<%server.execute "isdev.asp"%> Rare clinical presentation of diffuse large B-cell lymphoma as otitis media and facial palsy Siddiahgari SR, Yerukula P, Lingappa L, Moodahadu LS - J Pediatr Neurosci
home : about us : ahead of print : current issue : archives search instructions : subscriptionLogin 
Users online: 510      Small font sizeDefault font sizeIncrease font size Print this page Email this page


 
  Table of Contents    
CASE REPORT
Year : 2016  |  Volume : 11  |  Issue : 1  |  Page : 58-60
 

Rare clinical presentation of diffuse large B-cell lymphoma as otitis media and facial palsy


1 Department of Pediatric Hematooncology, Rainbow Children×s Tertiary Care Centre, Hyderabad, Telangana, India
2 Department of Pediatrics, Rainbow Children×s Tertiary Care Centre, Hyderabad, Telangana, India
3 Department of Pediatric Neurology, Rainbow Children×s Tertiary Care Centre, Hyderabad, Telangana, India
4 Surya Fertility Centre, Banjara Hills, Hyderabad, Telangana, India

Date of Web Publication27-Apr-2016

Correspondence Address:
Sirisha Rani Siddiahgari
Rainbow Children's Tertiary Care Centre, 22, Road No. 4, (Old Road No. 10), Karvy Lanes, Banjara Hills, Hyderabad - 500 034, Telangana
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1817-1745.181252

Rights and Permissions

 

   Abstract 

Extra nodal presentation of Non Hodgkins Lymphoma (NHL) is a rare entity, and data available about the NHL that primarily involves of middle ear and mastoid is limited. We report a case of diffuse large B cell lymphoma (DLBCL), in a 2 year 8 month old boy, who developed otalgia and facial palsy. Computed tomography revealed a mass in the left mastoid. Mastoid exploration and histopathological examination revealed DLBCL. This case highlights the importance of considering malignant lymphoma as one of the differential diagnosis in persistent otitis media and/facial palsy.


Keywords: Diffuse large B-cell lymphoma, facial palsy, non Hodgekin's lymphoma, Otalgia, otitis media


How to cite this article:
Siddiahgari SR, Yerukula P, Lingappa L, Moodahadu LS. Rare clinical presentation of diffuse large B-cell lymphoma as otitis media and facial palsy. J Pediatr Neurosci 2016;11:58-60

How to cite this URL:
Siddiahgari SR, Yerukula P, Lingappa L, Moodahadu LS. Rare clinical presentation of diffuse large B-cell lymphoma as otitis media and facial palsy. J Pediatr Neurosci [serial online] 2016 [cited 2020 Feb 26];11:58-60. Available from: http://www.pediatricneurosciences.com/text.asp?2016/11/1/58/181252



   Introduction Top


Non-Hodgkin lymphomas (NHLs) account for approximately 60% of all lymphomas in children and adolescents.[1] Childhood NHLs are subdivided into Burkitt's lymphoma, diffuse large B-cell lymphoma (DLBCL), lymphoblastic lymphoma, and anaplastic large-cell lymphoma. Incidence of DLBCL is varying from 6–10%[2] to 20% of pediatric NHL.[3] DLBCL is characterized by relatively more frequent extranodal presentation, seen in upto 40% of the cases.[4],[5] The most common extranodal location is the gastrointestinal tract. Primary involvement of the lymphoma of the middle ear is rare, with only about 18 cases being reported in literature so far.[6]

Here, we report a case of DLBCL, presented with features of facial palsy and otitis, who received initial symptomatic treatment and later chemotherapy after diagnosing DLBCL.


   Case Report Top


A 2 years 8 months old boy visited our Tertiary Care Hospital with complaints of ear ache (left side) and left facial palsy of 4 weeks duration. After initial 2 weeks of these symptoms, there was whitish serous discharge from the left ear. Before referral to our hospital, he was treated for otitis media with antibiotics, details of which were not available. His complete blood picture and biochemistry investigations done in our hospital were within normal limits. As the symptoms persisted for 4 weeks, computed tomography of head and neck was done which was suggestive of solid mass lesion of 2 cm × 2 cm size in the left mastoid with destruction of mastoid bone [Figure 1]. No abnormality was seen on the right side [Figure 2]. Suspecting chronic otitis media with cholesteatoma, left mastoidectomy was performed. A mass was found in the middle ear extending into the mastoid. The disease was in stage 1 (as per Murphy's staging) and was completely resected. Histopathological examination showed large cells of lymphoid cell proliferation immune histochemistry was positive for CD20, Bcl-2 and negative for CD3, with low MIB-1, which confirmed DLBCL. His positron emission tomography for staging, bone marrow and cerebrospinal fluid revealed no abnormality.
Figure 1: Computed tomography head and neck suggestive of solid mass lesion of 2 cm × 2 cm size in the left mastoid with destruction of mastoid bone

Click here to view
Figure 2: Computed tomography head and neck of right side showing no abnormality

Click here to view


His initial clinical symptoms of earache and facial nerve palsy followed by ear discharge resolved after starting chemotherapy as per B-cell lymphoma protocol for 6 months.

He tolerated chemotherapy well. Currently, he is 37 months off treatment, and no disease recurrence is seen clinically as well as radiologically.


   Discussion Top


DLBCL is a relatively a rare entity in children below 4 years.[7] It is an aggressive form of lymphoma, usually curable with appropriate treatment and has high survival rate. Rapid disease progression of DLBCL calls for an early, accurate diagnosis and appropriate treatment. Diagnosis is relatively easy if the patient presents with classical clinical features. However, unusual presentation can mislead the physician resulting in wrong diagnosis, which delays the treatment, thus promoting disease progression. Extranodal presentations of childhood DLBCL are relatively uncommon in clinical practice, and much rarer are those primarily involving middle ear and mastoid.

Involvement of middle ear and mastoid can resemble the features of otitis media and unusual facial palsy mimicking mastoiditis. Although these initial symptoms were suggestive of middle ear infection, the distinguishing factor was unresponsiveness to antibiotics.

There have been very few reports of NHL with facial nerve involvement; Ogawa et al. have reported 18 cases of NHL with facial nerve involvement.[6] Very few cases have been reported in children. McCabe et al.,[8] reported a case of 2-year-old, an immunocompetent boy with spontaneous regression of an Epstein-Barr-virus-associated monoclonal lymphoid proliferation who presented with acute otitis media and facial palsy. Kanzaki et al. have described a case of DLBCL with features of otitis media, mastoiditis, and facial palsy.[9]

We initially planned conservative management as per the symptoms and parent's preference. As symptoms persisted even after 4 weeks of treatment, the patient was started on chemotherapy, to which he promptly responded.

Extranodal NHLs of middle ear which is not a common clinical presentation may present as facial palsy, and misdiagnosed as otomastoiditis. There should be a high index of suspicion for primary neoplasms of the middle ear in patients with chronic otomastoiditis refractory to appropriate initial antibiotic therapy; early diagnosis and appropriate treatment results in good therapeutic outcome and minimizes further complications.

Acknowledgment

We acknowledge the parents of the child who consented to publish. We acknowledge the support of the management of Rainbow Hospital, Hyderabad, India, where this Case was reported. We acknowledge Dr. Srinivas Kishore, Consultant Otolaryngologist, Rainbow Hospital, Hyderabad, India.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Cairo MS, Raetz E, Perkins SL. Non-Hodgkin's lymphoma in children. In: Kufe DW, Bast RC, Hait WN, Hong WK, Pollock R, Decku BC. editors. Cancer Medicine. 7th ed. Ontario: BC Decker, Hamilton; 2005. p. 1962-76.  Back to cited text no. 1
    
2.
Oschlies I, Klapper W, Zimmermann M, Krams M, Wacker HH, Burkhardt B, et al. Diffuse large B-cell lymphoma in pediatric patients belongs predominantly to the germinal-center type B-cell lymphomas: A clinicopathologic analysis of cases included in the German BFM (Berlin-Frankfurt-Munster) multicenter trial. Blood 2006;107:4047-52.  Back to cited text no. 2
    
3.
Reiter A, Klapper W. Recent advances in the understanding and management of diffuse large B-cell lymphoma in children. Br J Haematol 2008;142:329-47.  Back to cited text no. 3
    
4.
Gatter KC, Warnke RA. Diffuse large B-cell lymphoma. In: Jaffe ES, Harris NL, Stein H, Vardiman JW. Editors. World Health Organization Classification of Tumors: Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. 3rd ed. Lyon, France: IARC Press; 2001. p. 171-4.  Back to cited text no. 4
    
5.
Coiffier B. Diffuse large cell lymphoma. Curr Opin Oncol 2001;13:325-34.  Back to cited text no. 5
    
6.
Ogawa S, Tawara I, Ueno S, Kimura M, Miyazaki K, Nishikawa H, et al. De novo CD5-positive diffuse large B-cell lymphoma of the temporal bone presenting with an external auditory canal tumor. Intern Med 2006;45:733-7.  Back to cited text no. 6
    
7.
Wood WA, Lee SJ. Malignant hematologic diseases in adolescents and young adults. Blood 2011;117:5803-15.  Back to cited text no. 7
    
8.
McCabe MG, Hook CE, Burke GA. Spontaneous regression of an EBV-associated monoclonal large B cell proliferation in the mastoid of a young child following surgical biopsy. Pediatr Blood Cancer 2008;51:557-9.  Back to cited text no. 8
    
9.
Kanzaki S, Saito H, Mori T, Shimasaki N, Mukai M, Ogawa K. Thirteen-month-old boy with malignant lymphoma having symptoms mimicking acute otitis media and mastoiditis with facial palsy. ORL J Otorhinolaryngol Relat Spec 2011;73:266-70.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2]



 

Top
Print this article  Email this article
 
 
  Search
 
  
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (794 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Report
   Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed1247    
    Printed9    
    Emailed0    
    PDF Downloaded82    
    Comments [Add]    

Recommend this journal