PMC5863316_01
A painful and firm mass at the level of the right orbital area of substantial size in a male Senegalese ten-month-old baby.
CRIONM2018-1684763.001.jpg
PMC5863316_01_CRIONM2018-1684763.001.jpg
fig1
In the following months, the neoplasm had met with a rapid expansive and infiltrative growth (Figure 1).
CT scan showing the orbital involvement with the lateral wall of the orbit infiltration.
CRIONM2018-1684763.002.jpg
PMC5863316_01_CRIONM2018-1684763.002.jpg
fig2
Furthermore, the neoplasm extended into the maxillary sinus and the temporal and infratemporal fossa (Figures 2 and 3).
CT scan showing the neoplasm extended into the maxillary sinus and the temporal and infratemporal fossa.
CRIONM2018-1684763.003.jpg
PMC5863316_01_CRIONM2018-1684763.003.jpg
fig3
Furthermore, the neoplasm extended into the maxillary sinus and the temporal and infratemporal fossa (Figures 2 and 3).
Histological features of desmoid fibromatosis.
CRIONM2018-1684763.004.jpg
PMC5863316_01_CRIONM2018-1684763.004.jpg
fig4
The histopathological evaluation revealed paucicellular fibrous proliferation in interlacing fascicles of bland fibroblasts (Figure 4(a), haematoxylin and eosin, original magnification 40x). Few mitotic figures and no atypia were observed (Figure 4(b), haematoxylin and eosin, original magnification 200x). Immunohistochemically, the spindle cells focally stain with smooth muscle actin (Figure 4(c), haematoxylin counterstaining, original magnification 100x).
Mutational status of the catenin beta-1 (CTNNB1) gene in the tumor analyzed by Sanger sequencing.
CRIONM2018-1684763.005.jpg
PMC5863316_01_CRIONM2018-1684763.005.jpg
fig5
Subsequently, the mutational status of the catenin beta-1 (CTNNB1) gene in the tumor was analyzed by Sanger sequencing, which revealed the S45F somatic mutation (Figure 5).
Postoperative period and tissue healing after the orbital exenteration with surgical resection of the tumor.
CRIONM2018-1684763.006.jpg
PMC5863316_01_CRIONM2018-1684763.006.jpg
fig6
The postoperative period has elapsed without any complication (Figure 6).
Application of an ocular prosthesis after the fornix deepening and the reconstruction of the lower eyelid.
CRIONM2018-1684763.007.jpg
PMC5863316_01_CRIONM2018-1684763.007.jpg
fig7
After a short retention period, the application of an ocular prosthesis has been scheduled (Figure 7).