppressed routine calcitonin testing for all nodules. TSH. PET scanning does not play a major All patients with role in the primary MTC should be tested for RET oncogene evaluation of thyroid nodules. mutations and have a Management. Malignant tumors are treated 24-hour urine collection with measurement by thyroidectomy, of levels of vanillylmandelic as discussed earlier and later in this acid (VMA), metanephrine, and catecholamine chapter in Surgical Treatment levels to rule out a coexisting under Malignant Thyroid Disease. Simple pheochromocytoma. About 10% thyroid cysts of patients with familial MTC and MEN2A resolve with aspiration in about 75% of have de novo RET cases, although some mutations, so that their children are at require a second or third aspiration. If risk for thyroid cancer. the cyst persists after Imaging Ultrasound is helpful for detecting three attempts at aspiration, unilateral nonpalpable thyroid thyroid lobectomy is recommended. nodules, differentiating solid from cystic Lobectomy also is recommended for cysts >4 nodules, and cm in identifying adjacent lymphadenopathy. diameter or complex cysts with solid and Ultrasound evaluation cystic components, as can identify features of a nodule that the latter have a higher incidence of increase the a priori risk malignancy (15%). When FNAB is used in complex nodules, the solid oncogenes and tumor suppressor genes are portion should be involved in thyroid sampled. If a colloid nodule is diagnosed tumorigenesis,22 as depicted in Table 38-5. by FNAB, patients The RET should still be observed with serial proto-oncogene (Fig. 38-15) plays a ultrasound and Tg measurements. significant role in the If the nodule enlarges, repeat FNAB often pathogenesis of thyroid cancers. It is is indicated. located on chromosome Although controversial, levothyroxine in 10 and encodes a receptor tyrosine kinase, doses sufficient to which binds several maintain a serum TSH level between 0.1 and growth factors such as glial-derived 1.0 U/mL may neurotrophic factor also be administered. In areas with a high and neurturin. The RET protein is expressed prevalence of iodine in tissues derived deficiency, this can decrease nodule size from the embryonic nervous and excretory and potentially prevent systems. Therefore, the growth of new nodules. In iodine- RET disruption can lead to developmental sufficient populations, the abnormalities in data are less impressive. Randomized organs derived from these systems, such as controlled trial analyses the enteric nervous have shown that less than 25% of benign system (Hirschsprung’s disease) and nodules shrink more kidney. Germline mutations than 50% with TSH suppression in iodine- in the RET proto-oncogene are known to replete populations. predispose to MEN2A, MEN2B, and familial Thyroidectomy should be performed if a MTCs, and somatic mutations nodule enlarges on have been demonstrated in tumors derived TSH suppression or causes compressive from the neural crest, symptoms, or for cosmetic such as MTCs (30%) and pheochromocytomas. reasons. An exception to this general rule The tyrosine is the patient kinase domain of RET can fuse with other who has had previous irradiation of the genes by rearrangement. thyroid gland or has These fusion products also function as a family history of thyroid cancer. In oncogenes and these patients, total or have been implicated in the pathogenesis of near-total thyroidectomy is recommended PTCs. At least 15 because of the high RET/PTC rearrangements have been described incidence of thyroid cancer and decreased and appear to be reliability of FNAB early events in tumorigenesis. Young age in this setting. and radiation exposure Malignant Thyroid Disease seem to be independent risk factors for the In the United States, thyroid cancer development accounts for <1% of all of RET/PTC rearrangements. Up to 70% of malignancies (2% of women and 0.5% of men) papillary cancers and is the most in children exposed to the radiation rapidly increasing cancer in women. Thyroid fallout from the 1986 Chernobyl cancer is responsible disaster carry RET/PTC rearrangements, the for six deaths per million persons most common annually. Most patients being RET/PTC1 and RET/PTC3. These present with a palpable swelling in the rearrangements neck, which initiates confer constitutive activation of the assessment through a combination of receptor tyrosine kinases. history, physical examination, and FNAB. Molecular Genetics of Thyroid Tumorigenesis. Several
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