|Year : 2017 | Volume
| Issue : 2 | Page : 40-42
A case of primary mucosa-associated lymphoid tissue lymphoma of the larynx
Sachin Gandhi, Shashank Gupta, Sujit Joshi, Suvojit Ghosh
Voice Clinic, Deenanath Mangeshkar Hospital, Pune, Maharashtra, India
|Date of Web Publication||26-Sep-2018|
Dr. Shashank Gupta
A1/7 Meenal Gardens, Ernadwane, Pune - 411 004, Maharashtra
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Mucosa- associated Lymphoid Tissue (MALT) Lymphoma is a Non-Hodgkin Lymphoma. This marginal zone lymphoma is typically a low-grade neoplasm. Primary Laryngeal MALT Lymphoma is an extremely rare entity. No case has been reported from India till now. We present a case of Laryngeal MALT Lymphoma in a 49 year old lady with complain of progressive hoarseness of voice since 6 months and history of orbital pseudo-tumour. MALT Lymphoma was diagnosed after biopsy and managed with 6 cycles of R-Benda Chemotherapy. The patient has had uneventful follow-up for past 3 years. Primary MALT lymphoma is a rare but curable disease and clinicians should keep it in mind while forming differential diagnosis for a suspicious mass during laryngeal evaluation.
Keywords: Larynx, lymphoma, MALToma, R-Benda
|How to cite this article:|
Gandhi S, Gupta S, Joshi S, Ghosh S. A case of primary mucosa-associated lymphoid tissue lymphoma of the larynx. J Laryngol Voice 2017;7:40-2
| Introduction|| |
Mucosa-associated lymphoid tissue (MALT) Lymphoma, also called extranodal marginal zone B-cell lymphoma, is a Non-Hodgkin lymphoma (NHL). The gastrointestinal tract is the most frequent site of extranodal lymphoma, and the stomach is involved in up to two-thirds of these cases. About 30%–45% of all extranodal lymphomas are detected in the stomach.
The marginal zone lymphoma – usually named MALT lymphoma – is typically a low-grade neoplasia, characterized by a dense lymphoid infiltrate mainly composed of small-size lymphocytes that invade and destroy gastric glands, configuring the so-called “lymphoepithelial lesion” which is pathognomonic of lymphoma.
Primary Laryngeal MALToma is an extremely rare entity. Since the first description by Diebold et al. in 1990, we could find only 45 reported cases of Laryngeal MALToma. No case has been reported from India.
| Case Report|| |
A 49-year-old female presented in July 2013 with complain of gradually progressive hoarseness of voice for the past 6 months. The patient had no history of smoking, alcohol or tobacco intake, vocal abuse, fever, weight loss, or night sweats. There was no history of gastroesophageal reflux. She had no comorbidities. She had been treated previously in 2005 for Orbital pseudo-tumor, a nonspecific idiopathic inflammatory condition, with systemic corticosteroids.
Her general physical examination was within normal limits. Videolaryngostroboscopy examination showed the presence of left false vocal cord bulge with bilateral vocal cords mobile [Figure 1]. The mucosal covering of the false cord appeared normal. The true vocal cords appeared normal. They were mobile, free edge epithelium appeared normal, and the mucosa over the arytenoids was normal. The patient was then taken up for microlaryngoscopic examination and biopsy. Biopsy revealed nodular proliferation of small, monotonous atypical lymphoid cells with high N:C ratio and scanty cytoplasm in the subepithelial layer. Few collections of monocytoid lymphoid cells were present with occasional areas showing follicle formation and many congested blood vessels. At places, small sized neoplastic lymphoid cells were seen infiltrating the submucosal glands (lymphoepithelial lesions). This was morphologically consistent with MALT lymphoma [Figure 2].
|Figure 1: Videolaryngoscopy findings – left false vocal cord smooth bulge|
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|Figure 2: Submucosal nodular growth rich in small sized neoplastic lymphoid cells at places infiltrating the submucosal gland (lymphoepithelial lesion). (a) - ×10, (b) - ×200|
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The tumor cells were strongly positive for CD20 and Bcl-2 protein while negative for CD3, CD5, CD10, and cyclin-D1. MiB-1 Index was 9%. CD21 and CD35 were not done. This suggested the diagnosis of low-grade NHL – marginal zone type [Figure 3].
|Figure 3: Neoplastic lymphoid cells showing diffuse and strong positivity for (a) CD20 and (b) Bcl-2 protein|
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Upper gastrointestinal endoscopy was done and found to be normal. Whole body positron emission tomography (PET) computed tomography scan showed localized increased uptake of radiotracer in the laryngeal region only without any other region of significant uptake. Therefore, the tumor was staged as IE on the Ann Arbor Staging system.
With the diagnosis of low-grade Primary Laryngeal MALT Lymphoma patient underwent six cycles of R-Benda (Bendamustine and Rituximab) chemotherapy. Six cycles of chemotherapy were administered between August 2013 and February 2014. Complete remission of disease was noted. Follow-up whole-body PET scan showed no area of increased uptake. The patient has been under follow-up for more than 3 years without any evidence of recurrence [Figure 4] and [Figure 5].
|Figure 4: Follow-up videolaryngoscopy pictures from (a). 2014 (b). 2015 after patient completed chemotherapy|
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| Discussion|| |
Primary MALT lymphoma of the Larynx is an extremely rare disease. It occurs most frequently in middle age (around 50 years of age) with no gender differences, and symptoms vary depending on location and size. Literature pegs the incidence in different regions of larynx as supraglottic - 77.3%, subglottic - 18.2%, and glottis - <5%.,
While the exact etiology remains unknown, MALT lymphoma is strongly related to a background of chronic inflammatory disease or autoimmune disease. It has found to be associated with Sjögren's syndrome, systemic lupus erythematosus, Hashimoto thyroiditis, and gastritis associated with Helicobacter pylori.,
Cytogenetic studies have shown that about 50% of MALT lymphomas have translocation (11; 18) (q21, q21). This leads to positive immunohistological studies for CD20, CD21, CD35, and immunoglobulin M; and negative for CD5, CD10, CD23, and Cyclin-D1.
There is no specific treatment for MALT Lymphoma. It has been treated using various modalities ranging from surgery, antibiotics, chemotherapy, radiotherapy, and a combination of these.,,, All treatment modalities have had good success rates. Recently, with the shift of treatment strategy to more conservative approach, Rituximab-Bendamustine (R-Benda) chemotherapy has been proposed as the first line treatment for NHL. Chemotherapy provides a conservative, effective, and acceptable cure for NHL and has been used successfully for NHL ranging from low-grade to aggressive tumors. R-Benda has shown to provide similar cure rates but better side-effect profile than R-CHOP chemotherapy regimens.
Our patient did not have a history of reflux. She had a prior episode of an idiopathic inflammatory condition. The association between MALT lymphoma and Orbital pseudotumor is yet to be studied although more such instances need to be identified before such a study can be taken up. Our patient responded favorably to R-Benda chemotherapy regimen, undergoing complete remission. She has had uneventful follow-up for more than 3 years.
Primary MALT lymphoma is a rare but curable disease and clinicians should keep it in mind while forming differential diagnosis for a suspicious mass during laryngeal evaluation.
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
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]