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Thyroid Lymphoma

Thyroid Gland Lymphoma

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].

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]
  • A clearly enlarging goiter was observed before the diagnosis of lymphoma in 3 patients.[ncbi.nlm.nih.gov]
  • In the second patient, primary thyroid lymphoma was an incidental finding following thyroidectomy performed for nodular goiter.[ncbi.nlm.nih.gov]
  • The observations in diffuse group included goiter (10/10, 100.0%), marked echogenesity (10/10, 100.0%), heterogeneous echotexture (10/10, 100.0%), and cervical lymphadenopathy (4/10, 40.0%).[ncbi.nlm.nih.gov]
  • We report an uncommon case of a 67-yr-old man who underwent total thyroidectomy for multinodular goiter with tracheal compression.[ncbi.nlm.nih.gov]
Thyroid Nodule
  • The most frequent clinical presentation was a palpable thyroid nodule. All patients with lymphoma died. CONCLUSION: Thyroid lymphomas are uncommon and tend to worse outcomes.[ncbi.nlm.nih.gov]
  • Thirteen (60%) patients presented with thyroid nodule(s). Eleven (50%) presented with aerodigestive tract obstructive symptoms.[ncbi.nlm.nih.gov]
  • First patient a sixty four year old woman, admitted to hospital because of bilateral thyroid nodules. Histological subtype was B cell follicular lymphoma.[ncbi.nlm.nih.gov]
  • Three pts presented with a multinodular goiter, one pt with a solitary thyroid nodule and all had compressive symptoms. Thyroid function tests were normal at presentation and lymphocytic thyroiditis (LT) had been previously diagnosed in one pt.[endocrine-abstracts.org]
  • Increased chaotic vascularity in the thyroid nodules is a helpful sonographic finding for the screening of thyroid malignancy and prompt FNA or core biopsy [15].[journals.plos.org]
Constitutional Symptom
  • 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.[symptoma.com]
Chest Discomfort
  • 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]
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]
  • Of the three patients presenting with stridor, two had an open biopsy followed by the initiation of dexamethasone therapy and resolution of symptoms within 48 h.[ncbi.nlm.nih.gov]
  • 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]
  • Poor prognostic factors include advanced stage of the tumor ( stage IE), size greater than 10 cm, mediastinal involvement, rapid clinical growth, and the presence of dysphagia or stridor.[shifrinmd.com]
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]
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
  • 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]
  • CONCLUSION: A rapidly enlarging neck mass in the setting of Hashimoto's thyroiditis should raise suspicion for thyroid lymphoma.[ncbi.nlm.nih.gov]
  • The thyroid lymphoma manifests as rapidly enlarging neck mass causing respiratory difficulty. On physical examination, patients usually exhibit a firm thyroid and lymphadenopathy.[en.wikipedia.org]
  • Both cases presented as enlarging neck masses in elderly female patients, in association with lymphocytic thyroiditis.[ncbi.nlm.nih.gov]
  • The case of a 66-year-old woman with rapidly progressive respiratory distress caused by a massive anterior neck mass with tracheal compression is presented.[ncbi.nlm.nih.gov]
Babinski Sign
  • Babinski's sign was present bilaterally. Endocrinologic studies showed subclinical hypothyroidism. A thoracolumbar radiograph showed disc space narrowing. Thyroid sonography revealed a hypoechoic mass lesion in the right lobe of the thyroid gland.[ncbi.nlm.nih.gov]
Hyperreflexia
  • Neurologic examination revealed decreased muscle power and hyperreflexia in both lower legs. Babinski's sign was present bilaterally. Endocrinologic studies showed subclinical hypothyroidism. A thoracolumbar radiograph showed disc space narrowing.[ncbi.nlm.nih.gov]

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.
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

  • Only one patient showed treatment resistance and the regimen was changed; 42 patients (98%) responded to the treatment.[ncbi.nlm.nih.gov]
  • Information on treatment response was available in 154 patients; 149 patients (97%) responded to treatment.[ncbi.nlm.nih.gov]
  • METHODS: We conducted a retrospective review of data for ten patients (four men, six women) of mean age 65 (range 48-88) years, with histopathologically confirmed malignant thyroid lymphoma who underwent pre-treatment and post-treatment 18 F-FDG PET between[ncbi.nlm.nih.gov]
  • SUMMARY: Advances in the understanding of the histologic subtypes of PTL have led to more accurate diagnosis and tailored treatments. The introduction of a new chemotherapeutic agent has expanded the treatment paradigm for PTL.[ncbi.nlm.nih.gov]
  • BACKGROUND: To review the current literature on the treatment of anaplastic thyroid cancer (ATC) and thyroid lymphoma (TL). RESULTS: Both anaplastic carcinoma (ATC) and TL represent rare forms of thyroid cancer.[ncbi.nlm.nih.gov]

Prognosis

  • This is at odds with the British Thyroid Association statement that the prognosis of this condition is 'generally excellent'. The only presenting symptom found to be significantly associated with prognosis was dysphagia (p 0.001).[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]
  • Age or 80 years, advanced stage, no radiation/surgery, and large B-cell or follicular histology predicted worse prognosis in multivariate analysis.[ncbi.nlm.nih.gov]
  • The prognosis usually is favorable with proper treatment. Herein, we discuss the clinical diagnosis and treatment of thyroid lymphoma.[ncbi.nlm.nih.gov]
  • Unfavorable prognosis is related to extracapsular extension, blood vessel wall infiltration, diffuse architectural pattern, and cervical lymph node involvement.[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]

Epidemiology

  • Abstract A previous patho-epidemiological study indicated that thyroid lymphoma (TL) evolves among active lymphoid cells into chronic lymphocytic thyroiditis (CLTH), a thyroid-specific autoimmune disease.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
Sex distribution
Age distribution

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]

Prevention

  • Transplantation Substance Use and Addiction Surgery Surgical Innovation Surgical Pearls Teachable Moment Technology and Finance The Rational Clinical Examination Tobacco and e-Cigarettes Toxicology Trauma and Injury Treatment Adherence United States Preventive[jamanetwork.com]
  • […] 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]
  • 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]
  • Updated on: 05/03/17 Preventing Hashimoto’s Thyroiditis[endocrineweb.com]

References

Article

  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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Last updated: 2019-07-11 21:43