Among the various tumours affecting the lacrimal gland, pleomorphic adenomas though most common can present clinically and radiologically as worrisome tumours. Histopathologic diagnosis hence is very critical for these tumours. We present a case of pleomorphic adenoma of the lacrimal gland which we encountered at our institutes. The patient was a 46-year-old male who came with complaints of a gradually progressing swelling in the right eye not associated with much pain or significant loss of vision. MRI revealed a large lobulated extrachoanal mass causing mass effect and scalloping of the adjacent bony structures. An excision biopsy was done and sent for histopathological examination. Histologically the tumour showed a biphasic morphology comprising of epithelial (ductal and myoepithelial) and chondromyxoid stromal components. Immunohistochemistry showed positivity for pan CK and CK 7 in ductal epithelial cells, p63 and S100 in myoepithelial cells and a low Ki67 index. On the basis of immunohistochemistry profile along with clinicoradiological correlation a final diagnosis of pleomorphic adenoma of the lacrimal gland was made. The patient post-surgery was doing well but was lost to follow up. It can thus be seen that is it absolutely necessary to accurately diagnose these benign but clinically misconceiving rare tumours of the lacrimal gland for appropriate management of such patients.
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