Primary thyroid lymphoma
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords:
Overview
Historical Perspective
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.
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
Genetics
Associated Conditions
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.
Common Causes
Causes by Organ System
| Cardiovascular | No underlying causes |
| Chemical / poisoning | No underlying causes |
| Dermatologic | No underlying causes |
| Drug Side Effect | No underlying causes |
| Ear Nose Throat | No underlying causes |
| Endocrine | No underlying causes |
| Environmental | No underlying causes |
| Gastroenterologic | No underlying causes |
| Genetic | No underlying causes |
| Hematologic | No underlying causes |
| Iatrogenic | No underlying causes |
| Infectious Disease | No underlying causes |
| Musculoskeletal / Ortho | No underlying causes |
| Neurologic | No underlying causes |
| Nutritional / Metabolic | No underlying causes |
| Obstetric/Gynecologic | No underlying causes |
| Oncologic | No underlying causes |
| Opthalmologic | No underlying causes |
| Overdose / Toxicity | No underlying causes |
| Psychiatric | No underlying causes |
| Pulmonary | No underlying causes |
| Renal / Electrolyte | No underlying causes |
| Rheum / Immune / Allergy | No underlying causes |
| Sexual | No underlying causes |
| Trauma | No underlying causes |
| Urologic | No underlying causes |
| Dental | No underlying causes |
| Miscellaneous | No underlying causes |
Causes in Alphabetical Order
- A...
- Z...
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Epidemiology and Demographics
Age
Gender
Race
Developed Countries
Developing Countries
Risk Factors
Screening
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.
Diagnosis
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
Ear
Nose
Mouth
Throat
Heart
Lungs
Abdomen
Extremities
Neurologic
Genitals
Other
Laboratory Findings
Electrolyte and Biomarker Studies
Electrocardiogram
Chest X Ray
CT
MRI
Echocardiography or Ultrasound
Other Imaging Findings
Other Diagnostic Studies
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
Chronic Pharmacotherapies
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.