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 Table of Contents  
Year : 2018  |  Volume : 15  |  Issue : 1  |  Page : 42-45

Cytodiagnosis of mucosa-associated lymphoid tissue lymphoma of thyroid gland: An extremely rare presentation in a young female

Department of Pathology, North Bengal Medical College, Darjeeling, West Bengal, India

Date of Web Publication23-Mar-2018

Correspondence Address:
Dr. Indranil Chakrabarti
Department of Pathology, North Bengal Medical College, Sushrutanagar, Darjeeling - 734 012, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/trp.trp_39_17

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Thyroid lymphoma, a rare tumor, constitutes about 1%–5% of all thyroid malignancies and 1%–2% of all extranodal lymphomas. The thyroid gland contains no native lymphoid tissue and is seen only in various pathological conditions such as primary thyroid lymphoma (PTL). PTL is more common in women than men (3:1 predominance) with a peak incidence in the sixth and seventh decades. Mucosa-associated lymphoid tissue (MALT) lymphoma of thyroid is one of the rare variants. Chronic autoimmune thyroiditis (Hashimoto's disease) has been associated with an increased risk of lymphoma, including MALT lymphoma. Here, we report a case of a 38-year-old female who presented with rapidly progressive swelling on the right side of the neck for previous 3 months. No history suggestive of hypothyroidism or hyperthyroidism was present. Ultrasonography showed enlarged thyroid gland with hypoechoic echotexture and multiple echogenic septations. Fine-needle aspiration cytology revealed features suggestive of MALT lymphoma. The swelling was operated, and subsequent histopathology and immunohistochemistry confirmed the diagnosis. Thereafter, the patient was treated by radiotherapy. However, after 6 months' follow-up, there was a recurrence of the tumor. Then, combination of cyclophosphamide, doxorubicin, vincristine, prednisone regime and rituximab was started, and the patient was free of recurrence at the next 12-month follow-up. Here, we report a very rare case of MALT lymphoma of Thyroid occurring in a young female with no known history of Hashimoto's thyroiditis. The tumor recurred after radiotherapy treatment and was successfully treated with subsequent chemotherapy.

Keywords: Fine needle aspiration cytology, immunohistochemistry, mucosa-associated lymphoid tissue lymphoma, thyroid, young female

How to cite this article:
Chakrabarti I, Mahata N, Das V, Mitra P. Cytodiagnosis of mucosa-associated lymphoid tissue lymphoma of thyroid gland: An extremely rare presentation in a young female. Thyroid Res Pract 2018;15:42-5

How to cite this URL:
Chakrabarti I, Mahata N, Das V, Mitra P. Cytodiagnosis of mucosa-associated lymphoid tissue lymphoma of thyroid gland: An extremely rare presentation in a young female. Thyroid Res Pract [serial online] 2018 [cited 2022 Dec 5];15:42-5. Available from: https://www.thetrp.net/text.asp?2018/15/1/42/228375

  Introduction Top

Primary thyroid lymphomas (PTLs) are rare tumors, constituting about 1%–5% of all thyroid malignancies and 1%–2% of all extranodal lymphomas.[1],[2] It is more common in women (3:1 predominance) with a peak incidence in the 60 s.[2],[3],[4],[5] Chronic autoimmune thyroiditis (Hashimoto's disease) has increased association with lymphoma, including mucosa-associated lymphoid tissue (MALT) lymphoma.[6] Pure thyroid MALT lymphomas are recognized as extranodal marginal zone B-cell lymphomas of MALT in the revised European American lymphoma classification of 1994 and the World Health Organization (WHO) classification of 1999[7],[8] and revised the WHO classification of 2008.[9] Clinically, it has an indolent course and better prognosis than non-MALT lymphomas.[10]

  Case Report Top

A 38-year-old female presented with rapidly progressive swelling on the right side of neck and difficulty in breathing especially on lying down for previous 3 months. No history suggestive of hypothyroidism or hyperthyroidism was present. As per the clinical notes, the swelling was firm in consistency and measured approx. 4 cm in diameter on the right side of the neck.

Ultrasonography (USG) showed diffusely enlarged right lobe of thyroid with heterogeneous hypoechoic echotexture and multiple echogenic septations [Figure 1]. Blood levels of triiodothyronine, thyroxine, and thyroid-stimulating hormone were within normal limits. Fine-needle aspiration was done in a private laboratory, and the patient was diagnosed as suffering from florid lymphocytic thyroiditis with foci of Hurthle cell changes. However, in view of progressively increasing swelling, the slides were reviewed by our center, and we found heterogeneous population of lymphoid cells of varying maturity and moderate atypia along with plasma cells and also the presence of lymphoepithelial lesions into the thyroid follicles. No Hurthle cell changes could be appreciated [Figure 2]. Based on the clinical, radiological, and cytological findings, a cytological diagnosis of Bethesda category V: Suspicious for lymphoma (non-Hodgkin lymphoma) was rendered. An additional note suggesting reaspiration for immunophenotyping studies by flow cytometry for definitive diagnosis, if clinically indicated, was added to the report. The patient did not turn up for repeat aspiration. Subsequently, after 2 weeks, right-sided hemithyroidectomy was done, and the resected specimen was received in our department for histopathological examination. Grossly, the specimen measured 5 cm × 8 cm × 3 cm, weighed 25 g. With no distinct nodules, cut section showed well-capsulated white homogeneous area. Microscopic examination showed that the presence of small lymphoid cells with variable proportions of centrocyte-like cells, plasma cells, plasmacytoid lymphocytes, monocytoid B-cells, and large transformed lymphocytes intermingled into thyroid follicles to form lymphoepithelial lesions [Figure 3]a. Immunohistochemistry (IHC) was done with a panel of PanCK, CD45, CD20, CD5, CD10, CD3, and cyclin D1 that showed strong positivity for PanCK [Figure 3]b, CD45 [Figure 3]c, and CD20 [Figure 3]d. All the other markers were negative. The cells showed light chain restriction by positive staining for Kappa and negative staining for lambda light chains. Histopathological diagnosis of extranodal marginal zone B-cell lymphoma-MALT type was rendered. Musshoff's modification of the Ann Arbor staging system was IE, and the International Prognostic Index was 0 for the patient.
Figure 1: Ultrasonogram showing enlarged right lobe with heterogeneous hypoechoic echotexture and multiple septations

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Figure 2: Fine needle aspiration cytology smear showing lymphoepithelial lesions without Hurthle cell changes and scanty colloid (Leishman, ×400)

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Figure 3: (a) Microphotograph showings histopathological picture of lymphoepithelial lesions and atypical lymphoid cells (H and E, ×400). (b) Thyroid epithelial islands having PanCK positivity (×400). (c) Lymphoid population having CD45 positivity (×400) and CD20 positivity (×400) (d)

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She was initially treated by radiotherapy, but on subsequent follow-up at 6 months, it was seen that there was a recurrence of the tumor that was confirmed by computed tomography scan of the neck [Figure 4]. Combination treatment of CHOP regime (cyclophosphamide, doxorubicin, vincristine, prednisone) and Rituximab were started thereafter. Ultimately, at the next 12 months' follow-up, the patient was free of tumor recurrence.
Figure 4: Postthyroidectomy follow-up computed tomography scan of neck showing homozygous, hypodense swelling at neck indicating recurrence of tumor

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  Discussion Top

PTL is a rare tumor, constitutes about 1%–5% of all thyroid malignancies and 1%–2% of all extranodal lymphomas.[1],[2] Among these, as extranodal marginal zone B-cell lymphoma-MALT type of thyroid is rarer variants that constitute 6%–27% of thyroid lymphoma overall.[11] MALT lymphomas, as they were previously known, generally occur in middle to older aged individuals, particularly females (male: female 1:2–4).[12] The clinical presentation of thyroid MALT lymphomas is variable. It may be asymptomatic to painful swelling with or without hoarseness, dysphagia, and dyspnea depending on predisposing thyroid lesion and also the growth of tumor. The studies had shown that this lymphoma arises in patients of autoimmune, lymphocytic, or Hashimoto's thyroiditis [4],[13] and the risk of lymphoma is 80 times more in autoimmune thyroiditis than control.[4] Imaging like USG has minimum role to diagnose such lesion. Cytological diagnosis can be very tricky in these situations with considerable overlap between lymphoid-rich lesions such as autoimmune thyroiditis and low-grade lymphomas. The presence of lymphoepithelial lesions and lack of Hurthle cell changes in a proper clinical setting should arouse the suspicion of a lymphoid malignancy. Flow cytometry of the aspirates and immunocytochemistry or IHC on cell blocks solve the issue in most of the cases. The high-grade lymphomas such as diffuse large cell lymphomas, on the other hand, are less challenging to diagnosis due the presence large atypical lymphoid cells on smears. Histologically, thyroid MALT lymphomas are characterized by the presence of atypical lymphoid cells, which originate within the marginal zone of the lymphoid follicles and can extend into the interfollicular space and/or into the germinal centers (follicular colonization).[14],[15] Small cell lymphomas (SLL) and mantle cell lymphomas are other common differentials for MALT lymphomas. The latter express B-cell-associated antigens (CD20, CD22, and CD79a) and are negative for CD5, CD10, CD23, CD3, and Cyclin D1.[7],[8] The lack of CD 5 and CD 23 in the present case ruled out the possibility of small cell lymphoma while the negativity for Cyclin D1 ruled out the possibility of mantle cell lymphoma.

The usual treatment for extranodal marginal zone B-cell lymphoma-MALT type is radiotherapy for localized disease and chemotherapy for disseminated disease, and recurrence can be treated with either radiotherapy or a combination of radiotherapy and chemotherapy. Retrospective reports suggest indolent behavior and an excellent clinical prognosis for this subset of thyroid lymphomas.[10],[16] Tsang et al.reported excellent local disease control in 13 patients with MALT lymphoma (stage I-IIE) treated with 30–35 Gy of radiotherapy.[11] For thyroid MALT lymphomas in stage IE or IIE with lymph nodal involvement, the combination chemotherapy treatment of CHOP Regime has been proposed.[17] In our case, the patient was better for follow-up of 12 months after starting of combination of CHOP regime and rituximab.

  Conclusion Top

PTLs are a rare group of tumors. Extranodal marginal zone B-cell lymphoma-MALT type is still rarer among thyroid lymphomas. Although uncommon, it can occur in a patient <40 years and that too without a background of autoimmune thyroiditis. Fine-needle aspiration cytology should be cautiously evaluated in such age group of the patients. Clinical features of rapid growth or obstructive symptoms should prompt suspicion of lymphomas in cases of florid lymphocytic collection in aspirates of thyroid swellings. The absence of Hurthle cell changes and lymphoepithelial lesions are important cytological clues for diagnosis. Flow cytometry of aspirates helps in diagnosis and categorization. Thus, clinical suspicion and proper investigations should be done to rule out MALT lymphoma of thyroid glands.

Research Highlights

  • Extranodal marginal zone B-cell lymphoma-mucosa-associated lymphoid tissue (MALT type) of thyroid are extremely rare malignancies
  • Very uncommon malignancy in young patients
  • Rarely reported without preexisting autoimmune thyroiditis
  • FNAC diagnosis of thyroid lymphoma rarely reported.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Graff-Baker A, Sosa JA, Roman SA. Primary thyroid lymphoma: A review of recent developments in diagnosis and histology-driven treatment. Curr Opin Oncol 2010;22:17-22.  Back to cited text no. 1
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Graff-Baker A, Roman SA, Thomas DC, Udelsman R, Sosa JA. Prognosis of primary thyroid lymphoma: Demographic, clinical, and pathologic predictors of survival in 1,408 cases. Surgery 2009;146:1105-15.  Back to cited text no. 3
Derringer GA, Thompson LD, Frommelt RA, Bijwaard KE, Heffess CS, Abbondanzo SL, et al. Malignant lymphoma of the thyroid gland: A clinicopathologic study of 108 cases. Am J Surg Pathol 2000;24:623-39.  Back to cited text no. 4
Belal AA, Allam A, Kandil A, El Husseiny G, Khafaga Y, Al Rajhi N, et al. Primary thyroid lymphoma: A retrospective analysis of prognostic factors and treatment outcome for localized intermediate and high grade lymphoma. Am J Clin Oncol 2001;24:299-305.  Back to cited text no. 5
Hyjek E, Isaacson PG. Primary B cell lymphoma of the thyroid and its relationship to Hashimoto's thyroiditis. Hum Pathol 1988;19:1315-26.  Back to cited text no. 6
Thieblemont C, Mayer A, Dumontet C, Barbier Y, Callet-Bauchu E, Felman P, et al. Primary thyroid lymphoma is a heterogeneous disease. J Clin Endocrinol Metab 2002;87:105-11.  Back to cited text no. 7
Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML, et al. Arevised European-American classification of lymphoid neoplasms: A proposal from the international lymphoma study group. Blood 1994;84:1361-92.  Back to cited text no. 8
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al. WHO Classification of tumours of haematopoietic and lymphoid tissues. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. Lyon: IARC Press; 2008. p. 11-2.  Back to cited text no. 9
Wozniak R, Beckwith L, Ratech H, Surks MI. Maltoma of the thyroid in a man with Hashimoto's thyroiditis. J Clin Endocrinol Metab 1999;84:1206-9.  Back to cited text no. 10
Tsang RW, Gospodarowicz MK, Pintilie M, Wells W, Hodgson DC, Sun A, et al. Localized mucosa-associated lymphoid tissue lymphoma treated with radiation therapy has excellent clinical outcome. J Clin Oncol 2003;21:4157-64.  Back to cited text no. 11
Pedersen RK, Pedersen NT. Primary non-Hodgkin's lymphoma of the thyroid gland: A population based study. Histopathology 1996;28:25-32.  Back to cited text no. 12
Cabanillas F. Except from Thyroid Lymphoma. E-Medicine; 2002.  Back to cited text no. 13
Isaacson P, Wright DH. Malignant lymphoma of mucosa-associated lymphoid tissue. A distinctive type of B-cell lymphoma. Cancer 1983;52:1410-6.  Back to cited text no. 14
Isaacson PG, Androulakis-Papachristou A, Diss TC, Pan L, Wright DH. Follicular colonization in thyroid lymphoma. Am J Pathol 1992;141:43-52.  Back to cited text no. 15
Banu Douan GN, Mustafazkan GN, Karamanoulu Z. Primary thyroid lymphoma arising in the setting of Hashimoto's thyroiditis. Turk J Med Sci 2004;34:395-8.  Back to cited text no. 16
Widder S, Pasieka JL. Primary thyroid lymphomas. Curr Treat Options Oncol 2004;5:307-13.  Back to cited text no. 17


  [Figure 1], [Figure 3], [Figure 2], [Figure 4]


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