Figure 1 B: H&E section shows crushing artifactual changes with round
blue cells infiltrating in between the benign glands.
Discussion
Primary lymphoma in the larynx is an extremely rare disease contributing
to less than 1 % of all laryngeal malignancies.(3) Although squamous
cell carcinoma is the most common neoplasm seen in the larynx, it is
necessary to keep the differential diagnosis broad including lymphoma of
the larynx. (4)
MALToma commonly presents in the stomach accounting for 35 % of all
cases but rare occurrences in the larynx have been reported. The
majority of the patients with MALToma present with symptoms in Stage I
and II which depend upon its localization. (1) MALT lymphoma has been
associated with a history of chronic inflammatory disorders which may be
the result of infection, autoimmunity, or unknown other stimuli. (1)
Furthermore, there is a well-established association between H. pylori
and gastric MALToma. (5) However, with MALT lymphoma of the larynx, the
pathophysiological association with H. pylori is not established.
Furthermore, infrequent presentation of MALT lymphoma in the larynx
makes building any kind of such association with H. pylori or other
stimuli challenging. (6) Likewise, there wasn’t any such chronic
inflammatory condition present in our case.
The common symptoms of extranodal laryngeal lymphoma are hoarseness,
dysphagia, pharyngalgia along with B symptoms. (7) Primary laryngeal
lymphoma usually affects the supraglottic area with or without the
involvement of the glottic or subglottic area. (8) In contrast-enhanced
CT, most primary laryngeal lymphomas are usually homogenous with
contrast enhancement. (9) Histologically, the characteristics of MALToma
are similar regardless of the site of their origin. The microscopic
features of marginal zone B cells are small to medium-sized, irregular
nuclei with moderately dispersed chromatin and inconspicuous nucleoli,
similar to those of centrocytes, and relatively abundant, pale
cytoplasm.
However, Immunophenotype is a mandatory investigation for confirming the
diagnosis. The neoplastic cells of MALT lymphoma express B
cell-associated antigens CD20, and CD79, and are usually negative for
CD5, CD10, CD23, and Cyclin D1.(1) As in our case, CD21 staining
indicates expanded meshworks of follicular dendritic cells corresponding
to colonized follicles. Like other low-grade lymphoproliferative
disorders which stain positive for BCL2, here also it demonstrates low
grade contrary to high-grade lymphomas. (10)
MALToma is usually indolent with recurrences that can occur after many
years in other extranodal sites. (1) It has a desirable prognosis of a
5-Year relative survival rate > 80% but varies depending
on the site of origin. (11) Rarity of these cases has made it difficult
to establish a consensus on their prognostic factors and choice of
treatments. The international guidelines recommend radiotherapy to be
the preferred choice for localized non-gastric MALT lymphomas. A
moderate dose (25-30 Gy) of radiation has shown a high rate of local
control even in patients with local recurrences. Contrary to gastric
MALT lymphomas, where antibiotic therapy to eradicate H. Pylori is the
wellestablished standard of care, the role of antibiotic therapy is
unclear and not recommended. Although the choice of treatment for severe
or recurrent cases has not been certain, chemotherapy with chlorambucil
in combination with rituximab has shown better results.(12)
Conclusion:
Although extranodal marginal zone lymphoma or MALToma of the larynx is a
rare disease without any specific signs and symptoms, it should be
considered in the differential diagnosis of a mass in the larynx. It has
been difficult to generate a consensus regarding its management due to
few cases, more case reports and research are necessary to better
understand the disease and establish a treatment plan.
Acknowledgement: None
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