A 67-year-old woman with a knee prosthesis and a history of cholecystectomy and umbilical herniorrhaphy attended the clinic due to a tumor in the left genial region of about 2 years of evolution, very slowly decreasing.
The only symptoms reported are those derived from the size of the tumor.
On examination, two tumors, apparently independent of each other and of the parotid, about 2 or 3 cm, mobile, well-defined and elastic consistency were observed.
Preoperative FNAC showed a smear with cytological picture suggestive of mixed salivary gland tumor.
Since in the exploration the tumor is located, in principle, independent of the parotid and in a rare location for a pleomorphic adenoma, we decided to perform MRI.
This evidences a space-occupying lesion in the soft parts of the left cheek, of homogeneous signal, hypointense in T1 and markedly hyperintense in T2.
This image is compatible with lymphangioma, which in its cranial part extends to the height of the upper limit of the orbita and descends in contact with the outer edge of the oral cavity anteriorly entering the oral cavity to the height.
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After preoperative surgery, the intervention was performed using an intraoral approach.
When the mucosa is involved, a herniated formation is observed, which is easy to separate from surrounding tissues by blunt dissection.
The final result shows a tumor of 2 x 7 cm, cystic appearance and tubular form arrosariated.
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The anatomopathological study of the specimen shows macroscopically an elongated formation, cydrica extirpated, of about 11 x 1.7 cm covered by a capsule with surface vessels and prominently attached cylindrical structures.
The cut presents areas of dense fibrous appearance, hyalinized, alternating with areas of more lax aspect.
The microscopic study revealed a proliferation of cells with eosinophilic cytoplasm, with ill-defined borders, with fusiform nuclei with their ends elongated, which are available in bundles acquiring sometimes a serous aspect.
No mitotic figures were observed.
The tumor presents zones more than identical to others much more alternating with others, containing walls vessels sometimes hyalinized, in general and cystic fibrosis.
The tumor appears well delimited and nervous fascicles are observed in the capsule, some with onset of proliferation.
The definitive pathological diagnosis was plexiform neurofibroma.
In our case, we found no clinical findings or personal or family history suggestive of neurofibromatosis type I or polyglandular syndrome MEN III (MEN IIb) and it seems to confirm that the isolated lesions are independent of these.
Currently, two years after surgery, the patient has not presented any signs of tumor recurrence in successive reviews.
