Syrnioti Georgia, Syrnioti Antonia, Abdullah Alharith, Lui Xuehui, Mendoza Ernesto
Surgery, Brookdale University Hospital Medical Center, New York, USA.
Pathology, Aristotle University of Thessaloniki, Thessaloniki, GRC.
Cureus. 2023 Mar 3;15(3):e35722. doi: 10.7759/cureus.35722. eCollection 2023 Mar.
Carcinoma ex pleomorphic adenoma (Ca-ex-PA) is a rare tumor that arises from the malignant transformation of a primary or recurrent pleomorphic adenoma. Despite being benign, pleomorphic adenomas can rarely undergo malignant transformation. Risk factors include a long-standing primary tumor, a prior history of radiation exposure, increased tumor size, and recurrent disease. Ca-ex-PA usually affects patients between the sixth and eighth decades of life, approximately 10 to 20 years after the development of a pleomorphic adenoma. Patients usually present with the rapid expansion of an already existing mass. We describe a case report of a patient who presented with Ca-ex-PA of the submandibular gland. The patient underwent surgical excision of the affected gland, which was consistent with a widely invasive myoepithelial Ca-ex-PA. The patient underwent postoperative radiation to the neck and the tumor bed. No local or distant recurrence was noted during the one-year follow-up. Due to the rarity of the disease entity and the infrequent location of the tumor, this case presents a particular diagnostic and therapeutic challenge.
多形性腺瘤恶变（Ca-ex-PA）是一种罕见的肿瘤，由原发性或复发性多形性腺瘤恶变而来。尽管多形性腺瘤是良性的，但很少会发生恶变。危险因素包括长期存在的原发性肿瘤、既往有辐射暴露史、肿瘤体积增大以及复发性疾病。Ca-ex-PA通常发生在60至80岁的患者中，大约在多形性腺瘤发生后的10至20年。患者通常表现为已有肿块的迅速增大。我们报告一例下颌下腺Ca-ex-PA患者的病例。患者接受了患侧腺体的手术切除，病理结果为广泛浸润性肌上皮Ca-ex-PA。患者术后接受了颈部及肿瘤床的放疗。在一年的随访中未发现局部或远处复发。由于该疾病实体罕见且肿瘤位置不常见，该病例带来了特殊的诊断和治疗挑战。
