PMC7441417_01
(a) Clinical photo of the patient at time of presentation, direct view. (b) Computed tomography maxillofacial, axial cut. Illustrating a 3.8 cm mass of the lateral orbit eroding the lateral orbital wall with a soft tissue component extending into the orbit causing proptosis and nonaxial displacement of the globe. (c) MRI brain and orbit T1 fat-suppressed image with gadolinium showing a lobulated mass with mixed cystic and solid components and inhomogeneous enhancement, involving the left lateral orbital wall and suprazygomatic left masticator space. Additionally, the mass demonstrates edema and enhancement suggesting infiltration by the mass.
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The external examination revealed edematous upper and lower eyelids, predominantly overlying the lateral orbital rim associated with temporal sloping and a nontender, palpable, and fixed mass of the temporal fossa (Figure 1(a)). Maxillofacial CT scan with contrast showed a lytic lesion of the left orbital wall with associated heterogeneous soft tissue mass measuring 3.8 x 2.7 cm medially displacing the left lateral rectus muscle (Figure 1(b)). Magnetic resonance imaging of the brain and orbits revealed an enhancing infiltrating mass of the left lateral orbital wall extending into the left supra zygomatic masticator space (Figure 1(c)).
(a) Moderately differentiated adenocarcinoma involving cores of fibrous connective tissue. H&E 2x. (b) High power view of the tumor revealed a cribriform pattern, moderate nuclear pleomorphism, and scattered mitoses. H&E 40x. (c) Neoplastic cystic structures in close proximity to lacrimal gland tissue (star) in a hyalinized stroma and lymphoid proliferation. H&E 2x. (d) Tumor that arose from a focus of pleomorphic adenoma (star) depicting cystic structures with small papillae. H&E 4x. Inset: papillary projections lined by large cuboidal cells with moderate nuclear atypia and apocrine features. H&E 20x. (e) Tumor cells were strongly positive for HMWK903 (4x). (f) Nuclear expression of androgen receptor was present in the tumor (20x). (g) p53, positive in less than 50% of tumor cells (4x). (h) p63 was negative within the invasive component of the tumor (4x).
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A core guided needle biopsy was performed, and the hematoxylin-eosin- (H&E-) stained slide revealed a moderately differentiated adenocarcinoma involving fibrous connective tissue and demonstrating a cribriform architectural pattern with moderate cytologic atypia and individual cell necrosis (Figures 2(a) and 2(b)). Histopathologically, the H&E-stained sections disclosed predominantly neoplastic cystic structures in the proximity of the lacrimal gland acini measuring 1 to 10 mm in diameter, infiltrating fibrous connective tissue and bone (Figure 2(c)). The invasive cystic component revealed intraluminal papillary architecture and cribriform arch formations of the lining epithelium (Figure 2(d)). TTF-1 stains (Figures 2(e)-2(h)). p53 was positive in less than 50% of the tumor cells. The Ki-67 proliferative index of the tumor cells was low (5-10%). p63 and SOX-10 stains highlighted the PA and the in situ component of the tumor.