Primary thyroid lymphoma: Difference between revisions
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==Historical Perspective== | ==Historical Perspective== | ||
==Classification== | ==Classification== | ||
Thyroid lymphomas may be classified according to type of cells into 6 subtypes: | * Primary thyroid lymphoma is almost always of B cell lineage.<ref name="pmid1929820">{{cite journal |vauthors=Skarsgard ED, Connors JM, Robins RE |title=A current analysis of primary lymphoma of the thyroid |journal=Arch Surg |volume=126 |issue=10 |pages=1199–203; discussion 1203–4 |date=October 1991 |pmid=1929820 |doi=10.1001/archsurg.1991.01410340037006 |url=}}</ref><ref name="pmid8838117">{{cite journal |vauthors=Pedersen RK, Pedersen NT |title=Primary non-Hodgkin's lymphoma of the thyroid gland: a population based study |journal=Histopathology |volume=28 |issue=1 |pages=25–32 |date=January 1996 |pmid=8838117 |doi=10.1046/j.1365-2559.1996.268311.x |url=}}</ref> | ||
* Thyroid lymphomas may be classified according to type of [[cells]] into 6 subtypes: | |||
** [[Diffuse large B-cell lymphoma with marginal zone]] which is the most common subtype. | |||
** [[Marginal zone B-cell lymphoma|diffuse large B-cell lymphoma without marginal zone]], the second most common subtype. | |||
** Non–Hodgkin's B-cell lymphomas | |||
** [[Hodgkin's lymphoma]] | |||
** [[MALT lymphoma|marginal zone В-cell lymphoma of mucosa-associated lymphoid tissue]] | |||
** [[Follicular lymphoma]] | |||
== Pathophysiology== | == Pathophysiology== | ||
* Thyroid lymphoma is a rare malignant tumor constituting 1% to 2% of all thyroid malignancies and less than 2% of lymphomas. | * Thyroid lymphoma is a rare malignant tumor constituting 1% to 2% of all thyroid malignancies and less than 2% of lymphomas. | ||
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== Natural History, Complications and Prognosis== | == Natural History, Complications and Prognosis== | ||
* The factors of poor [[prognosis]] for patients with thyroid lymphoma are an advanced stage of the [[tumor]], large size (>10 cm) as well as spreading to [[mediastinum]]. The overall survival for primary thyroid lymphoma is 50% to 70%, ranging from 80% in stage IE to less than 36% in stage IIE and IVE in 5 years. | * The factors of poor [[prognosis]] for patients with thyroid lymphoma are an advanced stage of the [[tumor]], large size (>10 cm) as well as spreading to [[mediastinum]]. The overall survival for primary thyroid lymphoma is 50% to 70%, ranging from 80% in stage IE to less than 36% in stage IIE and IVE in 5 years. | ||
* Possible complications of these tumors include:<ref name="HedhiliKedous2015">{{cite journal|last1=Hedhili|first1=F.|last2=Kedous|first2=S.|last3=Jbali|first3=S.|last4=Attia|first4=Z.|last5=Dhambri|first5=S.|last6=Touati|first6=S.|last7=Chebbi|first7=A. Goucha|last8=El May|first8=A.|last9=Gritli|first9=S.|title=Primary thyroid lymphoma: Case report and review of the literature|journal=Egyptian Journal of Ear, Nose, Throat and Allied Sciences|volume=16|issue=2|year=2015|pages=171–173|issn=20900740|doi=10.1016/j.ejenta.2015.05.002}}</ref> | |||
** [[Vocal cord paralysis]] | |||
* | ** [[Dyspnea]] | ||
** [[Dysphagia]] | |||
** [[Metastasis]] | |||
* [[Prognosis]] depends on the stage of the [[tumor]]. | |||
** [[MALT lymphoma]] have better prognosis with a slower progressive course. | |||
** Diffuse large cell lymphomas have worse prognosis and aggressive course. | |||
** 5 year survival rate varies between 50-60%. | |||
== Diagnosis == | == Diagnosis == | ||
=== Diagnostic Study of Choice === | === Diagnostic Study of Choice === | ||
Revision as of 18:33, 2 October 2019
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]
To review the wikidoc page on thyroid cancer , click here.
Overview
Thyroid lymphomas may be classified according to type of cells into 6 subtypes: non–Hodgkin's B-cell lymphomas, Hodgkin's lymphoma, diffuse large B-cell lymphoma with marginal zone, diffuse large B-cell lymphoma without marginal zone, MALT lymphoma, and follicular lymphoma. Thyroid lymphoma is a rare malignant tumor constituting 1% to 2% of all thyroid malignancies and less than 2% of lymphomas. BRAF and NRAS mutations are associated with thyroid lymphoma. Thyroid lymphoma is rare with an incidence of 1-2 per 100,0000. The factors of poor prognosis for patients with thyroid lymphoma are an advanced stage of the tumor, large size (>10 cm) as well as spreading to mediastinum. The overall survival for primary thyroid lymphoma is 50% to 70%, ranging from 80% in stage IE to less than 36% in stage IIE and IVE in 5 years. Symptoms of thyroid lymphoma include painless rapidly growing neck mass, hoarseness, and difficulty in swallowing. Neck CT scan may be helpful in the diagnosis of thyroid lymphoma. Findings on CT scan suggestive of thyroid lymphoma is a hypodense or heterogeneous enhancement in the thyroid gland. Biopsy may be helpful in the diagnosis of thyroid lymphoma. Findings on biopsy suggestive of thyroid lymphoma include lymphoepithelial lesion and lymphocytes. Combined modality therapy is the most common approach for the initial treatment of thyroid lymphomas. The CHOP regimen (cyclophosphamide, doxorubicin, vincristine and prednisone) has been showed high effectiveness for many types of thyroid lymphoma. However, it is suggested to perform radiation therapy only for MALT lymphoma resulting in a 96% complete response, with only a 30% relapse rate. Surgical treatment might be performed for patients with thyroid lymphoma in addition to chemotherapy and radiation, particularly for MALT lymphoma.
Historical Perspective
Classification
- Primary thyroid lymphoma is almost always of B cell lineage.[1][2]
- Thyroid lymphomas may be classified according to type of cells into 6 subtypes:
- Diffuse large B-cell lymphoma with marginal zone which is the most common subtype.
- diffuse large B-cell lymphoma without marginal zone, the second most common subtype.
- Non–Hodgkin's B-cell lymphomas
- Hodgkin's lymphoma
- marginal zone В-cell lymphoma of mucosa-associated lymphoid tissue
- Follicular lymphoma
Pathophysiology
- Thyroid lymphoma is a rare malignant tumor constituting 1% to 2% of all thyroid malignancies and less than 2% of lymphomas.
- The thyroid may be affected primarily or secondary to lymphoma elsewhere.
- There is increased risk with chronic inflammatory conditions.
- BRAF and NRAS mutations are associated with thyroid lymphoma.
- MAPK pathway mutations are associated with B cell thyroid lymphoma.[3]
- Thyroid lymphoma is associated with hashimoto’s thyroiditis.
- Variable size
- Rubbery mass with fish flesh appearance
Microscopic Pathology
- On microscopic histopathological analysis, characteristic findings of thyroid lymphoma are:
- Lymphoepithelial lesion
- Plasma cells
- Thyroid parenchyma displaced by lymphocytes
Causes
- The exact cause of primary thyroid lymphoma is unclear. However, Hashimoto thyroiditis is a known risk factor for this disorder.[2]
Risk Factors
- Preexisting Hashimoto thyroiditis is a known risk factor for the primary thyroid lymphoma and is present among half of the patients.[4][5][2]
- The evidence for such association is the higher incidence of thyroid lymphoma in areas with a higher prevalence of Hashimoto thyroiditis.
Screening
Differentiating thyroid lymphoma from Other Diseases
- Primary thyroid lymphoma should be differentiated from other disorders causing enlarging thyroid masses, and compression symptoms. These disorders include other thyroid cancers as well as thyroid follicular adenomas.
- For more information on primary thyroid lymphoma, please click here.
Epidemiology and Demographics
- Thyroid lymphoma is rare with an incidence of 1-2 per 100,0000.
- It typically presents between 50-70 years of age.[6]
- Females are more commonly affected with anaplastic thyroid cancer than males. The females to male ratio are approximately 2-8 to 1.
Natural History, Complications and Prognosis
- The factors of poor prognosis for patients with thyroid lymphoma are an advanced stage of the tumor, large size (>10 cm) as well as spreading to mediastinum. The overall survival for primary thyroid lymphoma is 50% to 70%, ranging from 80% in stage IE to less than 36% in stage IIE and IVE in 5 years.
- Possible complications of these tumors include:[7]
- Prognosis depends on the stage of the tumor.
- MALT lymphoma have better prognosis with a slower progressive course.
- Diffuse large cell lymphomas have worse prognosis and aggressive course.
- 5 year survival rate varies between 50-60%.
Diagnosis
Diagnostic Study of Choice
Staging
- Staging of thyroid lymphoma is showed in the table below:[8]
| Stage | Characteristics |
|---|---|
| IE | Lymphoma is located within the thyroid |
| 2E | Lymphoma is located within the thyroid and regional lymph-nodes |
| 3E | Lymphoma is located at both sides of diaphragm |
| 4E | Dissemination of lymphoma |
Diagnostic Criteria
Thyroid lymphoma shows a diagnostic and therapeutic challenge in many cases, because some manifestation patterns are similar to anaplastic thyroid carcinoma (ATC). Performance of fine needle aspiration biopsy has helped to distinguish these two entities preoperatively.
History and Symptoms
- Common symptoms of primary thyroid lymphoma include:[9][2]
- Painless enlarging neck mass
- Hoarseness
- Difficulty swallowing
- Difficulty breathing
- Classic B symptoms of lymphoma occurs in less than 20% of patients.
Physical Examination
Throat
- Rapidly growing painless mass in the neck
- Lymphadenopathy
Laboratory Findings
Electrocardiogram
X-Ray
Echocardiography or Ultrasound
- Three patterns have been described: nodular (hypoechoic mass), diffuse (mixed echotexture) or mixed. Calcifications are uncommon.
CT
- Goitre, which is hypodense to adjacent muscle
- Heterogeneous enhancement but still less than adjacent muscle
MRI
- T1/T2: iso- to hyperintense
- Pseudocapsule may be present
Biopsy
Biopsy may be helpful in the diagnosis of thyroid lymphoma. Findings on biopsy suggestive of thyroid lymphoma include lymphoepithelial leision and lymphocyte.
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Combined modality therapy is the most common approach for the initial treatment of thyroid lymphomas. The CHOP regimen (cyclophosphamide, doxorubicin, vincristine and prednisone) has been showed high effectiveness for many types of thyroid lymphoma. However, it is suggested to perform radiation therapy only for MALT resulting a 96% complete response, with only a 30% relapse rate.
Acute Pharmacotherapies
Chronic Pharmacotherapies
Surgery
- Surgical treatment might be performed for patients with thyroid lymphoma in addition to chemotherapy and radiation, particularly for MALT lymphomas.
- Thyroidectomy (rarely)
- Radical left neck dissection
- Tracheostomy is indicated for vocal cord paralysis
Primary Prevention
Secondary Prevention
References
- ↑ Skarsgard ED, Connors JM, Robins RE (October 1991). "A current analysis of primary lymphoma of the thyroid". Arch Surg. 126 (10): 1199–203, discussion 1203–4. doi:10.1001/archsurg.1991.01410340037006. PMID 1929820.
- ↑ 2.0 2.1 2.2 2.3 Pedersen RK, Pedersen NT (January 1996). "Primary non-Hodgkin's lymphoma of the thyroid gland: a population based study". Histopathology. 28 (1): 25–32. doi:10.1046/j.1365-2559.1996.268311.x. PMID 8838117.
- ↑ Aggarwal, Nidhi; Swerdlow, Steven H; Kelly, Lindsey M; Ogilvie, Jennifer B; Nikiforova, Mariana N; Sathanoori, Malini; Nikiforov, Yuri E (2012). "Thyroid carcinoma-associated genetic mutations also occur in thyroid lymphomas". Modern Pathology. 25 (9): 1203–1211. doi:10.1038/modpathol.2012.73. ISSN 0893-3952.
- ↑ Holm LE, Blomgren H, Löwhagen T (March 1985). "Cancer risks in patients with chronic lymphocytic thyroiditis". N. Engl. J. Med. 312 (10): 601–4. doi:10.1056/NEJM198503073121001. PMID 3838363.
- ↑ Hyjek E, Isaacson PG (November 1988). "Primary B cell lymphoma of the thyroid and its relationship to Hashimoto's thyroiditis". Hum. Pathol. 19 (11): 1315–26. doi:10.1016/s0046-8177(88)80287-9. PMID 3141260.
- ↑ Holm, Lars-Erik; Blomgren, Henric; Löwhagen, Torsten (1985). "Cancer Risks in Patients with Chronic Lymphocytic Thyroiditis". New England Journal of Medicine. 312 (10): 601–604. doi:10.1056/NEJM198503073121001. ISSN 0028-4793.
- ↑ Hedhili, F.; Kedous, S.; Jbali, S.; Attia, Z.; Dhambri, S.; Touati, S.; Chebbi, A. Goucha; El May, A.; Gritli, S. (2015). "Primary thyroid lymphoma: Case report and review of the literature". Egyptian Journal of Ear, Nose, Throat and Allied Sciences. 16 (2): 171–173. doi:10.1016/j.ejenta.2015.05.002. ISSN 2090-0740.
- ↑ {{cite book | last = Edge | first = Stephen | title = AJCC cancer staging manual | publisher = Springer | location = New York | year = 2010 | isbn = 978-0-387-88440-0 }
- ↑ Katna, Rakesh; Shet, Tanuja; Sengar, Manju; Menon, Hari; Laskar, Siddharth; Prabhash, Kumar; D'Cruz, Anil; Nair, Reena (2013). "Clinicopathologic study and outcome analysis of thyroid lymphomas: Experience from a tertiary cancer center". Head & Neck. 35 (2): 165–171. doi:10.1002/hed.22928. ISSN 1043-3074.