Primary Thyroid Lymphoma
Anastassia Doutova
Background
Primary thyroid lymphomas usually are of the non-
Hodgkin type. Primary thyroid Hodgkin disease is extremely rare. Further divided into aggressive and indolent cell types.
diffuse large B-cell lymphomas (80%) extranodal marginal zone lymphoma (20-30%) follicular lymphomas extranodal small lymphocytic lymphomas
Indolent NK cell T cell Mycosis fungoides T-cell large granular lymphocyte leukemia T-cell prolymphocytic leukemia B cell Small lymphocytic lymphoma Lymphoplasmacytic lymphoma Plasma cell myeloma Follicular lymphoma Marginal zone B-cell lymphoma Mantle cell lymphoma
Quick Lymphoma Overview NHL
Aggressive B cell
Follicular lymphoma (grade III) Diffuse large B-cell lymphoma Mantle cell lymphoma Burkitt’s Lymphoma (highly aggressive)
T cell
Peripheral T-cell lymphoma Anaplastic large cell lymphoma
Background
The normal thyroid gland contains no native
lymphocytic tissue.
Background
High association with Hashimotos thyroditis
and lymphocytic thyroiditis . Almost all are B-cell type Case reports of T-cell lymphomas in areas endemic for HTLV-I-associated adult T-cell leukemia/lymphoma
Statistics
Thyroid lymphomas constitute only 3% of all
NHLs approximately 5% of all thyroid neoplasms more common in women than in men (ratio 2:1 - 14:1) usually affect patients with a median age of 60 years
Clinical Presentation
enlarging thyroid mass/goiter
Hoarseness (vocal cord Paresis)
Respiratory difficulty Cough
dysphagia
Type B symptoms in 10% of patients
Work up
Serum LDH and beta2-microglobulin values
can be predictive of prognosis CT (donut sign)/PET((-) in MALT) FNA/ core biopsy Thyroid function tests Antithyroglobulin or antimicrosomal Ab’s Bone marrow aspiration and biopsy
HISTOLOGY
Important to avoid unnecessary operation
Histologic types of thyroid NHL:
Large cell (aggressive) Follicular MALT (indolent) Burkitt lymphoma (rare)
Ann Arbor staging
Thyroid limited = I E (50%)
Thyroid and locoregional nodes = II E (45%)
Nodal groups on both sides of diaphragm = III
E Diffuse organ involvement = IV E
bone marrow, gastrointestinal tract, lungs, liver, pancreas, and kidney
Prognosis
According to IPI (International Prognostic
Index) 1point per each
Age older than 60 years Performance status higher than 1 Elevated LDH Number of extranodal sites more than 1 Ann Arbor stage III-IV
5y survival – 0pt = 86%, 1-2 = 50% >3 = 20
%
Treatment
Treatment is based on the lymphoma subtype
and the extent of disease. management of large cell lymphoma based on prognostic factors With favorable IPI standard CHOP regimen (cyclophosphamide, doxorubicin, vincristine and prednisone) followed by radiation with # of courses ranging 3-6
Treatment
Patients with IPI >0 treated with 6 courses of
CHOP-Rituximab, irrespective of age those with poor prognoses are treated with experimental protocols
Role of Hashimoto Thyroditis
Goiter and intense lymphocytic infiltration Antibodies to thyroid peroxidase and
thyroglobulin Hashimoto’s Thyroditis increases the risk by 60 times chronic antigenic stimulation leads to proliferation of lymphoid tissue Hypothyroidism has been observed in 30-40% of patients with thyroid lymphoma.