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Thyroid Lymphoma
Lymphatic System Cancer in the Thyroid

Thyroid lymphoma is a very rare malignant disease and is primarily encountered in elderly individuals, more commonly women, who suffer from Hashimoto's thyroiditis. A rapidly developing mass in the neck, accompanied by fever and constitutional symptoms in some cases, is the typical clinical presentation. Ultrasonography followed by a biopsy of the lesion is necessary to confirm the diagnosis.

Presentation

Primary thyroid lymphoma (PTL) is very rarely encountered in clinical practice, as it comprises less than 5% of all malignancies that arise from this organ [1] [2] [3]. Lymphomas that develop in the thyroid are almost always of B-cell origin, the most common being diffuse large B-cell lymphoma (DLBCL) and mucosa-associated lymphoid tissue (MALT) lymphoma, while follicular lymphoma, small lymphocytic lymphoma, Burkitt's lymphoma and Hodgkin's lymphoma are other recognized forms [1] [3]. PTL is principally diagnosed in the sixth and seventh decades of life, and a significant predilection toward female gender has been established [1] [2] [4] [5]. Furthermore, a markedly higher number of PTLs are diagnosed in patients with preexisting Hashimoto's thyroiditis, implying its important role in the pathogenesis of PTL [1] [3] [5] [6]. An enlarging painless mass in the neck, seen in almost 70% of cases, is the main clinical manifestation of PTL, and the mass can compress the adjacent structures in the neck, resulting in dysphagia, dyspnea, stridor, and hoarseness in 30-50% of patients [3] [4] [6]. In addition, constitutional symptoms of fever, weight loss, and night sweats, which are typical for B-cell lymphomas, may be present as well but are reported in only 10% of individuals [2] [3].

Entire Body System

  • Goiter

    METHODS: The sonographic findings for 13 surgically proven primary thyroid lymphomas were analyzed and compared to those for 27 nodular goiters. [ncbi.nlm.nih.gov]

    The typical presentation is a rapidly enlarging goiter with symptoms or signs of tracheal, esophageal or neck vein compression. In 10–20% there is a known goiter often with hypothyroidism. [endocrine-abstracts.org]

  • Thyroid Nodule

    The clinical presentations include an enlarging neck mass, but patients may also present with symptoms of dysphagia, hoarseness and choking, or a cold thyroid nodule. [mayoclinic.pure.elsevier.com]

    thyroid nodules or goiter, because its prognosis and treatment differ substantially from that of the other disorders. [endocrine-abstracts.org]

    Thirteen (60%) patients presented with thyroid nodule(s). Eleven (50%) presented with aerodigestive tract obstructive symptoms. [ncbi.nlm.nih.gov]

  • Pain

    (A swollen lymph node is normally NOT as painful as an infected lymph node, which is common and can be painful.) [lymphoma.ca]

    I have not felt well and have had constant back pain for 2 months (feels like possible nerve pain). My hip bones also ache. [healthquestions.medhelp.org]

    But as it grows, it can cause pain and swelling in your neck. Several types of thyroid cancer exist. Some grow very slowly and others can be very aggressive. Most cases of thyroid cancer can be cured with treatment. [mayoclinic.org]

    Veterans Affairs Home-Based Primary Care Population for Patients at High Risk of Osteoporosis Using Voogle to Search Within Patient Records in the VA Corporate Data Warehouse Standardizing the Use of Mental Health Screening Instruments in Patients With Pain [mdedge.com]

    Her complaints on application were epigastric pain, burning, and constipation. [hindawi.com]

  • Familial Adenomatous Polyposis

    However, deciding who to test is difficult because, unlike familial adenomatous polyposis, there is no typical phenotypic appearance. [msdmanuals.com]

    Genetic syndromes that increase the risk of thyroid cancer include familial medullary thyroid cancer, multiple endocrine neoplasia, Cowden's syndrome and familial adenomatous polyposis. [mayoclinic.org]

    […] two; At least two generations have been affected; At least one person received a diagnosis of colorectal cancer before age 50; Familial adenomatous polyposis has been excluded. [my.clevelandclinic.org]

  • Anemia

    THE discovery of autoimmune thyroiditis by Witebsky and Rose 1 and Roitt and Doniach 2 has been confirmed by others 3, 4 and has established the thyroid gland as one of the commonest sites for the development of autoimmunization.Autoimmune hemolytic anemia [nejm.org]

    Some examples include: Addison's disease Graves' disease premature ovarian failure type 1 diabetes lupus erythematosus (a disorder that causes inflammation in a number of the body's systems, including the lungs and heart) pernicious anemia (a disorder [endocrineweb.com]

    Familial High-Risk Assessment: Colorectal Prostate Cancer Early Detection NCCN GUIDELINES FOR SUPPORTIVE CARE Cancer-Associated Venous Thromboembolic Disease Hematopoietic Growth Factors NCCN Guidelines Management of Cancer- and Chemotherapy-Induced Anemia [nccn.org]

    […] include: Presence of a lump in the neck/throat that may be accompanied by pain Hoarseness of voice; voice fatigue Tickling sensation in throat Dry and sore throat Dry cough Unintentional weight loss; changes in appetite Fatigue and weakness, headache Anemia [dovemed.com]

Respiratoric

  • Hoarseness

    The clinical presentations include an enlarging neck mass, but patients may also present with symptoms of dysphagia, hoarseness and choking, or a cold thyroid nodule. [mayoclinic.pure.elsevier.com]

    Primary or secondary laryngeal pathologies may manifest similar symptoms including dysphonia, hoarseness, dysphagia: a cervical mass can be found. [bjorl.org]

    Her treatment course was uneventful and her hoarseness improved after treatment (Fig. 5). [scielo.br]

    In some cases, patients may develop hoarseness or difficulty swallowing. Patients with compressive symptoms, enlarged lymph nodes, hoarseness, and/or a rapidly growing mass in the neck, should seek medical evaluation right away. [columbiasurgery.org]

    RESULTS: The most common complaints on admission were a rapidly growing cervical mass, hoarseness, and dyspnea. In all the cases, thyroid hormone levels were normal, but thyroglobulin levels were 5 to 10 times as high as normal. [ncbi.nlm.nih.gov]

  • Stridor

    We report the case of 71 year old woman who presented with severe inspiratory stridor due to a compressing primary thyroid lymphoma. [ncbi.nlm.nih.gov]

    We hereby conclude that, in a preexisting case of Hashimotos thyroiditis, PTL has to be ruled out especially if there is sudden increase in size, dysphagia, stridor, and hoarseness of voice. [cancerjournal.net]

    An enlarging painless mass in the neck, seen in almost 70% of cases, is the main clinical manifestation of PTL, and the mass can compress the adjacent structures in the neck, resulting in dysphagia, dyspnea, stridor, and hoarseness in 30-50% of patients [symptoma.com]

    Abstract Tumors originating in the neck are well-known causes of progressive dysphagia and dyspnea (including stridor), and thyroid lymphoma is an uncommon example. [uknowledge.uky.edu]

  • Cough

    This checklist lists the most common symptoms of lymphoma: Chills/temperature swings Fevers (especially at night) Unexplained weight loss Loss of appetite Unusual tiredness/lack of energy Persistent coughing Breathlessness Persistent itch all over the [lymphoma.ca]

    My initial symptoms were treated with the Levothyroxine for many years that's why I became concerned when the new symptoms began (trouble swallowing, lump growth, hoarseness, coughing, breathing problems). [medhelp.org]

    As thyroid cancer progresses, the following symptoms may occur: a lump in the throat a cough hoarseness pain in the throat and neck difficulty swallowing swollen lymph nodes in the neck Talk to your doctor if you have any of these symptoms. [healthline.com]

    Approximately 30%–50% of patients manifest compression symptoms of the adjacent structures, in addition to dysphagia, stridor, hoarseness, cough and a pressure sensation in the neck. [bjorl.org]

  • Persistent Cough

    This checklist lists the most common symptoms of lymphoma: Chills/temperature swings Fevers (especially at night) Unexplained weight loss Loss of appetite Unusual tiredness/lack of energy Persistent coughing Breathlessness Persistent itch all over the [lymphoma.ca]

Gastrointestinal

  • Dysphagia

    A 52-year-old female, known case of Hashimotos thyroiditis since 10 years on treatment came with dysphagia, dyspnea and rapidly enlarging thyroid swelling since 1 month. [cancerjournal.net]

    The clinical presentations include an enlarging neck mass, but patients may also present with symptoms of dysphagia, hoarseness and choking, or a cold thyroid nodule. [mayoclinic.pure.elsevier.com]

    CASE REPORT A 63-year-old male with a history of arterial hypertension, chronic active smoking and untreated hypothyroidism secondary to Hashimoto's thyroiditis, consulted by progressive cervical volume increase, associated with dysphonia, dysphagia and [pesquisa.bvsalud.org]

    Scott, "Thyroid Lymphoma as a Cause of Dysphagia and Dyspnea in a Patient without Palpable Nodules or Goiter" (2009). [uknowledge.uky.edu]

    DLBCL) diagnosed after surgery (subtotal thyroidectomy) by means of combined histology and immunohistochemistry (positive staining for CD-20) in two elderly female patients presenting at our institution for compressive symptoms (dysphonia in patient 1, dysphagia [ncbi.nlm.nih.gov]

  • Constipation

    Her complaints on application were epigastric pain, burning, and constipation. [hindawi.com]

    Other symptoms include bone pain and fractures, pain from kidney stones, depression, and constipation. Larger parathyroid cancers may also be found as a nodule near the thyroid. [cancer.org]

Cardiovascular

  • Hypertension

    CASE REPORT A 63-year-old male with a history of arterial hypertension, chronic active smoking and untreated hypothyroidism secondary to Hashimoto's thyroiditis, consulted by progressive cervical volume increase, associated with dysphonia, dysphagia and [pesquisa.bvsalud.org]

    Delivery Models Health Care Economics, Insurance, Payment Health Care Policy Health Care Quality Health Care Reform Health Care Safety Health Care Workforce Health Disparities Health Informatics Health Policy Hematology History of Medicine Humanities Hypertension [jamanetwork.com]

    She reported a history of diabetes mellitus, hypertension and hypothyroidism (chronic lymphocytic thyroiditis) and also cholecystectomy. [bjorl.org]

    Her medical history included hypertension. There was no known history of cervical irradiation or family history of thyroid cancer. [ncbi.nlm.nih.gov]

Musculoskeletal

  • Osteoporosis

    Organizations Through the Eyes of VIONE, A Clinical Program to Improve Patient Safety by Deprescribing Potentially Inappropriate Medications and Reducing Polypharmacy Evaluating a Veterans Affairs Home-Based Primary Care Population for Patients at High Risk of Osteoporosis [mdedge.com]

Face, Head & Neck

  • Neck Mass

    EVIDENCE SYNTHESIS: Primary thyroid lymphoma should be suspected in patients with a rapidly enlarging neck mass, especially in women with Hashimoto's thyroiditis. [ncbi.nlm.nih.gov]

    The clinical presentations include an enlarging neck mass, but patients may also present with symptoms of dysphagia, hoarseness and choking, or a cold thyroid nodule. [mayoclinic.pure.elsevier.com]

Workup

Many reports have emphasized the benefit of early recognition of PTL, primarily because therapy is quite effective in its earlier stages [1] [2] [4] [5]. However, studies have shown that up to 36 months may pass from the onset of symptoms to the diagnosis, which markedly affects survival rates and treatment outcomes [1] [2] [3] [4] [5]. For this reason, a meticulous clinical workup must be performed in order to obtain enough evidence to raise clinical suspicion toward PTL. Firstly, patients should be asked about the course and progression of symptoms, whereas a detailed physical examination of the neck can result in palpation of a hard and smooth-surfaced mass [4]. If a mass is palpated, immediate employment of imaging techniques, such as ultrasonography (US), is necessary, and a hypoechoic homogeneous mass with an enhanced posterior echoes is highly suggestive of PTL [2] [4]. Based on US findings, PTL is further divided into nodular (unilateral and pseudocystic changes), diffuse (bilateral and possessing indistinct borders) and mixed, which shows multiple patchy lesions [4]. Because the diagnosis is not conclusive without a histopathological examination, a biopsy of the lesion, most commonly in the form of fine-needle aspiration (although core-incision and open biopsy are mentioned as possible methods as well), is the vital step in workup [1]. Not only does it confirm the diagnosis, but it also helps in determining the stage of the tumor, together with computed tomography (CT) and other imaging modalities [1] [2] [4] [5] [6]. Based on the extent of tumor involvement and its dissemination, PTL is classified into [3]:

  • Stage I - Only local growth is observed.
  • Stage II - Dissemination into the lymphatic system within the same side of the diaphragm.
  • Stage III - Infiltration of distant lymph nodes and/or spleen.
  • Stage IV - The presence of distant metastases.

X-Ray

  • Mediastinal Mass

    Good luck and keep us posted Cheers 21 yr old male (Our Son) Diffuse Large B-Cell Non Hodgkins Lymphoma Stage 1A Mediastinal Mass 8cm x 7cm Diagnosed October 09 Started course of 6 x R-CHOP 14 in Nov 09 Course complete Jan 2010 Remission declared March [cancerforums.net]

Treatment

The ideal treatment of thyroid lymphoma was further assessed for both diffuse B-cell and MALT histologic subtypes. [ncbi.nlm.nih.gov]

Prognosis

The prognosis is generally excellent but can be varied because of the heterogeneous nature of thyroid lymphomas. [ncbi.nlm.nih.gov]

Etiology

His TSH was elevated & Anti-TPO antibodies were positive suggesting an autoimmune etiology. Ultrasound showed, an ill-defined heterogeneous mass in the left lobe of thyroid gland with multiple enlarged lymph nodes. [ncbi.nlm.nih.gov]

The etiology of thyroid lymphoma is unclear. Hashimoto thyroiditis is a major risk factor (~60 times increased risk) but development of thyroid lymphoma is still rare in this group. [radiopaedia.org]

According to one theory, the antigen triggering the reaction is a viral protein with molecular mimicry to a thyroid protein, although clear evidence for a viral etiology is lacking. [jpma.org.pk]

(Etiology) Lymphocytes are a type of white blood cells that are responsible for providing immunity in the human body. B-cells and T-cells are the two different types of lymphocytes. [dovemed.com]

Epidemiology

We review the epidemiology, clinical presentation, diagnosis, and treatment of this rare disorder. [ncbi.nlm.nih.gov]

Date: 1989-09 Reference: British Journal of Surgery 76(9):895-897 Sep 1989 Rights: Copyright: British Journal of Surgery Society Ltd Abstract: Abstract We have reviewed the local experience of 15 cases of thyroid lymphoma with a view to documenting the epidemiology [researchspace.auckland.ac.nz]

About the Journal European Journal of Oncology publishes contributions in the various areas of oncology: biology, epidemiology, pathology and clinical medicine. [mattioli1885journals.com]

Epidemiology and Prognosis Primary thyroid lymphoma is rare, constituting only 1-2% of all extranodal lymphomas and approximately 2-8% of all thyroid malignancies. [6, 5, 7, 8] As with other non-Hodgkin lymphomas, the median age of presentation in patients [emedicine.medscape.com]

Pathophysiology

[…] thyroid MALToma, radiation therapy alone is probably adequate. [2, 3] Primary T-cell lymphoma of thyroid is extremely rare, accounting for less than 2% of all primary thyroid lymphomas, and can present both diagnostic and therapeutic challenges. [4] Pathophysiology [emedicine.medscape.com]

Pathologically, two thirds of thyroid NHL are represented by DLBCL, arising either de novo or in the context of a concomitant indolent NHL of the mucosa-associated lymphatic tissue (MALT). 4 From a pathophysiological point of view, chronic antigen stimulation [hormones.gr]

Prevention

Prevention Doctors aren't sure what causes most cases of thyroid cancer, so there's no way to prevent thyroid cancer in people who have an average risk of the disease. [mayoclinic.org]

[…] and early detection of cervical cancer: American Cancer Society, American Society for Colposcopy and Cervical Pathology, and American Society for Clinical Pathology screening guidelines for the prevention and early detection of cervical cancer. [nccn.org]

If Lynch syndrome runs in a family, how can cancer be prevented? The only proven ways to reduce cancer risk is frequent cancer screenings and preventative prophylactic surgery when appropriate. [dph.illinois.gov]

References

  1. Gupta N, Nijhawan R, Srinivasan R, et al. Fine needle aspiration cytology of primary thyroid lymphoma: a report of ten cases. Cytojournal. 2005;2:21.
  2. Xia Y, Wang L, Jiang Y, Dai Q, Li X, Li W. Sonographic Appearance of Primary Thyroid Lymphoma-Preliminary Experience. Björklund P, ed. PLoS One. 2014;9(12):e114080.
  3. Peixoto R, Correia Pinto J, Soares V, Koch P, Taveira Gomes A. Primary thyroid lymphoma: A case report and review of the literature. Annals of Medicine and Surgery. 2017;13:29-33.
  4. Stein SA, Wartofsky L. Primary thyroid lymphoma: a clinical review. J Clin Endocrinol Metab. 2013;98(8):3131-3138.
  5. Verma D, Puri V, Agarwal S, Bhaskar A. Primary thyroid lymphoma: A rare disease. J Cytol. 2014;31(4):218-220.
  6. Lee SC, Hong SW, Lee YS, et al. Primary thyroid mucosa-associated lymphoid tissue lymphoma; a clinicopathological study of seven cases. J Korean Surg Soc. 2011;81(6):374-379.
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