Department of Oncology, Charles University, Faculty of Medicine and University Hospital Královské Vinohrady, Prague, Czech Republic;
Department of Pathology, Charles University, Faculty of Medicine and University Hospital Královské Vinohrady, Prague, Czech Republic.
In Vivo. 2024 Nov-Dec;38(6):3125-3130. doi: 10.21873/invivo.13798.
BACKGROUND/AIM: Adenoid cystic carcinoma (AdCC) is a rare malignant tumor that primarily affects the salivary glands but can also occur in other organs. Low incidence and unpredictable clinical behavior make AdCC one of the most difficult head and neck tumors to treat.
We present the case of a 54-year-old woman with AdCC localized at the base of the tongue, following radical surgical and oncological therapy. Due to advances in palliative oncological treatment, there is a more than five-year survival period before the progression of metastatic disease. Considering the rare occurrence of this disease, a literature search was also conducted, and therapy options are discussed. Ensuring a sufficient extent of the surgical procedure is still a challenge, and most specialists agree that subsequent postoperative radiotherapy reduces the risk of local recurrence. The effective dose of radiotherapy to the area of the primary tumor and lymph nodes is not clearly defined.
The distinct biological behavior of AdCC results in varying sensitivity to chemotherapy or radiotherapy compared to treatments commonly used for head and neck squamous cell carcinomas. Treatment recommendations for these rarer tumors are based mainly on case reports and small clinical trials. The acquired therapeutic experience can contribute to prolonging the survival period of patients and improving their prognosis and quality of life.
背景/目的:腺样囊性癌(AdCC)是一种罕见的恶性肿瘤,主要影响唾液腺,但也可能发生在其他器官。低发病率和不可预测的临床行为使 AdCC 成为最难治疗的头颈部肿瘤之一。
我们报告了一例 54 岁女性的病例,她患有舌根局部的 AdCC,经过根治性手术和肿瘤治疗。由于姑息性肿瘤治疗的进展,在转移性疾病进展之前有超过五年的生存时间。考虑到这种疾病的罕见发生,我们还进行了文献检索,并讨论了治疗选择。确保手术范围足够仍然是一个挑战,大多数专家认为随后的术后放疗可降低局部复发的风险。原发肿瘤和淋巴结区域的放疗有效剂量尚未明确界定。
与常用于头颈部鳞状细胞癌的治疗相比,AdCC 的独特生物学行为导致对化疗或放疗的敏感性不同。这些更罕见肿瘤的治疗建议主要基于病例报告和小型临床试验。所获得的治疗经验有助于延长患者的生存时间,改善其预后和生活质量。