Ashok Kumar Prashanth, Paulraj Shweta, Mirchia Kanish, Hahn Seung Shin, Sivapiragasam Abirami
Department of Internal Medicine, SUNY Upstate Medical University, Syracuse, NY 13210, USA.
Department of Pathology, SUNY Upstate Medical University, Syracuse, NY 13210, USA.
Case Rep Oncol Med. 2020 Feb 3;2020:1790106. doi: 10.1155/2020/1790106. eCollection 2020.
Carcinoma ex pleomorphic adenoma (Ca ex PA) is a rare malignant transformation of a benign primary pleomorphic adenoma (PA). We report the case of a 62-year-old male who presented with a swelling over his left temple. Imaging revealed a lytic lesion over the left orbital wall with soft tissue extension suggestive of malignancy. He underwent an en bloc resection of the mass with orbital exenteration, craniotomy, and reconstruction. Pathology demonstrated a lacrimal gland ductal adenocarcinoma arising from a PA which led to the diagnosis of ductal adenoCa ex PA. Postoperatively, he received chemotherapy with 6 cycles of cisplatin and concurrent radiation therapy (RT), but his course was complicated by recurrent bacterial meningitis and abscesses and he ultimately opted for comfort measures. Patients with PA of the lacrimal gland experience an insidiously enlarging painless swelling of the orbit with transformation to Ca ex PA highlighted by a rapid onset of bulbar enlargement, displacement, and often proptosis. Ductal adenoCa ex PA is aggressive with a poor prognosis and has no established standard of care. This case highlights the rarity of this condition and the need for more literature to help direct treatment.
多形性腺瘤恶变（Ca ex PA）是一种罕见的良性原发性多形性腺瘤（PA）恶性转化疾病。我们报告一例62岁男性患者，其左颞部出现肿胀。影像学检查显示左眶壁有溶骨性病变，并伴有软组织延伸，提示为恶性肿瘤。他接受了肿物整块切除并眶内容剜除术、开颅手术及重建手术。病理检查显示为起源于PA的泪腺导管腺癌，从而诊断为导管腺癌伴多形性腺瘤恶变。术后，他接受了6周期顺铂化疗及同步放疗（RT），但病程中并发复发性细菌性脑膜炎和脓肿，最终他选择了姑息治疗措施。泪腺PA患者会出现眼眶无痛性肿胀且逐渐增大，转变为Ca ex PA时则表现为眼球迅速增大、移位，常伴有眼球突出。导管腺癌伴多形性腺瘤恶变侵袭性强，预后差，且尚无既定的标准治疗方案。本病例凸显了这种疾病的罕见性以及需要更多文献来指导治疗。
