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== Overview == | == 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, [[Marginal zone B-cell lymphoma|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.<ref name=Radiopaedia 2015 Thyroid lymphoma>{{cite web | title = Radiopedia 2015 Thyroid lymphoma [Dr Henry Knipe]| url = http://radiopaedia.org/articles/thyroid-lymphoma }}</ref>. ''BRAF'' and ''NRAS'' mutations are asssociated 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 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. [[CT|Neck CT]] scan may be helpful in the [[diagnosis]] of thyroid lymphoma. Findings on [[CT]] scan suggestive of thyroid lymphoma is hypodense or heterogenous enhancement in the thyroid gland. [[Biopsy]] may be helpful in the [[diagnosis]] of thyroid lymphoma. Findings on [[biopsy]] suggestive of thyroid lymphoma include lymphoepithelial leision 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 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]]. | 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, [[Marginal zone B-cell lymphoma|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.<ref name=Radiopaedia 2015 Thyroid lymphoma>{{cite web | title = Radiopedia 2015 Thyroid lymphoma [Dr Henry Knipe]| url = http://radiopaedia.org/articles/thyroid-lymphoma }}</ref>. ''BRAF'' and ''NRAS'' mutations are asssociated 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 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. [[CT|Neck CT]] scan may be helpful in the [[diagnosis]] of thyroid lymphoma. Findings on [[CT]] scan suggestive of thyroid lymphoma is hypodense or heterogenous enhancement in the thyroid gland. [[Biopsy]] may be helpful in the [[diagnosis]] of thyroid lymphoma. Findings on [[biopsy]] suggestive of thyroid lymphoma include lymphoepithelial leision 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 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== | ==Classification== | ||
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, [[Marginal zone B-cell lymphoma|diffuse large B-cell lymphoma without marginal zone]], [[MALT lymphoma|marginal zone В-cell lymphoma of mucosa-associated lymphoid tissue]], and follicular lymphoma. | 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, [[Marginal zone B-cell lymphoma|diffuse large B-cell lymphoma without marginal zone]], [[MALT lymphoma|marginal zone В-cell lymphoma of mucosa-associated lymphoid tissue]], and follicular lymphoma. | ||
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* The [[thyroid]] may be affected primarily or secondary to lymphoma elsewhere. | * The [[thyroid]] may be affected primarily or secondary to lymphoma elsewhere. | ||
* There is increased risk with chronic inflammatory conditions. | * There is increased risk with chronic inflammatory conditions. | ||
* ''BRAF'' and ''NRAS'' mutations are asssociated with thyroid lymphoma. | * ''BRAF'' and ''NRAS'' mutations are asssociated with thyroid lymphoma. | ||
* MAPK pathway mutations are associated with B cell thyroid lymohoma.<ref name="AggarwalSwerdlow2012">{{cite journal|last1=Aggarwal|first1=Nidhi|last2=Swerdlow|first2=Steven H|last3=Kelly|first3=Lindsey M|last4=Ogilvie|first4=Jennifer B|last5=Nikiforova|first5=Mariana N|last6=Sathanoori|first6=Malini|last7=Nikiforov|first7=Yuri E|title=Thyroid carcinoma-associated genetic mutations also occur in thyroid lymphomas|journal=Modern Pathology|volume=25|issue=9|year=2012|pages=1203–1211|issn=0893-3952|doi=10.1038/modpathol.2012.73}}</ref> | * MAPK pathway mutations are associated with B cell thyroid lymohoma.<ref name="AggarwalSwerdlow2012">{{cite journal|last1=Aggarwal|first1=Nidhi|last2=Swerdlow|first2=Steven H|last3=Kelly|first3=Lindsey M|last4=Ogilvie|first4=Jennifer B|last5=Nikiforova|first5=Mariana N|last6=Sathanoori|first6=Malini|last7=Nikiforov|first7=Yuri E|title=Thyroid carcinoma-associated genetic mutations also occur in thyroid lymphomas|journal=Modern Pathology|volume=25|issue=9|year=2012|pages=1203–1211|issn=0893-3952|doi=10.1038/modpathol.2012.73}}</ref> | ||
* Thyroid lymphoma is associated with [[hashimoto’s thyroiditis]]. | |||
<br> | |||
* [[ | |||
* Variable size | * Variable size | ||
* Rubbery mass with fish flesh appearance | * Rubbery mass with fish flesh appearance | ||
Microscopic Pathology<br> | |||
* On microscopic histopathological analysis, characteristic findings of thyroid lymphoma are: | * On microscopic histopathological analysis, characteristic findings of thyroid lymphoma are: | ||
:* Lymphoepithelial lesion | :* Lymphoepithelial lesion | ||
:* [[Plasma cells]] | :* [[Plasma cells]] | ||
:* Thyroid parenchyma displaced by [[lymphocytes]] | :* Thyroid parenchyma displaced by [[lymphocytes]] | ||
== Causes == | |||
== Risk Factors == | |||
== Screening == | |||
== Differentiating thyroid lymphoma from Other Diseasea == | |||
== Epidemiology and Demographics == | == Epidemiology and Demographics == | ||
===Incidence=== | ===Incidence=== | ||
| Line 37: | Line 36: | ||
===Gender=== | ===Gender=== | ||
* Females are more commonly affected with anaplastic thyroid cancer than males. The females to male ratio is approximately 3 to 1. | * Females are more commonly affected with anaplastic thyroid cancer than males. The females to male ratio is approximately 3 to 1. | ||
== Natural History, Complications and Prognosis== | == Natural History, Complications and Prognosis== | ||
* The factors of poor [[prognosis]] for patients with thyroid lymphoma are 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 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. | ||
| Line 50: | Line 48: | ||
* Diffuse large cell lymphomas have worse prognosis and aggressive course | * Diffuse large cell lymphomas have worse prognosis and aggressive course | ||
* 5 year survival rate varies between 50-60%<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> | * 5 year survival rate varies between 50-60%<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> | ||
== Diagnosis == | |||
=== Diagnostic Study of Choice === | |||
===Staging=== | ===Staging=== | ||
* Staging of thyroid lymphoma is showed in the table below:<ref>{{cite book | last = Edge | first = Stephen | title = AJCC cancer staging manual | publisher = Springer | location = New York | year = 2010 | isbn = 978-0-387-88440-0 }</ref><br> | * Staging of thyroid lymphoma is showed in the table below:<ref>{{cite book | last = Edge | first = Stephen | title = AJCC cancer staging manual | publisher = Springer | location = New York | year = 2010 | isbn = 978-0-387-88440-0 }</ref><br> | ||
| Line 74: | Line 73: | ||
===Diagnostic Criteria=== | ===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. | 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=== | |||
===History=== | A directed history should be obtained to ascertain. | ||
A directed history should be obtained to ascertain | |||
*Painless [[neck mass]] | *Painless [[neck mass]] | ||
*[[Hoarseness]] | *[[Hoarseness]] | ||
*[[Dysphagia|Difficulty swallowing]] | *[[Dysphagia|Difficulty swallowing]] | ||
*[[Dyspnea|Difficulty breathing]] | *[[Dyspnea|Difficulty breathing]] | ||
=== Physical Examination === | === Physical Examination === | ||
====Throat ==== | ====Throat ==== | ||
* Rapidly growing painless mass in the neck | * Rapidly growing painless mass in the neck | ||
* [[Lymphadenopathy]] | * [[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 ==== | ====CT ==== | ||
* [[Goitre]], which is hypodense to adjacent muscle | * [[Goitre]], which is hypodense to adjacent muscle | ||
| Line 93: | Line 94: | ||
* T1/T2: iso- to hyperintense | * T1/T2: iso- to hyperintense | ||
* Pseudocapsule may be present | * Pseudocapsule may be present | ||
====Biopsy==== | ====Biopsy==== | ||
[[Biopsy]] may be helpful in the diagnosis of thyroid lymphoma. Findings on biopsy suggestive of thyroid lymphoma include lymphoepithelial leision and [[lymphocyte]]. | [[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 == | == 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. | 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 ==== | ==== Acute Pharmacotherapies ==== | ||
| Line 105: | Line 105: | ||
==== Chronic Pharmacotherapies ==== | ==== Chronic Pharmacotherapies ==== | ||
* [[Radiotherapy]] | * [[Radiotherapy]] | ||
=== Surgery | === Surgery === | ||
* Surgical treatment might be performed for patients with thyroid lymphoma in addition to chemotherapy and radiation, particularly for [[MALT]] lymphomas. | * Surgical treatment might be performed for patients with thyroid lymphoma in addition to chemotherapy and radiation, particularly for [[MALT]] lymphomas. | ||
:* [[Thyroidectomy]] (rarely) | :* [[Thyroidectomy]] (rarely) | ||
:* Radical left neck dissection | :* Radical left neck dissection | ||
:* [[Tracheostomy]] is indicated for vocal cord paralysis | :* [[Tracheostomy]] is indicated for vocal cord paralysis | ||
=== Primary Prevention === | |||
=== Secondary Prevention === | |||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
Revision as of 14:59, 1 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 asssociated 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 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 hypodense or heterogenous enhancement in the thyroid gland. Biopsy may be helpful in the diagnosis of thyroid lymphoma. Findings on biopsy suggestive of thyroid lymphoma include lymphoepithelial leision 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 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
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, marginal zone В-cell lymphoma of mucosa-associated lymphoid tissue, and 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 asssociated with thyroid lymphoma.
- MAPK pathway mutations are associated with B cell thyroid lymohoma.[1]
- 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
Risk Factors
Screening
Differentiating thyroid lymphoma from Other Diseasea
Epidemiology and Demographics
Incidence
- Thyroid lymphoma is rare with an incidence of 1-2 per 100,0000
Age
- It typically presents between 50-70 years of age
Gender
- Females are more commonly affected with anaplastic thyroid cancer than males. The females to male ratio is approximately 3 to 1.
Natural History, Complications and Prognosis
- The factors of poor prognosis for patients with thyroid lymphoma are 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.
Complications
- Vocal cord paralysis
- Dyspnea
- Dysphagia
- Metastasis
Prognosis
- Prognosis depends on the histological grade 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%[2]
Diagnosis
Diagnostic Study of Choice
Staging
- Staging of thyroid lymphoma is showed in the table below:[3]
| 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
A directed history should be obtained to ascertain.
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
- ↑ 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.
- ↑ 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 }