背景:副肿瘤性天疱疮(PNP)是一种罕见的,危及生命的自身免疫性大疱性疾病。在500例报告的PNP病例中,只有1例与扁桃体癌有关,具体来说,人乳头瘤病毒(HPV)阳性的鳞癌。然而,PNP在非HPV相关扁桃体癌中的发生非常罕见,迄今尚未有报道.
方法:我们介绍了一名58岁男性,有吸烟史,经历了复发性口腔溃疡,右颈部肿胀,声音嘶哑5个月.诊断为右扁桃体鳞状细胞癌(cT1N3bM0),经计算机断层扫描/磁共振成像及病理证实,与HPV无关。组织学和免疫组织化学结果提示PNP。
结果:患者接受原发肿瘤切除和同侧颈清扫术。局部使用类固醇和抗真菌药物来管理口腔病变并预防继发感染。顺铂辅助同步放化疗进展顺利。同步放化疗后随访3、6和9个月,利用计算机断层扫描/磁共振成像和鼻咽镜检查,没有发现复发癌症或PNP的迹象。
结论:早期指标,如口腔粘膜溃疡和皮肤水疱,及时考虑PNP中潜在的口腔癌。综合检查对于诊断PNP和识别并发的内部肿瘤至关重要。有效的管理包括隐匿性恶性肿瘤的治疗,术后类固醇治疗,和感染预防。
BACKGROUND: Paraneoplastic pemphigus (PNP) is a rare, life-threatening autoimmune bullous disease. Among the ≈500 reported cases of PNP, only 1 case has been associated with tonsillar cancer, specifically, human papillomavirus (HPV)-positive squamous carcinoma. However, the occurrence of PNP in non-HPV-related tonsillar cancer is exceptionally rare and has not been reported to date.
METHODS: We present a 58-year-old male with a history of smoking, who experienced recurrent oral ulcers, right neck swelling, and hoarseness for 5 months. Diagnosis of right tonsillar squamous cell carcinoma (cT1N3bM0) was confirmed through computed tomography/magnetic resonance imaging and pathology, not associated with HPV. Histological and immunohistochemical findings indicated PNP.
RESULTS: The patient underwent primary tumor resection and ipsilateral neck dissection. Topical steroids and antifungal agents were administered to manage oral lesions and prevent secondary infections. Adjuvant concurrent chemoradiotherapy with cisplatin proceeded smoothly. Postconcurrent chemoradiotherapy follow-up at 3, 6, and 9 months, utilizing computed tomography/magnetic resonance imaging and nasopharyngoscopy, revealed no signs of recurrent cancer or PNP.
CONCLUSIONS: Early indicators, such as oral mucosal ulcers and skin blisters, prompt consideration of underlying oral cancer in PNP. Comprehensive examination is crucial for diagnosing PNP and identifying concurrent internal neoplasms. Effective management includes occult malignancy treatment, postoperative steroid therapy, and infection prevention.