Paraneoplastic dermatomyositis associated with metastatic leiomyosarcoma of unknown primary
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Clinical Sarcoma Research Open Access
CASE REPORT
Paraneoplastic dermatomyositis associated with metastatic leiomyosarcoma of unknown primary Eve Merry1, Alannah Smrke1, Kapil Halai2, Gulam Patel2, Khin Thway1, Robin L. Jones1,3 and Charlotte Benson1*
Abstract Background: 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. Case presentation: 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. Secondline chemotherapy was commenced once the dermatomyositis was controlled on steroid therapy. Systemic anticancer therapy was again associated with mild improvement in dermatomyositis symptoms. Discussion: 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. Keywords: Dermatomyositis, Leiomyosarcoma, Soft tissue sarcoma, Chemotherapy, TIF1γ antibody, Paraneoplastic Introduction Dermatomyositis is a form of autoimmune inflammatory myopathy, with characteristic cutaneous features and myositis-related weakness [1]. Stertz first described the
*Correspondence: [email protected] 1 Sarcoma Unit, The Royal Marsden Hospital NHS Foundation Trust, 203 Fulham Road, London SW3 6JJ, UK Full list of author information is available at the end of the article
association between dermatomyositis and visceral malignancy in 1916, in a patient with gastric carcinoma [2]. The underlying mechanism of paraneoplastic dermatomyositis remains incompletely understood. A leading hypoth
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