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. 2022 Oct;74(Suppl 2):2599-2606.
doi: 10.1007/s12070-020-02277-y. Epub 2020 Nov 25.

Lacrimal Gland Adenoid Cystic Carcinoma with High Grade Transformation: A Case Report and Current Concepts in Multi Modality Management

Siddharth A Shah  1 , Loma Parikh  2 , Raghuvir Solanki  1 , Jatin Bhojani  1 , Raviraj Gohil  1
Affiliations

Lacrimal Gland Adenoid Cystic Carcinoma with High Grade Transformation: A Case Report and Current Concepts in Multi Modality Management

Siddharth A Shah et al. Indian J Otolaryngol Head Neck Surg. 2022 Oct.

Abstract

Lacrimal gland adenoid cystic carcinoma (AdCC) is associated with an aggressive clinical course and grave prognosis. A high grade transformation within adenoid cystic carcinoma of lacrimal gland is a rare condition which is even more locally aggressive with frequent neck and distant metastasis. We present a case of left lacrimal gland adenoid cystic carcinoma with high grade transformation to adenocarcinoma NOS type presenting with orbital pain and proptosis. After thorough evaluation for locoregional and distant spread of the disease, the patient underwent left orbital exenteration with orbitectomy and neck dissection with free flap reconstruction. Patient received adjuvant radiation therapy and is presently disease free for last 6 months. A multi-modality management protocol involving surgery, radiotherapy and chemotherapy has been proposed for management of lacrimal gland AdCC with high grade transformation. We report the 4th case in the literature of lacrimal gland adenoid cystic carcinoma with high grade transformation.

Keywords: Adenoid cystic carcinoma; Adenoid cystic carcinoma with high grade transformation; Case report; Dedifferentiated adenoid cystic carcinoma; Lacrimal gland carcinoma; Multi-modality management.

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Conflict of interest statement

Conflict of interestThe authors declare that they have no conflict of interest.

Figures

Fig. 1
Fig. 1
Radiological images (MRI and PET CT). a Coronal and axial PECT-CT images show metabolically active left lacrimal gland tumor (large arrow) and it is abutting and minimally displacing retrobulbar portion of left optic nerve (arrowhead). b Coronal T2WFS and Post contrast T1WFS images show large heterogeneously enhancing left lacrimal gland tumor (large arrow) causing erosion of roof of orbit and adherent to dura of left anteroinferior aspect of frontal lobe (arrowhead)
Fig. 2
Fig. 2
Clinical and surgical profile of the patient. a Clinical picture, b Surgical Specimen, c Surgical Defect, d post operative period
Fig. 3
Fig. 3
Histopathological and IHC findings. a H&E slide showing high and low grade area. b Mib1 index higher (95%) in high grade area as compared to 10% in low grade area
See this image and copyright information in PMC

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