Primary thyroid lymphoma

Amarendra Amar; Seema Singhal; G Sharat Chandra; Harsh Dua

doi:10.4103/am.am_1_19

CASE REPORT

Year : 2019 | Volume : 16 | Issue : 1 | Page : 58-60

Primary thyroid lymphoma

Amarendra Amar¹, Seema Singhal², G Sharat Chandra¹, Harsh Dua¹
¹ Department of Medical Oncology, Indraprastha Apollo Hospital, New Delhi, India
² Department of Pathology, Indraprastha Apollo Hospital, New Delhi, India

Date of Web Publication

11-Mar-2019

Correspondence Address:
Amarendra Amar
Department of Medical Oncology, Indraprastha Apollo Hospital, New Delhi - 110 076
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/am.am_1_19

Abstract

Primary thyroid lymphoma is a rare neoplasm consisting of 1%–5% of all thyroid neoplasms. These are predominantly B-cell in origin. It is curable without surgery. The literature search shows it to be presenting as rapidly developing thyroid mass. It has been shown to be associated with autoimmune thyroiditis. The rarity of this condition mandates it to be differentiated from other rapidly developing thyroid malignancies to recognize it early as it is curable. The treatment consists of chemotherapy, radiation, or both combined with excellent results.

Keywords: Anaplastic carcinoma, autoimmune thyroiditis, lymphoma, thyroid

How to cite this article:
Amar A, Singhal S, Chandra G S, Dua H. Primary thyroid lymphoma. Apollo Med 2019;16:58-60

How to cite this URL:
Amar A, Singhal S, Chandra G S, Dua H. Primary thyroid lymphoma. Apollo Med [serial online] 2019 [cited 2023 Feb 5];16:58-60. Available from: https://apollomedicine.org/text.asp?2019/16/1/58/253862

Introduction

Primary thyroid lymphomas (PTLs) constitute 1%–5% of thyroid malignancies.^[1] Most PTLs are B-cell non-Hodgkin's lymphomas (NHLs). A high percentage of PTLs affects patients with a long-standing Hashimoto's thyroiditis (HT); therefore, PTL pathogenesis is probably related to chronic inflammatory stimulation.^[2],[3] Most common thyroid lymphomas are diffuse, large, B-cell lymphoma (DLBCL) and mucosa-associated lymphoid tissue (MALT) lymphoma.^[4] The other differential diagnosis to be kept in mind, when dealing with DLBCL, is anaplastic thyroid carcinoma.^[5]

Here, we present a case of rapidly developing symptomatic thyroid mass in a 69-year-old female who was earlier thought to be having thyroid carcinoma which, however, after workup, was diagnosed as lymphoma along with a very high value of markers related to autoimmune thyroiditis.

Case Report

A 69-year-old female, a known diabetic with a long-standing history of hypothyroidism, presented with a history of neck swelling for 1 month with a rapid progression for 10 days associated with hoarseness and progressive difficulty in swallowing. Breathlessness worsened in the last few days, especially in lying down position. She was earlier investigated at another hospital with a magnetic resonance imaging scan of the neck which showed space-occupying lesion of the isthmus and the left lobe of the thyroid with airway and laryngeal compression. A fine-needle aspiration cytology (FNAC) done outside was suggestive of lymph proliferative disorder; however, it was not substantiated further.

Clinical examination showed a neck mass moving with deglutition with trachea shifted toward the right. She had to be tracheostomized because of rapidly developing breathlessness. An ultrasonogram of the neck, done after clinical stabilization, showed a heterogeneous mass lesion with increased vascularity in the region of the thyroid gland; however, there were no cervical lymph nodes [Figure 1].

Figure 1: Ultrasonogram of the neck shows a heterogeneous lesion of the thyroid with increased vascularity

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A biopsy done from the mass showed the presence of diffuse infiltration by tumor cells having scant cytoplasm and large, round nucleus with opened up chromatin and prominent nucleoli. Frequent atypical mitotic figures and foci of necrosis were seen in a background of mixed inflammatory cells [Figure 2]a. Immunohistochemistry (IHC) showed diffuse intense positivity for CD45 [Figure 2]b and CD20 [Figure 2]c. Ki67 was approximately 30%–40% [Figure 2]d.

Figure 2: (a) Biopsy from the thyroid shows intense diffuse infiltration by the tumor cells. (b) Immunohistochemistry marker for CD45 shows intense diffuse staining. (c) Immunohistochemistry staining of CD20 shows intense diffuse staining. (d) Immunohistochemistry staining for Ki67

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IHC was negative for thyroid transcription factor-1. A few reactive T-cells were highlighted by CD3. Tumor cells were negative for Epstein–Barr virus immunostaining. Thus, biopsy with IHC confirmed it to be NHL, B-cell type.

A positron emission tomography–computed tomography scan was done for staging purpose, and it showed a large, enhancing, metabolically active soft-tissue mass of 6.2 cm × 8.6 cm × 9.7 cm size in the left side of the neck, inseparable and possibly arising from the thyroid. The mass extended superiorly till the left pyriform fossa and inferiorly till suprasternal notch. It produced mass effect on the tracheal lumen with its deviation toward the right. There was a small, metabolically active left retropharyngeal node. There was no evidence of any lesion elsewhere in the body [Figure 3] and [Figure 4].

Figure 3: Contrast computed tomography image of the neck shows a thyroid mass with its extension and mass effect

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Figure 4: Positron emission tomography image of the patient shows a thyroid lesion with no lesion elsewhere in the body

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Her blood counts, kidney function, and liver function tests were within normal limits. Viral markers for hepatitis and human immunodeficiency virus were negative. Her blood sugar level was controlled.

Thyroid function was normal. However, antithyroid peroxidase antibody (anti-TPO) and thyroglobulin antibody (TGA) were very high, suggesting an antecedent autoimmune thyroiditis before the development of lymphoma.

Considering the nature of her disease, the patient was started on prephase intravenous steroids and planned for rituximab, cyclophosphamide, adriamycin, vincristine, and prednisolone (R-CHOP) regimen. She showed clinical improvement after the first cycle of treatment with reduction in the size of the neck mass and improvement in the general condition. She was given a written treatment protocol to pursue treatment at another hospital close to her home due to monetary conditions. She continues to show improvement after the second cycle of treatment.

Discussion

PTL is most commonly observed in middle-to-older-aged females.^[6] The main presenting findings are a mass in the thyroid gland or diffuse enlargement that causes symptoms related to compression, such as hoarseness, dysphagia, and dyspnea, the same presentation as observed in our case. The thyroid gland contains no native lymphoid tissue although, in autoimmune thyroid disease, notably in HT, the thyroid gland accumulates lymphoid cells.^[7] The relative risk of developing a malignant thyroid lymphoma has been estimated to be 40–80 times greater in patients with HT than in the general population, with lymphoma typically manifesting 20–30 years after the diagnosis of thyroiditis.^[8] This evolution from HT to lymphoma (typically a MALT lymphoma) occurs in 0.5% of cases and is generally characterized by an indolent course. In some cases, however, transformation from MALT lymphoma to aggressive lymphoma may occur, with a poor prognosis.^[9] Our patient was never subjected to any investigation for HT in the past. However, a long-standing history of hypothyroidism and highly elevated anti-TPO and TGA levels in our patient point toward underlying autoimmune thyroiditis. Moreover, rapid progression of the disease suggests transformation to NHL from an indolent lymphoma in our patient. The most common B-cell lymphoma is the diffuse large cell accounting for 50%–70% of all lymphomas, as was seen in the series by Onal et al.^[10] Pure MALT lymphomas of the thyroid make up 23%–30% of primary lymphomas.^[11],[12]

Treatment with radiotherapy (RT) alone is typically reserved for cases with an indolent histology, whereas combined modality treatment (CMT) with chemotherapy and RT is preferred for aggressive lymphomas.^[13],[14] Due to its rarity, very few studies have investigated the optimal treatment of PTL, and many reviews are based on studies including small numbers of patients. The chemotherapy consists of R-CHOP regimen and radiation consists of involved field RT. CMT improves all treatment-related outcomes in PTL.

Conclusions

PTL is a rare thyroid malignancy which can mimic anaplastic thyroid carcinoma in its presentation. FNAC may not yield the accurate diagnosis, necessitating a biopsy with IHC in such cases. Keeping the possibility of NHL in a rapidly progressing thyroid mass is important because it may lead to timely cure of the patient.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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