International Journal of Surgery Case Reports 114 (2024) 109126

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International Journal of Surgery Case Reports

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Case report

Primary thyroid MALT lymphoma with a probable brain secondary in a

male patient: A case report
B.M. Munasinghe a, c, *, C.T. Karunatileke b, J. Prashanthan c, N.D. Ranathunga d
a
Department of Anaesthesiology and Intensive Care, Kent and Canterbury Hospital, Canterbury CT1 3NG, UK
b
Department of Surgery, District General Hospital, Mannar, Sri Lanka
c
Department of Anaesthesiology and Intensive Care, District General Hospital, Mannar, Sri Lanka
d
Department of Histopathology, District General Hospital, Mannar, Sri Lanka

A R T I C L E I N F O A B S T R A C T

Keywords: Introduction and importance: Primary Mucosa-associated lymphoid tissue (MALT) lymphoma of the thyroid is a
MALT lymphoma rare tumor.
Thyroid Presentation of case: A previously well male in his 50s presented to our institution with difficulty in breathing and
Hashimoto thyroiditis
sleep apnea. He was diagnosed with a large retrosternal multinodular goiter with level 2 unilateral cervical
Prognosis
lymphadenopathy. Fine needle aspiration cytology of the thyroid revealed chronic thyroiditis and the enlarged
lymph node cytology was inconclusive. He underwent total thyroidectomy and level VI bilateral cervical lymph
node clearance. The histology revealed an extra-nodal marginal zone lymphoma of MALT. A whole-body CT scan
did not demonstrate any other primary site. The patient received 4 cycles of local radiotherapy. Subsequently, he
was diagnosed with a brain tumor not amenable to surgical interventions following persistent headaches. He died
shortly after due to complications of probable cerebral metastasis.
Case discussion: MALT lymphomas of the thyroid carry a good prognosis; however, no universal guidance exists
regarding the optimal therapy and follow-up.
Conclusion: This case report highlights the importance of early diagnosis, identification of poor prognostic fac­
tors, and patient-tailored therapy and follow-up.

1. Introduction 2. Case presentation

Extra-nodal marginal zone lymphoma of mucosa-associated
lymphoid tissue (MALT) in the thyroid gland is a rare, slow-growing
malignant tumor accounting for 1–2 % of extranodal lymphomas [1].
Thyroid MALT lymphomas are B-cell derived and predominantly occur
in the background of chronic inflammation, mainly Hashimoto
thyroiditis [2]. Most patients remain asymptomatic and surgery is
warranted when pressure symptoms occur or the histopathology is
inconclusive [3]. Treatment includes surgery in the form of thyroidec­
tomy, neck dissection, and adjuvant radiotherapy or chemotherapy. The
prognosis is favorable compared to diffuse large B cell lymphoma, the
commonest lymphoma-variant of the thyroid [2,4]. We present a case of
a MALT lymphoma of the thyroid in a middle-aged male who succumbed
following a probable brain secondary. The case is reported in line with
the SCARE criteria [5].
A previously well South Asian male in his 50s presented to the sur­
gical clinic of our institution, a District General Hospital, with an ante­
rior neck swelling for 5 years with a recent rapid enlargement and
associated difficulty in breathing, sleep apnea, and hoarseness of voice.
He was euthyroid clinically. There was no history of radiation exposure
and no family history of thyroid disorders. A large multinodular goiter
(World Health Organization grade 4) with retrosternal extension was
diagnosed by clinical examination. His body mass index was 30 kg m− 2.
Airway assessment revealed a mallampati grade of 4 which indicated
difficulty.
3. Investigations
X-ray neck showed mild tracheal compression and mild tracheal
deviation. Ultrasound of the neck confirmed a large multinodular goiter

* Corresponding author at: Department of Anaesthesiology and Intensive Care, Kent and Canterbury Hospital, Canterbury CT1 3NG, UK.
E-mail address: b.munasinghe@nhs.net (B.M. Munasinghe).

https://doi.org/10.1016/j.ijscr.2023.109126
Received 26 October 2023; Received in revised form 27 November 2023; Accepted 3 December 2023
Available online 7 December 2023
2210-2612/© 2023 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY license
(http://creativecommons.org/licenses/by/4.0/).
B.M. Munasinghe et al.
with features of thyroiditis and areas of calcification. A 2 cm retrosternal
extension was also noted with level 2 cervical lymphadenopathy on the
left side. The thyroid profile was in the normal range. Fine needle
aspiration cytology of the thyroid gland suggested chronic thyroiditis
and was inconclusive for cervical lymph nodes. A computed tomography
of the neck was not performed due to the unavailability in our center and
the long waiting list for a patient with worsening obstructive symptoms.
An early surgery was planned. Preoperative vocal cord assessment was
normal.
Histology revealed diffuse infiltration of a polymorphic population
of small to medium size lymphoid cells. Some cells were plasmacytoid.
Lymphoepithelial lesions were evident (Fig. 1). Mitoses were sparse.
There were no diffuse areas of large cells to suggest transformation into
Fig. 1. Lymphoepithelial lesions H&E x40.
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International Journal of Surgery Case Reports 114 (2024) 109126
diffuse large B cell lymphoma. A limited IHC panel was performed due to
financial restraints. Neoplastic lymphoid cells were positive for CD20
and negative for CD3. confirming the B cell origin. Ki67 index was
around 5 %, confirming the low-grade nature of the lymphoma.
Therefore, immunohistochemically this was confirmed as a low-grade B
cell non-Hodgkin lymphoma, together with morphology that arrived at
MALT lymphoma diagnosis. The rest of the thyroid showed chronic
autoimmune thyroiditis (Fig. 2). The whole-body CT study was negative
for any other tumor sites. The CT head performed later for the persistent
headaches revealed a new metastatic deposit.
B.M. Munasinghe et al.
Fig. 2. Back ground thyroiditis H&E x10.
4. Differential diagnosis
MALT lymphoma of the thyroid in the background of chronic auto­
immune thyroiditis with subsequent probable metastasis into the brain.
5. Treatment
For the initial thyroidectomy, an awake fiberoptic intubation was
performed. A wide collar incision was made to gain adequate exposure.
During the surgery, both recurrent laryngeal nerves were preserved and
a total thyroidectomy was performed. The patient was admitted to the
intensive treatment unit and was ventilated to allow the airway oedema
to settle. He was extubated on day 02 and discharged home with oral
thyroxine and calcium replacement with 1 alpha cholecalciferol.
He was referred to the oncologist following the diagnosis of MALT
lymphoma. Localized radiotherapy was commenced in the absence of
distant tumor sites. At the end of the fourth cycle of radiotherapy, he was
further investigated for new onset, severe headache with pupil
inequality. The metastatic deposit of the brain was not amenable to
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International Journal of Surgery Case Reports 114 (2024) 109126
surgical correction.
6. Outcome and follow-up
He died shortly after complications of tumor growth in the brain.
7. Discussion
MALT lymphoma of the thyroid is a type of primary thyroid lym­
phoma. These occur more commonly in females (male: female 1:4) of old
age (mean age of diagnosis 65 years). [6–8]. Around 90 % of patients
have underlying Hashimoto thyroiditis [9]. The studies have shown a
40- to 80-fold increase in the risk of MALT lymphoma in the background
of Hashimoto thyroiditis [10]. Diagnosis of MALT lymphoma of the
thyroid using cytology and histology is challenging as there can be
simultaneous reactive and neoplastic tissue changes [3,11]. Clinically
the classical ‘B’ symptoms seen in other lymphomas are also uncommon
(around 10 %) in MALT lymphomas of the thyroid [12,13]. A rapid, non-
tender enlargement of the thyroid can be the presenting symptom while
B.M. Munasinghe et al.
around 30 % may have compressive symptoms due to mass effect
[14,15].
Diagnosis of MALT lymphoma of the thyroid using fine needle
aspiration cytology carries a low sensitivity specially in early disease
[2,15] and may illustrate chronic thyroiditis in the case of associated
Hashimoto thyroiditis. Most recent systematic reviews point out the low
sensitivity and specificity of ultrasound in making accurate diagnosis
[2]. Definite diagnosis is thus made by histopathological analysis of
surgical resection specimens [16]. Further immunohistochemistry
studies that yield CD20, Bcl-2, immunoglobulin light chain, and absence
of CD5, CD10, and CD23 aid the diagnosis [17]. Co-existing other types
of thyroid malignancies (follicular, papillary, and anaplastic) may also
be diagnosed using immunohistochemistry. The unavailability of such
sophisticated tests in developing countries poses an obstacle and histo­
logical analysis following surgical resection of the thyroid for symp­
tomatic goiters may provide an incidental diagnosis [18].
The staging of thyroid MALT lymphomas is still not definite as stage
1E (thyroid only) and 11E (thyroid and regional lymph nodes) cannot be
differentiated, however, modified classification systems have been
studied [19]. Still, no universal guidance exists with regard to treatment.
Open biopsy and isolated-site radiotherapy (OB-ISRT), curative re­
sections, and adjuvant radiotherapy or chemotherapy are all practiced
depending on the extent of the disease.
MALT lymphoma of the thyroid has an excellent prognosis (5-year
overall survival of 94 % and event-free survival rate of 92 %) [20].
Despite the rarity of both the disease and relapse rates, extended follow-
up is recommended by some authors [21,22]. MALT lymphoma usually
has an indolent course, and it is quite unusual to metastasize to brain
early. The brain tumor in our patient is most likely a secondary from the
thyroid tumor considering the temporal association of the events;
however, this could not be proven definitely as no histology of the brain
lesion was performed.
Consent
Written informed consent was obtained from the next-of-kin of the
patient for the publication of this case report and accompanying images.
A copy of the written consent is available for review by the Editor-in-
Chief of this journal on request.
Provenance and peer review
Not commissioned, externally peer-reviewed.
Ethical approval
Our institution does not require ethical approval for reporting indi­
vidual cases or case series.
Funding
This research did not receive any specific grant from funding
agencies in the public, commercial, or not-for-profit sectors.
Author contribution
Clinical management, Concept, consent, literature review, drafting
of the initial and final manuscript - BM, CK. JP, ND.
Approval of the final manuscript- All authors.
Guarantor
B.M. Munasinghe.
4
International Journal of Surgery Case Reports 114 (2024) 109126
Research registration number
Not applicable.
Conflict of interest statement
None declared.
Acknowledgment
The authors would like to acknowledge the inputs from Dr. N.D.
Ranathunge (Consultant Pathologist, District General Hospital, Mannar,
Sri Lanka) and all the clinicians involved in the management of the
patient.
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