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

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.

Introduction

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.[45] 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

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

Figure 2

Computed tomography head and neck of right side showing no abnormality

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

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.