A CASE OF PRIMARY THYROID LYMPHOMA. Prof Dr.Dilek Gogas Yavuz Marmara University School of Medicine Endocrinology and Metabolism Istanbul, Turkey

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1 A CASE OF PRIMARY THYROID LYMPHOMA Prof Dr.Dilek Gogas Yavuz Marmara University School of Medicine Endocrinology and Metabolism Istanbul, Turkey

2 38 year old female She recognized a mass in her right neck in April 2011 No pain, difficulty in swallowing, or change in voice P E: 2.5 cm nodül palpated in right thyroid lobe. No LAP

3 Thyroid USG Heterogen thyroid gland 26x16x29 mm heterogen nodül in istmus june 2011 FNAB july 2011 Bening cytology Lenfoid cells,thyrocytes Hashimato s thyroiditis?

4 LAB: (july 2011) TSH: 3.47µıU/L st3 : 3 pg/ml ( ) st4: 0.78 ng/dl ( ) Antithyroglobulin Ab: IU/mL (<115) Anti TPO Ab + (Agust 2011) TSH: 5,74 µıu/l st3: 5,56 pg/ml (3,26-5,83) st4: 10,31 ng/dl (7,71-16,09)

5 Thyroid USG August 2011 Nodules : right lob inferior 9x16mm right upper 25x19x16mm September 2011 Nodules Right lobe: 16x12 mm 22x12 mm, 27x17 mm, 23x12 mm,17x11 mm Nodules Tends to be conglomerate

6 Neck MRI Mass lesion : 14x10 cm: from ıstmus level to vacal cord area and extending under the skin and capsulated No LAP

7 Rapidly enlarging thyroid mass Re biopsy : lymphoid cells lymphocytic thyroiditis?

8 3 days after FNAB,she complained of rapidly growing mass in the middle of the neck It was soft and then it hardened. No pain or tenderness After 2 weeks, she recognised the color change on skin.

9 What is the most possible diagnosis? A) Riedel thyroiditis B) Hashimato s thyroiditis C) Medullary thyroid carcinoma D) Anaplastic thyroid carcinoma E) Thyroid lymphoma

10 FDG PET -CT

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12 After 6 months of her first visit thyroid mass was removed surgically Peripheral tissue was infiltrated

13 Pathology report Lymphoid hyperplasia,follicular pattern High grade diffuse B cell lymphoma

14 Immunohistochemistry CD20 + Bcl6 + Bcl2 + Pax5 + CD5 - CD3 - SiklinD1 - CD 20 Pax5

15 Ki 67 index > % 50

16 Lymphoma Located in thyroid and regional lymph nodes Bone marrow biopsy : negative for malignancy Serum LDH: x2 Serum b2 microglobulin : x3 Primary thyroid lymphoma

17 Ann Arbor staging Thyroid limited = I E (50%) Thyroid and locoregional nodes = II E (45%) Nodal groups on both sides of diaphragm = III E Stage II E Diffuse organ involvement = IV E bone marrow, gastrointestinal tract, lungs, liver, pancreas, and kidney

18 After surgery: Chemotherapy : 6 courses of Rituximab-CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone)

19 After surgery and chemotherapy No medication TSH :1.2 miu/ml TG: 34 ng/ml Anti TPO: positive LDH: normal B2 microglobulin:n PET-CT: no pathologic activity

20 Primary thyroid lymphomas non-hodgkin lymphomas Almost all are B-cell type Histologic types of thyroid NHL: Large cell (aggressive) Follicular MALT (indolent) Burkitt lymphoma (rare) High association with Hashimoto thyroditis and lymphocytic thyroiditis

21 Role of Hashimoto Thyroditis 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.

22 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

23 Was surgery neccessary?

24 Primary Thyroid Lymphoma and surgery Surgery alone has been proposed for the management of localized (stage IE) intrathyroidal MALT lymphoma Palliative surgery may be required to relieve pressure symptoms

25 Surgical biopsy may still be required to confirm the diagnosis : results of FNA are inconclusive diagnosis by using FNA is difficult ddx : Hashimoto s thyroiditis? FNA has a low accuracy for the diagnosis Open biopsy have recommended in all cases in order to definitely diagnose PTL or subtype and grade

26 What are treatment of choice in Primary thyroid lymphoma?

27 Treatment highly curable (it is vital that this condition be recognized early and treated correctly) 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

28 Optimal management of PTL remains controversial Local control of the disease can be achieved by surgery or radiotherapy alone or in conjunction Chemotherapy can control possible occult or disseminated disease

29 Survival the 5-year diseasefree survival by treatment was 76% for surgery/radiation therapy, 50% for chemotherapy alone 91% for combined multimodality therapy 10-year progression-free survival: 36.5% for R-CHOP 20% for CHOP 10-year overall survival was 43.5%, Int J Rad Oncol Biol Phys 1986;12:1813e21.

30 Chemotherapy : cisplatin or doxorubicin has limited efficacy tyrosine kinase inhibitors Selumetinib, sorafenib, sunitinib Thank you