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].

• Abdominal Lymphadenopathy

  No organomegaly or abdominal lymphadenopathy was noticed. Bone marrow aspirate and biopsy did not show any evidence of lymphoma. Serum lactate dehydrogenase was also within normal limits. Primary thyroid lymphoma is a rare disease. [jcytol.org]

• 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]

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

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

  […] diagnostic strategy for a thyroid nodule, but accuracy is quite low in thyroid lymphoma or anaplastic cancer. [bjorl.org]

• Soft Tissue Mass

  The most common soft tissue masses in anterior mediastinum are thymomas, teratomas, lymphomas, and ectopic or aberrant thyroid tissue ( thyroid not in its place). Yes, they can also be a chain of internal mammary lymph nodes. [healthquestions.medhelp.org]

  Type-2 PTL is the most common type in this series and shows diffuse lesions, which revealed mainly a homogeneous soft-tissue mass which was moderately enhanced on contrast CT. Calcification and necrosis are rare in this type. [tcr.amegroups.com]

  Li Y, Wang XB, Tian XY, Li B, Li Z: Unusual primary osseous Hodgkin lymphoma in rib with associated soft tissue mass: a case report and review of literature. Diagn Pathol. 2012, 7: 64- 27. [diagnosticpathology.biomedcentral.com]

• 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]

  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]

  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]

  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]

• Inspiratory 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]

• Progressive Dysphagia

  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]

• Subcutaneous Nodule

  Isolated thyroid mass was seen in 3 patients, thyroid mass with cervical lymphadenopathy was found in 6 patients and 3 patients had thyroid mass with multiple subcutaneous nodules ( Table 2 ). [npplweb.com]

• Bilateral Leg Weakness

  The patient, a 42-year-old man, presented with chest discomfort and bilateral leg weakness of one week's duration. Physical examination of his neck showed a right-sided mass lesion. [ncbi.nlm.nih.gov]

• Proximal Muscle Weakness

  A 55-year-old woman presented with a 3-week history of weakness. On examination, she had a heliotrope rash, V-neck and shawl signs. There was symmetric proximal muscle weakness, and 2/5 strength in upper and lower extremities. [ncbi.nlm.nih.gov]

• 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]

  Painless neck mass Hoarseness Difficulty swallowing Signs of tracheal compression Diagnosis[edit] Thyroid lymphoma poses a diagnostic and therapeutic challenge. [en.wikipedia.org]

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.

• 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]

• Left Pleural Effusion

  Ultrasound showed asymmetrical diffuse enlargement of left thyroid lobe lasting left thoracal area and a cervical lymph node. Chest xray revealed pleural effusion on left lung. [turkjem.org]

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

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

Prognosis[edit] The factors of poor prognosis for people with thyroid lymphoma are advanced stage of the tumor, large size (>10 cm) as well as spreading to mediastinum. [en.wikipedia.org]

CT goiter, which is hypodense to adjacent muscle heterogeneous enhancement but still less than adjacent muscle 3 MRI T1/T2 : iso- to hyperintense pseudocapsule may be present 3 Treatment and prognosis Prognosis is generally considered "excellent" for [radiopaedia.org]

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]

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]

[…] 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 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]

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.