A 45-year-old woman, with no personal or family history of interest, who noticed a left mandibular clicking in relation to the mandibular opening preauricular palpable tumor, did not improve with treatment, to Emergency Care for Maxillofacial Surgery.
Facial manifestations are present in physical examination, as well as consolidation in the left preauricular region, arch cica and left mandibular angle.
This increase in consistency is poorly defined, and a clear tumor cannot be defined.
In the oral cavity, irregular induration of the left chewing space is observed, under suspicion of tumor involvement.
The patient had an oral opening of 40 mm. She had no palpable cervical or supraclavicular lymph nodes.
Imaging tests showed a solid tumor in the left hemimandible, 2.5 x 2 x 1.8 cm in the region of the mandibular condyle, with well-defined borders, probably an aggressive branch of bone destruction.
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Aspiration was performed with needle aspiration with pathological diagnosis of round cell tumor, negative for cytokeratins, common leukocyte antigen, synaptophysin and CD99.
Finally, the diagnosis was confirmed with a tumoral biopsy in which 5 irregular reddish tissue were sent, diagnosed as " round cell tumor and small bone fragments".
An immunohistochemical study was performed on the material fixed in formalin embedded in paraffin in which only positivity was observed with vimentin and CD99.
The proliferation index measured as nuclear positivity with MIB-1 was approximately 15% of the tumor cellularity.
Given the scarce material examined, it was not possible to determine whether the tumor corresponded to an Ewing's tumor or a peripheral neuroepithelioma.
Cytogenetic analysis was not performed.
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CT and bone scintigraphy did not show the presence of metastatic disease.
It was decided to start chemotherapy and evaluate surgical treatment or radiotherapy according to the response.
There were 5 cycles for 4 months.
After the third cycle required admission due to febrile neutropenia.
Magnetic resonance imaging (MRI) after chemotherapy suggested the possible progression of the tumor, so it was decided to give planned radiotherapy with radical intention for a month and a half.
Irradiation was performed in two phases, with a total dose on the tumor and a reduced margin of 60 Gy.
After radiotherapy treatment she developed mucositis grade 2, external otitis and epithelitis grade 2 in the treatment field.
Follow-up CT showed findings compatible with rests/recurrence of PAP, so it was decided to treat another 5 more cycles of chemotherapy, following the same previous scheme, for another 4 months.
The patient is currently 6 years old and remains free of disease under dental rehabilitation treatment.
