Lacrimal Gland Adenoid Cystic Carcinoma with High Grade Transformation: A Case Report and Current Concepts in Multi Moda
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CLINICAL REPORT
Lacrimal Gland Adenoid Cystic Carcinoma with High Grade Transformation: A Case Report and Current Concepts in Multi Modality Management Siddharth A. Shah1 • Loma Parikh2 • Raghuvir Solanki1 • Jatin Bhojani1 Raviraj Gohil1
•
Received: 27 October 2020 / Accepted: 9 November 2020 Ó Association of Otolaryngologists of India 2020
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 Case report Lacrimal gland carcinoma Adenoid cystic carcinoma Adenoid cystic carcinoma with high grade transformation Dedifferentiated adenoid cystic carcinoma Multi-modality management
& Siddharth A. Shah [email protected] 1
Department of Surgical Oncology, Zydus Cancer Centre, Zydus Hospital, Ahmedabad, Gujarat 380054, India
2
Department of Histo-Pathology, Zydus Cancer Centre, Zydus Hospital, Ahmedabad, Gujarat, India
Abbreviations AdCC Adenoid cystic carcinoma HGT High grade transformation AdCC-HGT Adenoid cystic carcinoma with high grade transformation CECT Contrast enhanced computed tomography RT Radiation therapy 3D CRT 3 Dimensional conformal radiation therapy IMRT Intensity modulated radiation therapy AJCC American Joint Committee on Cancer IJV Internal jugular vein IACC Intra-arterial cytoreductive chemotherapy As2O3 Arsenic Trioxide TNF-a Tumor necrosis factor
Introduction Lacrimal gland tumors are a kind of salivary gland tumors representing 6–12% of orbital space occupying lesions. Primary malignant epithelial tumors represent around 20–30% of all lacrimal gland tumors which are associated with significant morbidity and mortality [1–3]. Adenoid cystic carcinoma (AdCC) is the commonest malignant epithelial tumor of lacrimal gland associated with poor prognosis despite aggressive local treatment due to high rates of local recurrences and late distant metastasis [3, 4]. Classical AdCC may undergo high grade transformation (HGT) or dedifferentiation which is associated with even worse clinical outcome due to high incidence of nodal metastasis and distant metastasis [5–7]. Since first description of ‘dediffe
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