Shah Siddharth A, Parikh Loma, Solanki Raghuvir, Bhojani Jatin, Gohil Raviraj
Department of Surgical Oncology, Zydus Cancer Centre, Zydus Hospital, Ahmedabad, Gujarat 380054 India.
Department of Histo-Pathology, Zydus Cancer Centre, Zydus Hospital, Ahmedabad, Gujarat India.
Indian J Otolaryngol Head Neck Surg. 2022 Oct;74(Suppl 2):2599-2606. doi: 10.1007/s12070-020-02277-y. Epub 2020 Nov 25.
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.
泪腺腺样囊性癌（AdCC）具有侵袭性的临床病程和严重的预后。泪腺腺样囊性癌中的高级别转化是一种罕见情况，其局部侵袭性更强，常伴有颈部和远处转移。我们报告一例左泪腺腺样囊性癌高级别转化为非特指型腺癌的病例，该患者表现为眼眶疼痛和眼球突出。在对疾病的局部和远处扩散进行全面评估后，患者接受了左眼眶内容剜除术、眶切除术及颈部清扫术，并进行了游离皮瓣重建。患者接受了辅助放疗，目前已无病生存6个月。我们提出了一种包括手术、放疗和化疗的多模式管理方案，用于治疗伴有高级别转化的泪腺AdCC。我们报告了文献中第4例泪腺腺样囊性癌高级别转化的病例。
