Merry Eve, Smrke Alannah, Halai Kapil, Patel Gulam, Thway Khin, Jones Robin L, Benson Charlotte
Sarcoma Unit, The Royal Marsden Hospital NHS Foundation Trust, 203 Fulham Road, London, SW3 6JJ UK.
Rheumatology Department, Ashford and St Peter's NHS Hospitals NHS Foundation Trust, London, TW15 3AA UK.
Clin Sarcoma Res. 2020 Aug 25;10:15. doi: 10.1186/s13569-020-00140-w. eCollection 2020.
Sarcomas are rare and heterogeneous tumours of mesenchymal origin, with over 100 histological subtypes. Paraneoplastic dermatomyositis has rarely been described in sarcoma. This is the first documented case of paraneoplastic dermatomyositis in a patient with metastatic leiomyosarcoma.
A 43-year-old female diagnosed with metastatic leiomyosarcoma of unknown primary presented with a mild rash in sun-exposed areas of her face and upper chest, with no other neuromuscular symptoms. This rash resolved with systemic treatment with doxorubicin for metastatic leiomyosarcoma. Imaging assessment confirmed overall stable disease after chemotherapy completion. She presented acutely 2 months later with new onset rash in a shawl-like distribution, periorbital oedema and proximal muscle weakness. Based on the characteristic cutaneous signs and symmetrical proximal muscle weakness, abnormal electromyography and raised skeletal muscle enzymes with a positive anti-transcription intermediary factor-1 gamma antibody result, a diagnosis of paraneoplastic dermatomyositis was made. Re-evaluation of her metastatic leiomyosarcoma revealed disease progression. Second-line chemotherapy was commenced once the dermatomyositis was controlled on steroid therapy. Systemic anti-cancer therapy was again associated with mild improvement in dermatomyositis symptoms.
Paraneoplastic dermatomyositis heralded disease progression after first-line chemotherapy; however, in hindsight, subtle cutaneous features were present at sarcoma diagnosis. The temporal relationship between paraneoplastic dermatomyositis and metastatic leiomyosarcoma is key in this case, as fluctuations in dermatomyositis severity correlated with growth of metastatic disease. Understanding this relationship may provide clues for tumour progression and prompt timely initiation of anti-cancer therapy. It is important to recognise that in addition to the more common cancers associated with paraneoplastic dermatomyositis, it can also occur in rarer tumours such as leiomyosarcoma.
肉瘤是起源于间充质的罕见且异质性肿瘤，有超过100种组织学亚型。副肿瘤性皮肌炎在肉瘤中很少被描述。这是首例记录在案的转移性平滑肌肉瘤患者发生副肿瘤性皮肌炎的病例。
一名43岁女性，诊断为原发灶不明的转移性平滑肌肉瘤，面部和上胸部暴露于阳光下的部位出现轻度皮疹，无其他神经肌肉症状。该皮疹经多柔比星全身治疗转移性平滑肌肉瘤后消退。化疗完成后影像学评估证实疾病总体稳定。2个月后，她急性发作，出现披肩样分布的新发皮疹、眶周水肿和近端肌无力。基于特征性的皮肤体征、对称性近端肌无力、异常肌电图以及骨骼肌酶升高且抗转录中介因子-1γ抗体结果呈阳性，诊断为副肿瘤性皮肌炎。对其转移性平滑肌肉瘤的重新评估显示疾病进展。在皮肌炎通过类固醇治疗得到控制后开始二线化疗。全身抗癌治疗再次使皮肌炎症状有轻度改善。
副肿瘤性皮肌炎预示着一线化疗后疾病进展；然而，事后看来，在肉瘤诊断时就存在细微的皮肤特征。在该病例中，副肿瘤性皮肌炎与转移性平滑肌肉瘤之间的时间关系是关键，因为皮肌炎严重程度的波动与转移性疾病的生长相关。了解这种关系可能为肿瘤进展提供线索，并促使及时启动抗癌治疗。重要的是要认识到，除了与副肿瘤性皮肌炎相关的更常见癌症外，它也可能发生在如平滑肌肉瘤等罕见肿瘤中。
