CASE PRESENTATION
A 7-year-old girl presented at her local maxillofacial department with
pain on the left side of the mandible. Two months before presentation
these complaints began as intermittent pain. The left jaw began to swell
and ache intensively. The diagnosis of inflammation was most probable
given the young age, pain and the localized
swelling.11 Before any imaging, the local
maxillofacial surgeon tried to treat a possible abscess by incision
combined with intravenous (IV) amoxicillin clavulanic acid. Only
significant edema of the soft tissue without pus drainage was recorded.
Superficial necrosis was noticed due to friction of the upper molars in
the left cheek (Figure 1A). The patient had no medical history. A
referral to the university hospital took place.
After panoramic radiograph (Figure 2) further imaging modalities were
initiated based on osteolytic lesions found in the lower left quadrant.
Absence of the thin circumferential cortical layer around the second
molar in the lower jaw on the left side was
recorded.12 Through ultrasound imaging an
inhomogeneous, relatively well-defined soft tissue mass was shown above
the submandibular salivary gland with invasion in the mandible. A
computed tomography (CT) (Figure 3C) was performed which showed a mass
in the left masseter muscle region, fixed to the ascending mandibular
ramus with osteolysis of the bony tissue. These findings were confirmed
using magnetic resonance imaging (MRI)(Figure 3B). For staging a
positron emission tomography – computed tomography (PET-CT)(Figure 3A)
was conducted indicating metastases which were confirmed through
ultrasonography of the abdomen.
A final diagnosis was made by biopsy and histopathological examination
respectively.13 A partial resection of the mass that
interfered with the occlusion was performed. The histopathological
examination revealed an ulcerated fragment mucosa with the presence of a
poorly differentiated neoplastic process, consisting of a diffuse
proliferation of medium-sized, monomorphic blastoid cells with round
vesicular to hyperchromatic nuclei. The nuclei contained small nucleoli
and numerous mitotic figures were present. Between the cells there are
tingible body macrophages giving rise to a starry sky appearance.
(Figure 4) The immunophenotype identified the presence of a B-cell
lymphoproliferative process expressing CD20, CD10, BCL6, MYC and a
proliferation rate of 100%, but no expression of BCL2, TDT,
cytokeratin, desmin, myogenin, CD99, MPO, CD3 or EBV. PCR showed
monoclonal immunoglobulin heavy and light chain rearrangements.
Fluorescence in-situ hybridization (FISH) confirmed diagnosis of Burkitt
Lymphoma (MYC rearrangement, stage III, LDH>2N, high risk
group)14 resulting in an overproduction of the MYC
protein due to genetic rearrangement. The involvement of adenopathies on
both sides of the diaphragm on PET-CT defines it as a stage III lesion
based on the St. Jude classification. There was no invasion of the bone
marrow. The LDH concentration was 786 U/L (normal range 192 – 321 U/L)
indicating highly proliferative disease. Risk stratification was
performed for treatment planning and is linked to the stage of the
disease, site as well as LDH level.13–15 Chemotherapy
was initiated combined with rituximab according to the intergroup B-NHL
Ritux 2010 trial group B at the department for pediatric oncology and
hematology.14
During hospitalization sufficient pain relief was achieved with IV
administration of paracetamol and tramadol by weight and oral hygiene
was restarted subsequently. After a part of the chemotherapy was
administered the swelling diminished significantly. (Figure 1B)