|Year : 2021 | Volume
| Issue : 1 | Page : 149-151
|Concomittant occurence of well-differentiated thyroid carcinoma metastasis and chronic lymphocytic leukemia in the same lymph node along with internal jugular vein thrombus: a case report
Havva Sezer1, Dilek Yazıcı1, Serdar Tezelman2, Mehmet O Demirkol3, Ömer F Ünal4, Şükrü Dilege5, Orhun Ç Taşkın6, Önder Peker7, Yersu Kapran6, Bülent Çolakoğlu8, Murat S Aygün9, Burhan Ferhanoğlu10, Faruk Alagöl1
1 Department of Medicine, Division of Endocrinology, Koc University School of Medicine, Istanbul, Turkey
2 Department of Medicine, Division of General Surgery, American Hospital, Istanbul, Turkey
3 Department of Medicine, Division of Nuclear Medicine, Koç University School of Medicine, Istanbul, Turkey
4 Department of Medicine, Division of Otorhinolaryngology, Koc University School of Medicine, Istanbul, Turkey
5 Department of Medicine, Division of Thoracic Surgery, Koc University School of Medicine, Istanbul, Turkey
6 Department of Division of Pathology, Koc University School of Medicine, Istanbul, Turkey
7 Department of Medicine, Division of Pathology, American Hospital, Istanbul, Turkey
8 Department of Medicine, Division of Radyology, American Hospital, Istanbul, Turkey
9 Department of Medicine, Division of Radyology, Koc University School of Medicine, Istanbul, Turkey
10 Department of Medicine, Division of Hematology, American Hospital, Istanbul, Turkey
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|Date of Submission||13-May-2020|
|Date of Decision||19-Jun-2020|
|Date of Acceptance||09-Jul-2020|
|Date of Web Publication||8-Jan-2021|
| Abstract|| |
Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is the most common adult leukemia. The coexistence of CLL and papillary thyroid carcinoma (PTC) is extremely rare. PTC sometimes shows microscopic vascular invasion but rarely cause a tumor thrombus in the internal jugular vein (IJV). It is also rare to find both differentiated and poorly differentiated types of thyroid cancer in the same metastatic location. We report a case of 63-year-old Turkish man with history of CLL who had CLL/SLL involvement and PTC metastasis in the same lymph node. Additionally, there was macroscopic metastasis to the IJV with poorly differentiated areas in the removed tumor thrombus. Patient was treated with total thyroidectomy, left radical neck dissection, resection of the left IJV segment that contained the tumor thrombus and radioactive iodine (RAI) therapy. Furthermore, metastatic lesions were found in the brain, lung and bone. Radiotherapy and chemotherapy were performed. However, our patient died approximately 12 months after thyroidectomy. To our knowledge, our present report is the first description with its current features.
Keywords: Chronic lymphocytic leukemia/small lymphocytic lymphoma, internal jugular vein thrombus, papillary thyroid carcinoma, poorly differentiated thyroid carcinoma
|How to cite this article:|
Sezer H, Yazıcı D, Tezelman S, Demirkol MO, Ünal &F, Dilege &, Taşkın O&, Peker &, Kapran Y, Çolakoğlu B, Aygün MS, Ferhanoğlu B, Alagöl F. Concomittant occurence of well-differentiated thyroid carcinoma metastasis and chronic lymphocytic leukemia in the same lymph node along with internal jugular vein thrombus: a case report. Indian J Pathol Microbiol 2021;64:149-51
|How to cite this URL:|
Sezer H, Yazıcı D, Tezelman S, Demirkol MO, Ünal &F, Dilege &, Taşkın O&, Peker &, Kapran Y, Çolakoğlu B, Aygün MS, Ferhanoğlu B, Alagöl F. Concomittant occurence of well-differentiated thyroid carcinoma metastasis and chronic lymphocytic leukemia in the same lymph node along with internal jugular vein thrombus: a case report. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 Apr 15];64:149-51. Available from: https://www.ijpmonline.org/text.asp?2021/64/1/149/306523
| IntroductİOn|| |
Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is the most common adult leukemia in the western countries. CLL/SLL is characterized by presence of mature small B-cell lymphocytes in the peripheral blood, bone narrow and lymphoid tissues. The term CLL is used when the disease manifests primarily in the blood, whereas the term SLL is used when involvement is predominantly in the lymph nodes. Association between CLL/SLL and other malignancies has been known. This is explained by defects in both cell-mediated and humoral immunity related to disease or applied therapy.
Papillary thyroid carcinoma (PTC) is the most common thyroid malignant tumor, generally has a favorable prognosis and commonly spreads to the lymph nodes. PTC rarely cause a tumor thrombus in the internal jugular vein (IJV). Poorly differentiated thyroid carcinoma (PDTC) is a rare subtype of thyroid carcinoma that is biologically situated between well-differentiated papillary/follicular thyroid carcinoma and anaplastic thyroid carcinoma.
The coexistence of CLL and PTC is extremely rare., It is also rare to find both differentiated and poorly differentiated types of thyroid cancer in the same metastatic location. We describe here a rare case of CLL/SLL and PTC coexistence. Additionally, there were both CLL/SLL involvement and PTC metastasis in the cervical lymph nodes. Our case had also PDTC into the removed IJV tumor thrombus. To our knowledge, our present report is the first description with its current features.
| Case Report|| |
We report a 63-year-old man with known history of CLL for 6 years. The patient was on remission and was followed only by complete blood count monitoring. He presented with a 1-month history of neck mass measuring 1.5 cm. Clinical examination revealed a hard solitary central-sided cervical lymphadenopaty. Neck ultrasonography (USG) confirmed the clinical diagnosis with the presence of a left-sided thyroid nodule measuring 1 cm.
He had leukocytosis with dominant neutrofile series (white blood cell count of 22.7 K/uL with 88.6% neutrophile). Hematocrit value was slightly below normal and platelet count was normal (35.8% and 219 K/uL, respectively). Lymph nodes were sampled by FNA and the results were consistent with PTC metastases. The patient then underwent total thyroidectomy with central cervical lymph node dissection. The histopathological findings were consistent with the presence of classical papillary thyroid microcarcinomas in the left lobe 0.9 cm and 0.1 cm in size [Figure 1]a, [Figure 1]b. Tumor capsules were absent. There was minimal extrathyroidal extension. In total, 6 lymph nodes were removed, 2 of them were positive for PTC metastasis. Perinodal invasion was present. There were findings in the same lymph nodes which were also positive for lymph node involvement with the patient's known CLL [Figure 1]e, [Figure 1]f. The diagnosis was supported by immunohistochemistry that showed positivity with CD20, CD5, CD23, and PAX5+. Bone marrow biopsy and body CT-scan revealed no further evidence of extranodal disease. No specific treatment was recommended for the CLL. [Figure 1].
|Figure 1: Classical histomorphologic features including papillary structures and typical nuclear features (a and b). Areas with poorly differentiated features including sheet-like/solid infiltration pattern and numerous mitosis were also encountered (c and d). One of the affected lymph nodes. Small neoplastic lymphoid cells were seen. Immunohistochemistry showed positivity with CD20, CD5, CD23, and PAX5+ (not shown). Compatible with CLL/SLL (e and f). Transvers T1 weighted image of the neck with tumor thrombus in the left IJV (g)|
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He was then referred for radioactive iodine (RAI) ablation treatment. When he received RAI therapy, serum values were: TSH; 67.0 μIU/mL; thyroglobulin (tg): 64.5 ng/mL; anti-thyroglobulin antibodies (anti-Tg): 12.2 IU/mL (reference range <115). Three months after RAI therapy, our patient presented with a new hard mass on the left side of the neck. Serum values were: TSH: 0.08 μIU/mL; tg: 0.7 ng/mL. Radiological examination showed a solid mass 9 cm in size arising from the operated area and invading the left jugular vein wall with intravascular tumor thrombus [Figure 1]g. A left radical neck dissection with segmental resection of the left IJV that contained the tumor thrombus was performed. In total, 32 lymph nodes were removed, 11 of them had metastases. Pathological examination verified that the removed tumor thrombus tissue showed poorly differentiated features [Figure 1]c, [Figure 1]d. There were vascular and perinodal invasion. There was increased Ki-67 expression (25%). The BRAF V600E mutation was not found.
Furthermore, metastatic lesions were found in the brain, lung, and bone. Radiotherapy and chemotherapy were performed. Our patient complained of shortness of breath and fever. He was hospitalized to intensive care unit, but unfortunately he died.
| Discussion|| |
To our knowledge, this is the first case of CLL/SLL involvement and differentiated thyroid cancer (DTC) metastasis in the same lymph node, together with macroscopic metastasis to the IJV and poorly differentiated component in the tumor thrombus in the background of PTC.
The 2017 World Health Organization (WHO) Classification of Tumors of Endocrine Organs defined PDTC as a tumor with conventional criteria of malignancy (capsular penetration or vascular invasion) with solid, insular, or trabecular growth, without nuclear features of PTC, and increased mitotic activity, tumor necrosis or convoluted nuclei. Prognostic factors are increased age (≥45 years), large tumor size (≥5 cm), macroscopically evident of extrathyroidal extension and distant metastases. More than 10% expression of Ki-67 might be associated with poorer prognosis. Presence of even a minor component of poorly differentiated area in DTC is associated with worse outcomes.
Thrombus in the great cervical vascular structures is an extremely rare condition in thyroid cancer but it is related to mortality as in our patient. The pathogenesis of dedifferentiation in PTC is still unknown. BRAF and RAS mutations were the main drivers in aggressive thyroid carcinoma but PDTC had additional mutations such as TERT promoter mutation, CTNNB1, and TP53 mutation. We could only analyse BRAF mutation but it was absent. Intraluminal extension is not a contraindication for aggressive surgical treatment in DTCs. However, histopathological findings in the removed thrombus tissue supported PDTC in the present case. Doxorubicin treatment was then started. The average relapse free survival is less than one year and about 50% of patients die of the PDTC. Our patient died approximately 9 months after the second operation.
As well as surgery, RAI therapy, external beam of radiotherapy and chemotherapy, treatment with new tyrosine kinase inhibitors (TCIs) may be a new approach for treating PDTC in the future. There were only a few patients of PDTC treated with lenvatinib. Interestingly, the drug was effective in reducing the tumoral mass.
| Conclusion|| |
CLL/SLL and PTC can be rarely seen with together. Additionally, the presence of IJV thrombus contained poorly differentiated areas is extremely rare in patients with DTC. New TCIs may be tried in these patients.
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Conflict of interest
The authors declare that they have no conflict of interest.
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Department of Medicine, Division of Endocrinology, Koc University School of Medicine, Istanbul
Source of Support: None, Conflict of Interest: None