case_images: PMC5637853_01
case_images: (a) Tri-dimensional CT scan reveals increasing of the thickness of right maxillary sinus soft tissue. (b) Massive destruction of right maxillary body. (c) Right maxillary sinus opacity with destruction of sinus wall.
case_images: CRIM2017-2324717.001.jpg
case_images: PMC5637853_01_CRIM2017-2324717.001.jpg
case_images: fig1
case_images: Tri-dimensional head and neck computed tomography (CT) scan revealed increasing thickness of soft tissue of the right maxillary sinus area with massive destruction of the right maxillary sinus wall (Figures 1(a), 1(b), and 1(c)).
case_images: (a) Nests of neoplastic cells with peripheral palisading pattern within fibromyxoid stroma (Hematoxylin and eosin staining, original magnification x20). (b) Marrow involvement of orbital and maxillary sinus bones. (c) Neoplastic cells have dark hyperchromatic nuclei arranged in cribriform, solid nests, and trabecular formations. (d) Perineural invasion in the depth of tumor is evident (Hematoxylin and eosin stain, original magnification x20).
case_images: CRIM2017-2324717.002.jpg
case_images: PMC5637853_01_CRIM2017-2324717.002.jpg
case_images: fig2
case_images: Histopathological examination of Hematoxylin and Eosin (H&E) stained glass slides revealed a tumoral lesion composed of dark hyperchromatic neoplastic cells with round nuclei (basaloid-like cells) that were arranged in solid nests and acinar structures with peripheral palisading pattern in the fibromyxoid stroma without characteristic features of adenoid cystic carcinoma (Figure 2(a)). Due to the excessive extension of tumor cells into the surrounding tissues and the involvement of orbital bone, safe margins could not be made during the surgery (Figure 2(b)). The histopathological examination of received tissues showed tumoral lesion composed of small, dark, round cells with angulated nuclei, arranged in solid nests, trabeculae, cribriform, and tubular structures within fibromyxoid stroma in the depth of the tumor, with characteristic features of ACC tumors (Figure 2(c)). Numerous perineural and intraneural invasions with lymph node metastasis were present (Figure 2(d)).
case_images: (a), (b) Neoplastic cells have a positive reaction pattern for S-100 (immunohistochemical staining, original magnification, x20 and x40). (c) Positive reaction pattern for pan-cytokeratin (IHC staining original magnification x40). (d), (e) Positive reaction pattern for Ki67 (IHC staining, x20 and x40). (f) Negative reaction pattern of neoplastic cells for p63 in initial biopsy specimen (IHC staining, x20). (g), (h), (i) Positive reaction pattern of neoplastic cells for p63 in secondary excised lesion (immunohistochemical staining, x20 and x40).
case_images: CRIM2017-2324717.003.jpg
case_images: PMC5637853_01_CRIM2017-2324717.003.jpg
case_images: fig3
case_images: For definite diagnosis IHC studies were done and neoplastic cells showed positive reaction pattern to the S-100 (diffusely cytoplasmic and nuclear staining) (Figures 3(a) and 3(b)), pan-cytokeratin (AE1/AE3) (cell membrane staining) (Figure 3(c)), Vimentin, p53, C-kit, alpha smooth muscle actin, and also a ki67 labeling index of 60% (nuclear staining) (Figures 3(d) and 3(e)) (Dako, Denmark). All neoplastic cells showed negative staining pattern for p63 (Figure 3(f)). IHC studies revealed compartment staining pattern of neoplastic cells for p63 (Figures 3(g), 3(h), and 3(i)).