Garajei Ata, Modarresi Zahra Sadat, Bakhtiari Zeinab, Nazarpour Yousefdeh Ali
Department of Oral and Maxillofacial Surgery, School of Dentistry, Tehran University of Medical Sciences, Tehran, Iran; Cancer Research Center, Cancer Institute, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran.
Department of Oral and Maxillofacial Surgery, School of Dentistry, Tehran University of Medical Sciences, Tehran, Iran.
Int J Surg Case Rep. 2025 Sep;134:111765. doi: 10.1016/j.ijscr.2025.111765. Epub 2025 Aug 5.
Pleomorphic adenomas can undergo malignant transformation into carcinoma ex pleomorphic adenoma (CXPA). CXPA is challenging to diagnose due to its resemblance to benign tumors. Surgical excision is the primary treatment, with adjuvant radiotherapy recommended for high-risk cases. Despite the aggressive nature of CXPA, extensive surgery may not always be feasible, especially in cases with contraindications for neck dissection.
A 69-year-old female with a 20-year history of a slowly growing buccal mass presented with rapid enlargement over the last four months. Imaging and biopsy confirmed high-grade CXPA, and surgery was performed with careful dissection to preserve facial nerve function. The tumor measured 24 × 18 × 15 cm, and no perineural or lymphovascular invasion was detected. The patient received adjuvant radiotherapy and remained disease-free at 12 months, with preserved facial nerve function.
This case highlights the challenges of managing large CXPA tumors, particularly in preserving nerve function during surgery. The absence of perineural invasion in histological findings suggested a favorable prognosis, and postoperative radiotherapy significantly reduced the recurrence risk. While elective neck dissection is generally indicated in high-grade CXPA, it was contraindicated in this patient due to her medical condition.
CXPA, despite its aggressive nature, can often be managed without extensive resection or nerve sacrifice. In cases where neck dissection is not feasible, radiotherapy can be an effective treatment. Future research into the genetic basis and targeted therapies for CXPA is essential to enhance personalized treatment strategies and improve outcomes for patients with this complex malignancy.
多形性腺瘤可发生恶性转化为多形性腺瘤癌变(CXPA)。CXPA因其与良性肿瘤相似,诊断具有挑战性。手术切除是主要治疗方法,高危病例建议辅助放疗。尽管CXPA具有侵袭性,但广泛手术并不总是可行的,尤其是在有颈部清扫禁忌证的病例中。
一名69岁女性,有一个缓慢生长的颊部肿物20年病史,在过去四个月肿物迅速增大。影像学检查和活检确诊为高级别CXPA,并进行了手术,术中仔细解剖以保留面神经功能。肿瘤大小为24×18×15cm,未检测到神经周围或淋巴管侵犯。患者接受了辅助放疗,12个月时无疾病复发,面神经功能得以保留。
该病例突出了处理大型CXPA肿瘤的挑战,特别是在手术中保留神经功能方面。组织学检查结果中未发现神经周围侵犯提示预后良好,术后放疗显著降低了复发风险。虽然高级别CXPA一般建议进行选择性颈部清扫,但该患者因自身病情存在禁忌证。
CXPA尽管具有侵袭性,但通常无需广泛切除或牺牲神经即可得到治疗。在无法进行颈部清扫的病例中,放疗可能是一种有效的治疗方法。未来对CXPA的遗传基础和靶向治疗的研究对于加强个性化治疗策略和改善这种复杂恶性肿瘤患者的治疗效果至关重要。