Spontaneous Regression Of An Ebv-associated Monoclonal Large B Cell Proliferation In The Mastoid Of A Young Child Following Surgical Biopsy
McCabe, M. G., Hook, C. E., & Burke, G. A. (2008). spontaneous regression of an EBV-associated monoclonal large B cell proliferation in the mastoid of a young child following surgical biopsy. Pediatric blood & cancer, 51(4), 557–559. https://doi.org/10.1002/pbc.21637
View Original Source →Abstract
We report the spontaneous regression of an Epstein-Barr virus-associated monoclonal lymphoid proliferation in an immunocompetent child. A 2-year-old male with acute otitis media presented with a right-sided facial palsy secondary to acute mastoiditis. During mastoid decompression a polypoid mass, a histologically diffuse large B cell lymphoma, was found. Staging revealed localized disease. At surgical re-exploration 5 weeks later the disease had resolved. Retrospective serological testing was consistent with an acute Epstein-Barr viral infection and in situ hybridization of the tumour tissue was positive for Epstein-Barr RNA.
Case Details
Disease Location
Right mastoid
Personal Characteristics
2 -year-old male diplegic cerebral palsy
Clinical Characteristics
Presented with a 2-week febrile illness associated with a discharging right ear, amoxicillin had not resolved either the fever or aural discharge. There was no immunosuppressive therapy used. 24 hrs prior to presentation, the child had a right-sided facial weakness the the patient became increasingly lethargic systemic exam revealed long-standing neurological abnormaliteis relating to his cerebral palsy in addition to right sided facial palsy complete blood count was normal, serum IGA and iggl were mildly abnormal. Peripheral blood lymphocyte subset analysis and further immune function testing were not performed CT scan found fluid in the right mastoid, a presumptive diagnosis of acute mastoiditis was made and surgical mastoidectomy was performed exam of the right ear under anesthesia revealed a granulating polypoid mass filling the middle ear, no tympanic membrane could be identified the mass was biopsied and the remainder of the mass was left in situ. Following surgical decompression and broad-spectrum IV antibiotics, the mastoiditis improved there was no evidence of bacterial or fungal infection in the biopsy, however histological exam showed an unexpected lymphoma. The biopsy comprised a partly necrotic tumor of non-cohesive cells, polygonal in shape and with large nuclei, granular chromatin and prominent nucleoli immunohistochemical exams confirmed lymphoid origin with the expression of CD45 and no expression for a broad panel of non-lymphoid markers. The cells had a b-cell phenotype and were negative for CD10, bcl-6, and tdt. There was an expression of bcl-2. There was a monoclonal rearrangement of igk and a heavy chain locus consistent with clonal b-cell population. Diffuse large b-cell lymphoma was considered at diagnosis CT scan of mastoid 1 month later revealed a persistent soft-tissue abnormality which enhanced with contrast. This suggested residual tumor or post-op inflammation. Further staging with chest, abdomen and pelvis CT were normal. A second mastoid surgery was performed to obtain fresh tumor material 5 weeks after the first biopsy, however no residual tumor was apparent histological exam of biopsies taken during the second surgery composed of only granulation tissue and fibrosis with no evidence of neoplasia retrospective review of the original biopsy revealed evidence of ebv infection and HIV 1 and 2 negativity. In-situ hybridization for eber was positive in the large atypical cells. Ebv DNA positivity was not assessed at the time of initial presentation or follow up
Remission Characteristics
The mastoiditis improved following surgical decompression and IV antibiotics second biopsy could’t find a residual tumor the right-sided facial palsy resolved within 3 months of diagnosis and there was no recurrence of symptoms the child was last seen 2 years following diagnosis and remains symptom free
Treatment & Mechanisms
Proposed Remission Mechanisms
Surgery linked with an immune response leading to immune recognition and destruction of the remaining neoplastic cells…a post-inflammatory repair process the ebv infection may have played a part
Clinical Treatment
Amoxicillin mastoidectomy surgical decompression and IV antibiotics
Non-Clinical Treatment
None reported