Sawamura Eiho, Hamamoto Hiroki, Suzuki Yusuke, Kuramoto Toru, Shima Takafumi, Kitada Kazuya, Asakuma Mitsuhiro, Lee Sang-Woong
Department of General and Gastroenterological Surgery, Osaka Medical and Pharmaceutical University, Takatsuki, Japan.
Medicine (Baltimore). 2025 Aug 8;104(32):e43758. doi: 10.1097/MD.0000000000043758.
Dermatomyositis (DM) is a systemic inflammatory disorder involving the muscles and skin and is often associated with malignancies in adults. Anti-transcriptional intermediary factor 1-gamma (anti-TIF1-γ) antibodies are specific markers of a subset of DM with a high risk of cancer, highlighting the importance of recognizing this paraneoplastic syndrome for early diagnosis and treatment. This report describes a rare case of tumor-associated DM that developed during chemotherapy for advanced rectal cancer and emphasizes the importance of multidisciplinary management in such conditions.
A 75-year-old woman presented with rectal bleeding and progressive symptoms, including severe erythema, rash, muscle weakness, and general fatigue, following preoperative chemotherapy for rectal cancer. The symptoms severely impacted her quality of life, raising concerns about a potential paraneoplastic syndrome.
The patient was diagnosed with tumor-associated DM based on clinical findings of a heliotrope rash, Gottron's sign, and elevated muscle enzyme levels. The presence of anti-TIF1-γ antibodies confirmed the diagnosis. Preoperative evaluation identified rectal cancer as stage IIa (pT3, N0, M0) and the likely underlying malignancy contributing to DM.
The patient underwent immunotherapy with steroids and high-dose intravenous immunoglobulin, which stabilized her condition. Subsequently, a robot-assisted Hartmann's operation was performed to resect the rectal tumor and establish a colostomy. Postoperative care included monitoring for DM symptom resolution and managing her nutritional and rehabilitation needs.
Within 3 weeks of surgery, the patient's muscle enzyme levels normalized, and her symptoms, including erythema and muscle weakness, resolved. Follow-up evaluations showed no recurrence of DM or cancer over the subsequent year, indicating a successful outcome with a multidisciplinary approach.
This case underscores the importance of early recognition and management of paraneoplastic syndromes such as tumor-associated DM. A multidisciplinary approach combining immunotherapy and surgical intervention for the underlying malignancy can lead to favorable outcomes. This case also highlights the significance of long-term monitoring for both cancer recurrence and autoimmune complications in such patients.
皮肌炎(DM)是一种累及肌肉和皮肤的系统性炎症性疾病,在成人中常与恶性肿瘤相关。抗转录中介因子1-γ(抗TIF1-γ)抗体是DM中一部分患癌风险较高的亚组的特异性标志物,凸显了识别这种副肿瘤综合征对于早期诊断和治疗的重要性。本报告描述了一例在晚期直肠癌化疗期间发生的罕见肿瘤相关性DM病例,并强调了在这种情况下多学科管理的重要性。
一名75岁女性在直肠癌术前化疗后出现直肠出血及进行性症状,包括严重红斑、皮疹、肌肉无力和全身疲劳。这些症状严重影响了她的生活质量,引发了对潜在副肿瘤综合征的担忧。
根据向阳疹、Gottron征及肌肉酶水平升高的临床表现,该患者被诊断为肿瘤相关性DM。抗TIF1-γ抗体的存在证实了诊断。术前评估确定直肠癌为IIa期(pT3,N0,M0),且可能是导致DM的潜在恶性肿瘤。
患者接受了类固醇和大剂量静脉注射免疫球蛋白的免疫治疗,病情得以稳定。随后,进行了机器人辅助Hartmann手术以切除直肠肿瘤并建立结肠造口术。术后护理包括监测DM症状的缓解情况以及满足她的营养和康复需求。
术后3周内,患者的肌肉酶水平恢复正常,包括红斑和肌肉无力在内的症状得到缓解。后续评估显示,在随后的一年中,DM或癌症均未复发,表明多学科方法取得了成功。
该病例强调了早期识别和管理肿瘤相关性DM等副肿瘤综合征的重要性。将免疫治疗与针对潜在恶性肿瘤的手术干预相结合的多学科方法可带来良好的结果。该病例还凸显了对此类患者进行癌症复发和自身免疫并发症长期监测的重要性。