PTW
  • Users Online: 126
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 

 
CASE REPORT
Ahead of print publication  

A rare case of mantle cell lymphoma of nasopharynx and larynx with synchronous presentation treated with volumetric modulated arc technique radiotherapy


 Department of Radiotherapy, Batra Hospital and Medical Research Centre, New Delhi, India

Date of Submission02-Feb-2022
Date of Decision14-Mar-2022
Date of Acceptance17-Mar-2022
Date of Web Publication24-Aug-2022

Correspondence Address:
Sujata Sarkar,
Department of Radiotherapy, Batra Hospital and Medical Research Centre, New Delhi
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jrcr.jrcr_11_22

  Abstract 

Mantle cell lymphoma (MCL) or mature peripheral B-cell lymphoid neoplasm is a type of non-Hodgkin's lymphoma. It is more common in elderly males. It is very rare, accounting for less than 1% of head and neck malignancies. MCL is usually positive for CD20, CD5, CD43, cyclin D1 and negative for CD10, CD23, and BCL6. It is a very aggressive neoplasm, and hence, patients often present in advanced stage. Due to its rare incidence and short clinical course, there are limited data on standard treatment protocols. Even with the present treatment protocols, only 30% of patients achieve complete response. Here, we present a case of synchronous presentation of MCL of the nasopharynx and larynx in a 69-year-old male. We aim to discuss the investigations and treatment done with volumetric modulated arc technique radiotherapy that led to complete response.

Keywords: Larynx, Mantle cell lymphoma, nasopharynx, synchronous, volumetric modulated arc technique radiotherapy



How to cite this URL:
Sarkar S, Bashir I, Yotham RR, Sharma R. A rare case of mantle cell lymphoma of nasopharynx and larynx with synchronous presentation treated with volumetric modulated arc technique radiotherapy. J Radiat Cancer Res [Epub ahead of print] [cited 2022 Dec 4]. Available from: https://www.journalrcr.org/preprintarticle.asp?id=354436


  Introduction Top


Non-Hodgkin's lymphomas (NHLs) are a heterogeneous group of lymphoid malignancies that originate from B cells, T cells, or natural killer cells. There are 70 different types of NHL as per the 2016 WHO Classification.[1] Among them, Mantle cell lymphoma (MCL) is one of the rarest varieties, comprising only 6% of NHL.[2] It is more common in males than females (M: F is 4:1 as in 2015). Median age of presentation is 63 years. 80% of patients have Stage III or Stage IV disease on presentation due to its aggressive nature.[2] The most common location of NHL is gastrointestinal tract followed by head and neck (mostly Waldeyer's ring).[2] Primary nasopharyngeal lymphoma is seen in only 8% of NHL of head and neck, and among them, less than 1% are MCL. Lymphoma of the larynx is less than 1% of laryngeal malignancies; MCL is even more rare. To the best of our knowledge, this is the second reported case of synchronous presentation of MCL of the supraglottic larynx (SGL) and nasopharynx, the first being reported in 2019.[3] Three-year survival of MCL patients is only 5%–10%.[4],[5] The aggressive and heterogeneous behavior of tumor makes it difficult to establish a treatment of choice,[2],[5] and it is still incurable.[6] Treatment includes either chemotherapy alone or with radiotherapy.[4] Only 30% of patients achieve complete response following treatment.[6]

Here, we focused on the investigations and treatment done with volumetric modulated arc technique (VMAT) radiotherapy explaining the critical structures taken care of, contouring, planning, and doses achieved. With this technique, we achieved a complete response in our patient with minimal side effects.


  Case Report Top


A 69-year-old male from Congo presented to the outpatient department in October 2019 with hoarseness of voice and dysphagia to solids for the past 6 months. He had no comorbidities. Fibreoptic laryngoscopy showed a large mass of 4 cm over epiglottis extending into lumen. He had 1 cm × 1 cm firm, mobile swelling in the left side of the upper neck. Gastroscopy showed a large mass involving epiglottis. Positron emission tomography-computed tomography (PET-CT) showed FDG avid lobular mass involving epiglottis, preglottic space, extending superiorly up to base of tongue and bilateral tonsillar fossa, measuring 4.2 cm × 4.7 cm × 4.7 cm (SUVmax 19.4). Another FDG avid soft tissue thickening in the right fossa of Rosenmuller (SUVmax 15.8). FDG avid left level II, III, IV lymph nodes, largest in level II 13 mm × 8 mm (SUVmax 12.1) [Figure 1].
Figure 1: Positron emission tomography-computed tomography image showing FDG avidity in the nasopharynx and larynx

Click here to view


Biopsy from nasopharynx and epiglottis showed atypical small lymphoid cells arranged in diffuse sheets and focal nodules, suggesting lymphoproliferative disorder. Immunohistochemistry (IHC) was positive for CD20, CD5, CD23, Bcl-2, cyclin D1, and CD43, and negative for CD3, CD10, and MUM-1; Ki-67 was 50%. It was confirmed to be MCL of the nasopharynx and SGL, stage IIE.

The patient was planned for involved site radiotherapy (ISRT) by VMAT to ensure maximum coverage and minimum toxicity of nearby organs at risk (OARs) such as both parotid glands, cochlea, spinal cord, eye, lens, and optic nerves. Target area was taken as gross tumor volume (GTV) in the nasopharynx and larynx and gross lymph nodes, with bilateral level Ia–IV cervical and retropharyngeal lymph nodes with 1 cm margin on GTV to form clinical target volume (CTV) which includes areas at risk of microscopic disease spread, 5 mm margin on CTV to form planning target volume (PTV) to account for uncertainties in position during daily treatment [Figure 2]. All OARs' dose constraints were within limits [Supplementary Table 1].[Additional file 1] The patient was treated in Varian Truebeam Machine with Eclipse Planning System, 6 MV energy by VMAT radiotherapy. PTV was covered by 99.8% of prescribed dose [Figure 3]a and [Figure 3]b. He received 36 Gy/18fractions (Fr) at 2 Gy/Fr, 1 Fr/day, and 5 Fr/week. The patient tolerated the radiation well with no remarkable complications. The patient had CTCAE v4.0 (Common Terminology Criteria for Adverse Events, published 2009, updated June 14, 2010)[7] grade 1 neutropenia, grade 1 fatigue, grade 1 dermatitis, grade 2 dysphagia, and grade 1 mucositis. PET-CT after 3 months showed complete response. After that, he was lost to follow-up.
Figure 2: Contouring images with GTV (blue), CTV (green), and PTV (red). GTV: Gross tumor volume, CTV: Clinical target volume, PTV: Planning target volume

Click here to view
Figure 3: (a) Dose distribution from treatment planning system showing the target area and planning target volume covered by 95% of prescribed dose. (b) Dose–volume histogram of target area (GTV, CTV, and PTV) and OARs. GTV: Gross tumor volume, CTV: Clinical target volume, PTV: Planning target volume, OARs: Organs at risk

Click here to view



  Discussion Top


NHL accounts for less than 5% of oral cavity malignancies. MCL is one of the rarest and most aggressive subtypes of NHL. Extranodal NHL is usually seen in two subgroups of NHL, diffuse large B-cell lymphoma and extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue. Both nodal and extranodal presentations of MCL in the head and neck are very rare. Diagnosis of MCL requires IHC confirmation. MCL is usually positive for CD20, CD5, CD43, cyclin D1, as well as immunoglobulin IgM and/or IgD. SOX11 is exclusively overexpressed in MCL, including the cyclin D1-negative cases; MCL is negative for CD10, CD23, and BCL6.[4] Cyclin D1 overexpression leads to dysregulation of G1/S phase transition of cell cycle.[6] [Supplementary Table 2][Additional file 2] explains the IHC markers for different NHL.[8]

MCL has aggressive clinical course and very poor prognosis.[2],[4] The MCL International Prognostic Index classifies MCL into low-, intermediate-, and high-risk groups.[2] However, irrespective of the risk category, 3-year survival of MCL patients is only 5%–10%.[4],[6] The aggressive and heterogeneous behavior of tumor makes it difficult to establish a treatment of choice,[2],[6] and it is still incurable.[5] Treatment includes either chemotherapy alone or with radiotherapy.[4] Only 30% of patients achieve complete response following treatment.[6]

A British Columbia Cancer Centre Study indicated that stage I and II patients showed significant improvement with localized radiotherapy or chemoradiation.[9] In our case, as it is stage IIE, we treated with ISRT to the involved site with bilateral cervical nodal chain. VMAT radiotherapy ensured proper coverage of target with minimum toxicity to surrounding critical structures, i.e., OARs.


  Conclusion Top


MCL is a very rare and aggressive tumor. This is the second reported case of synchronous presentation of MCL of the nasopharynx and larynx, to the best of our knowledge. There is no standard treatment for patients with synchronous presentation of MCL in head and neck sites due to its rare incidence and aggressive course. Radiotherapy by newer techniques such as VMAT shows excellent response with minimal side effects, as in our case. It requires more case studies to improve the outcome for such patients.

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.

 
  References Top

1.
Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016;127:2375-90.  Back to cited text no. 1
    
2.
Skarbnik AP, Goy AH. Mantle cell lymphoma: State of the art. Clin Adv Hematol Oncol 2015;13:44-55.  Back to cited text no. 2
    
3.
Kumbul YC, Tuz M, Bircan S, Yasan H, Sivrice ME, Akin V. Synchronous Mantle cell lymphoma of larynx and nasopharynx. Turk J Geriatr 2019;22. [doi: 10.31086/tjgeri. 2020.129].  Back to cited text no. 3
    
4.
Guggisberg K, Jordan RC. Mantle cell lymphoma of the oral cavity: Case series and comprehensive review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;109:98-104.  Back to cited text no. 4
    
5.
Lenz G, Dreyling M, Hiddemann W. Mantle cell lymphoma: Established therapeutic options and future directions. Ann Hematol 2004;83:71-7.  Back to cited text no. 5
    
6.
Chen Y, Wang M, Romaguera J. Current regimens and novel agents for Mantle cell lymphoma. Br J Haematol 2014;167:3-18.  Back to cited text no. 6
    
7.
Common Terminology Criteria for Adverse Events (CTCAE) v. 4.0 Published 2009. Available from: http://evs.nci.nih.gov/ftp1/CTCAE/CTCAE_4.03_2010-06-14_QuickReference_5x7.pdf. [Last updated on 2010 Jun 14].  Back to cited text no. 7
    
8.
Patel JN, Gupta S, Faujdar M, Patel NP. The nodal non-Hodgkin's lymphoma: Histomorphological study with special emphasis on immunomarker profile of nodal NHL. Ann Pathol Lab Med 2019;6. [doi: 10.21276/APALM.2301].  Back to cited text no. 8
    
9.
Leitch HA, Gascoyne RD, Chhanabhai M, Voss NJ, Klasa R, Connors JM. Limited-stage Mantle-cell lymphoma. Ann Oncol 2003;14:1555-61.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

 
Top
 
 
  Search
 
     Search Pubmed for
 
    -  Sarkar S
    -  Bashir I
    -  Yotham RR
    -  Sharma R
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
Abstract
Introduction
Case Report
Discussion
Conclusion
References
Article Figures

 Article Access Statistics
    Viewed254    
    PDF Downloaded8    

Recommend this journal