Primary thyroid lymphoma
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords:
Overview
Classification
Thyroid lymphomas are classified as non–Hodgkin's B-cell lymphomas in a majority of cases, although Hodgkin's lymphoma of the thyroid has also been identified
Histopathology
The majority of thyroid lymphomas are non–Hodgkin's B-cell lymphomas; the rest exhibit properties of T-cell lymphomas .
- 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 (MALT)
- Follicular lymphoma
Pathophysiology
Thyroid lymphoma is a rare malignant tumor constituting 1% to 2% of all thyroid malignancies and less than 2% of lymphomas.Invalid parameter in <ref> tag
Rare.
Thyroid lymphoma is rare, accounting for a minority of both thyroid malignancies andlymphoma in general. Typically, it presents as a rapidly enlarging goitre with compressive symptoms and cervical lymphadenopathy common. B-type symptoms are uncommon (20%) and approximately half of patients are euthyroid.
The thyroid may be affected primarily or secondary to lymphoma elsewhere. This article is concerned with primary thyroid lymphoma.
Increased risk with chronic inflammatory conditions. Fit in the the greater category of MALT lymphoma.
Histopathology
The majority of thyroid lymphomas are non–Hodgkin's B-cell lymphomas; the rest exhibit properties of T-cell lymphomas .
- 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 (MALT)
- Follicular lymphoma
Features: Lymphoepithelial lesion - key feature. Plasma cells. "Overgrowth" - thyroid parenchyma displaced by lymphocytes.
Genetics
Associated Conditions
- Hashimoto’s thyroiditis
Gross Pathology
Microscopic Pathology
Causes
Life Threatening Causes
Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.
Epidemiology and Demographics
Incidence
- Thyroid lymphoma is rare with an incidence of 1-2 per 1,000,000
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 lymphomas 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%[1]
Staging
Staging of thyroid lymphoma is showed in the table below
| Stage | Characteristics |
|---|---|
| 1Е | Lymphoma is located within the thyroid |
| 2Е | Lymphoma is located within the thyroid and regional lymph-nodes |
| 3Е | Lymphoma is located at both sides of diaphragm |
| 4Е | 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 FNAB has helped to distinguish these two entities preoperatively.
History
A directed history should be obtained to ascertain
Symptoms
- Painless neck mass
- Hoarseness
- Dysphagia
- Signs of tracheal compression
Past Medical History
Family History
Social History
Occupational
Alcohol
The frequency and amount of alcohol consumption should be characterized.
Drug Use
Smoking
Allergies
Physical Examination
Appearance of the Patient
Vital Signs
Skin
Head
Eyes
Throat
- Rapidly growing painless mass in the neck
- Lymphadenopahy
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
Echocardiography or Ultrasound
- Three patterns have been described: nodular (hypoechoic mass), diffuse (mixed echotexture) or mixed. Calcifications are uncommon.
Treatment
Pharmacotherapy
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
- Steroids
Chronic Pharmacotherapies
- Radiotherapy
Surgery and Device Based Therapy
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
References
- ↑ 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.