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Paraneoplastic dermatomyositis associated with metastatic leiomyosarcoma of unknown primary

Paraneoplastic dermatomyositis associated with metastatic leiomyosarcoma of unknown primary 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 pre- sented 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. 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 para- neoplastic 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 association between dermatomyositis and visceral malig- Dermatomyositis is a form of autoimmune inflammatory nancy in 1916, in a patient with gastric carcinoma [2]. myopathy, with characteristic cutaneous features and The underlying mechanism of paraneoplastic dermato - myositis-related weakness [1]. Stertz first described the myositis remains incompletely understood. A leading hypothesis is that there is cross-reactivity with tumour- directed autoantibodies attacking similar autoantigens within muscle and skin cells [3]. Paraneoplastic dermato- *Correspondence: charlotte.benson@rmh.nhs.uk Sarcoma Unit, The Royal Marsden Hospital NHS Foundation Trust, 203 myositis has been most commonly observed in ovarian, Fulham Road, London SW3 6JJ, UK lung, pancreatic, stomach and colorectal cancers [4, 5]. Full list of author information is available at the end of the article © The Author(s) 2020. This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creat iveco mmons .org/licen ses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creat iveco mmons .org/publi cdoma in/ zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. Merry et al. Clin Sarcoma Res (2020) 10:15 Page 2 of 7 Sarcomas are rare and heterogeneous cancers with over shawl-like distribution (upper chest, neck and arms), 100 different histologic subtypes [6] and are uncom - with flagellate erythema (Fig.  1) and associated perior- monly associated with paraneoplastic syndromes. A bital oedema (Fig.  2) and proximal limb weakness. The small number of cases of paraneoplastic dermatomyositis patient denied breathing or swallowing difficulty. On have been described in chondrosarcoma [7–10] and one examination periungual erythema and Gottron’s pap- case in a soft tissue neoplasm characterised only as ‘low- ules were noted, without visible dilated capillary nailfold grade mesenchymal neoplasm’ [11]. To our knowledge, loops. There was reduced proximal power in both upper the association between paraneoplastic dermatomyosi- and lower limb (3–4/5), but no neck or truncal weak- tis and leiomyosarcoma has not been reported. Here, we ness. Vital signs found she was tachycardic and febrile. report a case of a patient presenting with paraneoplastic Routine blood tests on admission showed c-reactive pro- dermatomyositis associated with metastatic leiomyosar- tein < 4 and mildly raised white cell count 11.4 (normal coma of unknown primary. range 4–11 × 10 L). Alanine aminotransferase (ALT) was mildly raised at 61 (normal 10–49 U/L) with unremark- able renal function. Blood cultures grew Staphylococcus Case report hominis and she was treated with intravenous antibiotics A 43-year-old, previously healthy Caucasian female pre- and appropriate medical management. sented to her general practitioner with a few-months his- Rheumatology and dermatology review led to the tory of left knee pain. She had also noticed a left-sided diagnosis of dermatomyositis, likely paraneoplastic, supraclavicular fossa (SCF) mass. She had no significant in the context of known metastatic leiomyosarcoma. medical history, never smoked, with moderate alcohol Investigation supporting this included; raised creati- intake. A maternal grandmother had endometrial cancer nine kinase (CK) and erythrocyte sediment rate at aged 65 years. 695 (normal range 25–200 U/L) and 32 (normal range An ultrasound-guided core biopsy of the suspicious SCF mass was urgently arranged. Review by an expert soft tissue pathologist revealed grade 2 leiomyosarcoma, with immunohistochemistry positive for desmin, smooth muscle actin and h-caldesmon. CT and PET-CT imaging showed extensive metastatic disease, with no clear pri- mary site. There were widespread cutaneous, subcutane - ous, soft tissue, lung, liver, right adrenal and peritoneal metastases. A lytic lesion involving the left femoral head was identified as at risk for pathological fracture and a likely cause of the patient’s longstanding left knee pain. She initially underwent urgent prophylactic stabilisation of the left femur, followed by radiotherapy. She commenced first-line palliative doxorubicin chem - otherapy. The patient was noted to have a mild erythe - matous maculopapular rash on her face and upper chest in sun-exposed areas at her pre-treatment consultation. This developed several days following femoral surgery and persisted for a few weeks. No drug, environmental or infective triggers were identified. The rash was treated with topical hydrocortisone 1% and emollient cream (diprobase), and resolved during cycle one of doxorubicin with a sustained remission throughout chemotherapy. She did not report any muscle weakness or pain during this time. Six cycles of doxorubicin were well tolerated, with one episode of febrile neutropenia requiring a 25% dose reduction. Imaging assessment at the end of chem- otherapy showed overall stable metastatic disease by RECIST 1.1 criteria [12], with a minor reduction in some Fig. 1 Clinical features of dermatomyositis with shawl-like rash metastatic deposits noted mid-treatment. affecting neck, chest and back. a Flagellate erythema upper back. b Two months later, the patient presented acutely ‘V’ neck distribution of erythema unwell to her local hospital with a new-onset rash in a M erry et al. Clin Sarcoma Res (2020) 10:15 Page 3 of 7 was not performed, given the potential risk of meta- static disease seeding. Treatment was commenced with a weaning course of prednisolone (initial dose 40  mg daily) and topi- cal clobetasol 0.05% with clinical improvement in both weakness and rash over several months. Diagnosis of paraneoplastic dermatomyositis triggered re-evaluation of the patient’s leiomyosarcoma with CT imaging, which showed multifocal progression. Second-line chemother- apy with trabectedin was commenced following recovery from the acute admission and once the dermatomyositis was controlled with steroid therapy. Systemic anti-cancer therapy was again associated with mild improvement in dermatomyositis symptoms. Discussion Paraneoplastic dermatomyositis was first described in 1916 and represents approximately 30% of dermatomy- ositis cases [2, 4].Population-based studies have sup- ported an increased risk of cancer in dermatomyositis patients, with standardised incidence ratios for cancer Fig. 2 Clinical features of dermatomyositis including facial heliotrope between 3.0 and 7.7 [4, 5, 13, 14]. Strongest associations rash and periorbital oedema have been seen with ovarian, lung, pancreatic, stomach and colorectal cancers [4, 5], and tumours with high prevalence in specific populations, namely nasopharyn - geal carcinoma in a southeast Asian population [13]. 0–27  mm/h) respectively. Autoantibody screen nega- Paraneoplastic dermatomyositis has been seen with tive for ENA, dsDNA, anti-CCP, with normal comple- chondrosarcoma (4 cases) [7–10] and an unspecified soft ment levels and weakly positive ANA (1:160). Myositis tissue sarcoma (1 case) [11]. Like other paraneoplastic antibody panel was positive for transcription interme- phenomena, the pathophysiology of paraneoplastic der- diary factor-1 gamma (TIF1γ) antibodies alone, whilst matomyositis is thought to relate to autoimmune cross- anti-Jo1 and anti-Mi2 antibody negative. In addition to reactivity between similar autoantigens within the cancer intramuscular metastatic disease, MRI femur showed tissue and normal tissue in muscle and skin. mild increased signal within the muscles compatible The diagnosis of paraneoplastic dermatomyositis is with myositis. An echocardiogram confirmed normal supported by both the clinical and serological features cardiac function. Electromyography (EMG) found myo- found in this case. Bohan and Peter proposed five diag - pathic changes of moderate degree in lower and upper nostic criteria (Table 1) for dermatomyositis in 1975 [15, limbs, worse in the lower limbs with proximal predomi- 16]. Our patient displayed four out of five of these criteria nance. The findings were in keeping with a diagnosis of with the absent criterion being a positive muscle biopsy, proximal myopathy. Right chest punch biopsy histology which was performed due to high risk for metastatic was consistent with dermatomyositis. Muscle biopsy Table 1 Bohan and Peter’s proposed diagnostic criteria for dermatomyositis Criterion I Symmetrical proximal muscle weakness ± dysphagia or respiratory muscle involvement II Increase of skeletal muscle enzymes; creatine kinase, aspartate aminotransferase, alanine aminotransferase and lactate dehydrogenase III Abnormal EMG characteristic of myopathy IV Abnormal muscle biopsy V Typical cutaneous features (e.g. Gottron’s sign, heliotrope rash with periorbital oedema) Definite diagnosis: Criterion V and at least 3 of criteria I–IV Probable diagnosis: Criterion V and 2 of criteria I–IV Possible diagnosis: Criterion V and 1 of criteria I–IV Merry et al. Clin Sarcoma Res (2020) 10:15 Page 4 of 7 spread. MRI did show signal changes consistent with muscle weakness, increase of skeletal muscle enzymes myositis (Fig. 3). MRI imaging has been used for diagno- (namely ALT and CK), abnormal EMG and typical skin sis and assessment of treatment response for inflamma - rash, for a definite diagnosis as seen in this case [15, 16, tory myositis [17]. The results of MRI imaging coupled 18, 19]. In addition, this patient also meets the definite with the patient’s clinical presentation fit diagnostic cri - criteria of the Paraneoplastic Neurological Syndrome teria without the need for biopsy. Euronetwork for paraneoplastic neurological syndrome, The Bohan and Peter diagnostic criteria requires pres - with dermatomyositis as the classical syndrome and a ence of characteristic skin manifestations alongside three cancer developing within 5  years of dermatomyositis out of the four remaining criteria, symmetrical proximal diagnosis [20]. While the Bohan and Peter classification Fig. 3 MRI signal change of the musculature of the lower limbs on (a) left sagittal (b) axial (c) coronal inversion recovery sequence in keeping with myositis M erry et al. Clin Sarcoma Res (2020) 10:15 Page 5 of 7 remains widely used for the diagnosis of dermatomyositis contrast, surgical excision of a humeral chondrosarcoma [21], updated diagnostic criteria combining clinical fea- did not lead to complete resolution of dermatomyositis tures and serological testing, including myositis-specific but control of the cutaneous features improved [10]. In a and myositis-associated antibodies have been proposed case of paraneoplastic dermatomyositis related to a cav- [21, 22]. The goal of updates to the classification is to ernous sinus chondrosarcoma, complete surgical excision ensure robust, reliable criteria for clinical trial enrolment was not possible and the skin lesions persisted despite and understand the distinct clinical behaviour of differ - appropriate steroid and immunosuppressive therapy [30]. ent inflammatory myopathies. However, recent EULAR/ These limited cases suggest that even with optimal medi - ACR consensus criteria lack incorporation of MRI find - cal treatment for dermatomyositis, in patients with unre- ings and non anti-Jo1 antibodies [23], leaving room for sectable or metastatic disease, dermatomyositis does not improvement of this contemporary diagnostic criteria. fully resolve. In our case, serological results support paraneoplastic The severity of the skin lesions appeared to wax and dermatomyositis, with an isolated TIF1γ antibody posi- wane, with the metastatic disease suggesting a temporal tive test. The presence of the TIF1γ antibody indicates relationship between cutaneous features and leiomyo- increased risk of cancer-associated dermatomyositis sarcoma disease status. In patients with paraneoplastic with high specificity and moderate sensitivity [24, 25]. dermatomyositis and advanced cancer, similar temporal A meta-analysis assessed the clinical value of a posi- relationships have been described [31, 32]. The devel - tive TIF1γ antibody test to predict cancer-associated opment of erythema in the ‘V’ neck distribution, in dermatomyositis, and concluded a positive predictive hindsight, may have been the first early sign of dermato - value of 58%, and a negative predictive value of 95% [26]. myositis and mild progression of metastatic disease. The TIF1γ antibody-positive dermatomyositis and cancer has subsequent severe, acute presentation with severe der- been more commonly seen in older patients (age over matomyositis, complicated by septicaemia, was a dra- 39 years). It has a different phenotypic expression in chil - matic, and alarming red flag for cancer progression, as dren or young adults, with skin ulceration and chronic confirmed on imaging. Severe dermatomyositis compli - disease, without a cancer association [27, 28]. The only cated by septicaemia was also seen in uterine carcino- other reported association between TIF1γ antibody-pos- sarcoma and highlights the potential seriousness of this itive dermatomyositis and sarcoma has been described in paraneoplastic condition if untreated [33]. Now that this a Caucasian woman of similar age with chondrosarcoma pattern has been identified, the clinical severity of der - of the humerus [10]. Importantly, the absence of other matomyositis may predict response to anti-cancer ther- myositis antibodies in this patient, including anti-Jo1 and apy for metastatic leiomyosarcoma. Unfortunately, given anti-Mi2, is also supportive of paraneoplastic dermato- the metastatic nature of the leiomyosarcoma, treatment myositis [25]. In a cross-sectional study of UK Caucasian intent is palliative and it is unlikely that the dermatomy- adults with dermatomyositis, patients without myositis- ositis will resolve completely. However, good control has specific autoantibodies, like anti-Jo1 and anti-Mi2, had been achieved with steroid therapy without the need for a significantly increased risk of cancer-associated der - alternative immunosuppressive therapy. Understanding matomyositis [25]. They concluded that a negative rou - the relationship between the dual pathologies alongside tine myositis antibody panel is highly sensitive for cancer multidisciplinary management between rheumatology associated dermatomyositis. and oncology teams is key to providing optimal treat- The diagnosis of cancer has been reported to occur ment for paraneoplastic dermatomyositis. before, at the same time or after dermatomyositis onset [14, 29]. Most cancers are detected within the first year Conclusion of diagnosis of dermatomyositis and the risk remains This is, to our knowledge, the first description of paraneo - high for up to 5 years [25]. A cohort study focusing spe- plastic dermatomyositis associated with leiomyosarcoma. cifically on TIF1γ antibody-positive dermatomyositis Given leiomyosarcoma is a cancer of smooth muscle, it patients found that malignancy occurred between 3 years seems feasible that like other tumour types, the tumour prior and 2.5 years after dermatomyositis diagnosis [29]. cells may harbour autoantigens that mimic antigen within In this case the diagnosis of metastatic leiomyosarcoma regenerating normal muscle allowing for autoimmune of unknown primary preceded the TIF1γ-positive der- cross-reactivity and resultant myositis [3]. The temporal matomyositis diagnosis by several months. Resolution of relationship between dermatomyositis and cancer diagno- dermatomyositis has been variably associated with cura- sis varies. Cancer risk is significantly elevated in patients tive-intent surgical excision and optimal immunosup- with dermatomyositis, with the risk for cancer diagno- pressive treatment [11]. Symptoms have been reported to sis highest in the first year [13]. Given the poor prognosis resolve in a case of resected soft tissue sarcoma [11]. In associated with metastatic leiomyosarcoma, and soft tissue Merry et al. Clin Sarcoma Res (2020) 10:15 Page 6 of 7 2. G S. Polymyositis Berl Klin Wochenschr. 1916;53:489. sarcomas in general, it is important for physicians to be 3. Casciola-Rosen L, Nagaraju K, Plotz P, Wang K, Levine S, Gabrielson E, aware in the work-up of dermatomyositis, that there may et al. Enhanced autoantigen expression in regenerating muscle cells in be an underlying rare cancer. Early diagnosis of a sarcoma idiopathic inflammatory myopathy. J Exp Med. 2005;201(4):591–601. 4. Hill CL, Zhang Y, Sigurgeirsson B, Pukkala E, Mellemkjaer L, Airio A, et al. may provide the opportunity for curative surgical resec- Frequency of specific cancer types in dermatomyositis and polymyositis: tion, and possible resolution of the dermatomyositis that a population-based study. Lancet. 2001;357(9250):96–100. heralded their disease. This case emphasises that multi-dis - 5. Stockton D, Doherty V, Brewster D. Risk of cancer in patients with dermatomyositis or polymyositis, and follow-up implications: a Scottish ciplinary management is key for patients with paraneoplas- population-based cohort study. Br J Cancer. 2001;85(1):41–5. tic dermatomyositis. 6. Brennan MF, Antonescu CR, Maki RG. Management of soft tissue sarcoma: Springer; 2013. 7. Baker MC, Smith GP, Miloslavsky EM. Dermatomyositis associated with a Abbreviations skull base chondrosarcoma. J Clin Rheumatol. 2019;25(4):50. SCF: Supraclavicular fossa; US: Ultrasound; CT: Computerised Tomography; PET: 8. Patel SJ, Sanjana NE, Kishton RJ, Eidizadeh A, Vodnala SK, Cam M, et al. Positron emission tomography; FDG: Fluorodeoxyglucose; RECIST: Response Identification of essential genes for cancer immunotherapy. Nature. Evaluation Criteria in Solid Tumours; CRP: C-reactive protein; ALT: Alanine 2017;548(7669):537. Aminotransferase; CK: Creatine kinase; ESR: Erythrocyte Sedimentation Rate; 9. Mol MT, Stalenhoef A, Boerbooms AT. Chondrosarcoma coexistent with ENA: Extractable nuclear antigen; dsDNA: Double-stranded Deoxyribonucleic dermatomyositis. J Am College Rheumatol. 1986;29(6):813–4. acid; CCP: Cyclic citrullinated peptides; ANA: Antinuclear antibodies; TIF1γ: 10. Dziwis J, Agnihothri R, Lieberman A, Richardson CT. A unique case of der- Transcription intermediary factor-1 gamma; MRI: Magnetic resonance imaging; matomyositis associated with anti-TIF1γ antibody and chondrosarcoma. EMG: Electromyography; UK: United Kingdom; EULAR/ACR : European League JAAD case Rep. 2019;5(9):828–30. Against Rheumatism/American College of Rheumatology. 11. Ali M, Sendur N, Aksoy S, Yaman S, Arik Z, Kilinç L, et al. Dermatomyositis complicated with a soft tissue sarcoma. Rheumatol Int. 2012;32(10):3329. Acknowledgements 12. Eisenhauer EA, Therasse P, Bogaerts J, Schwartz LH, Sargent D, Ford R, Dr Annabel Scott, Dermatology Consultant, Ashford and St Peter’s Hospitals et al. New response evaluation criteria in solid tumours: revised RECIST NHS Trust for collaborative care and provision of medical photography and Dr guideline. Eur J Cancer. 2009;45(2):228–47. Siraj Yusuf from The Royal Marsden Hospital NHS Trust for assistance with MRI 13. Chen Y-J, Wu C-Y, Huang Y-L, Wang C-B, Shen J-L, Chang Y-T. Cancer risks images. of dermatomyositis and polymyositis: a nationwide cohort study in Taiwan. Arthritis Res Ther. 2010;12(2):R70. Authors’ contributions 14. Sigurgeirsson B, Lindelöf B, Edhag O, Allander E. Risk of cancer in patients Conceived design–EM, AS, RLJ, CB, Collected data–EM, KS, GP, KT, Analysis–All with dermatomyositis or polymyositis. N Engl J Med. 1992;326(6):363–7. authors, Writing of manuscript–EM, AS, CB, RJ, Review of Manuscript–All 15. Bohan A, Peter J. 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Cutaneous manifestations in dermatomy- ositis: key clinical and serological features—a comprehensive review. Clin Ethics approval and consent to participate Rev Allergy Immunol. 2016;51(3):293–302. Not applicable. 20. Graus F, Delattre J, Antoine J, Dalmau J, Giometto B, Grisold W, et al. Recommended diagnostic criteria for paraneoplastic neurological syn- Consent for publication dromes. J Neurol Neurosurg Psychiatry. 2004;75(8):1135–40. Consent obtained from patient for publication of this case report including 21. Leclair V, Lundberg IE. New Myositis Classification Criteria—What We images. Have Learned Since Bohan and Peter. Curr Rheumatol Rep. 2018;20(4):18. 22. Benveniste O, Stenzel W, Allenbach Y. Advances in serological diagnostics Competing interests of inflammatory myopathies. Curr Opin Neurol. 2016;29(5):662–73. RLJ is the recipient of grants/research support from MSD, GSK. RLJ is the recipi- 23. 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The of myositis autoantibody testing for predicting the risk of cancer-associ- Institute of Cancer Research, 237 Fulham Road, London SW3 6JB, UK. ated myositis. Ann Rheum Dis. 2007;66(10):1345–9. 26. Trallero-Araguás E, Rodrigo-Pendás JÁ, Selva-O’Callaghan A, Martínez- Received: 17 July 2020 Accepted: 17 August 2020 Gómez X, Bosch X, Labrador-Horrillo M, et al. Usefulness of anti-p155 autoantibody for diagnosing cancer-associated dermatomyositis: a systematic review and meta-analysis. Arthritis Rheum. 2012;64(2):523–32. 27. Waller R, Ahmad N. TIF1-γ associated dermatomyositis. Rheumatol Adv Practice. 2019;3(1):030. References 28. Fiorentino D, Casciola-Rosen L. Autoantibodies to transcription interme- 1. Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet. diary factor 1 in dermatomyositis shed insight into the cancer-myositis 2003;362(9388):971–82. M erry et al. Clin Sarcoma Res (2020) 10:15 Page 7 of 7 connection. Arthritis Rheum. 2012;64(2):346–9. https ://doi.org/10.1002/ 32. Ono K, Shimomura M, Toyota K, Kagimoto A, Tsukiyama N, Shishida M, art.33402 . et al. Successful resection of liver metastasis detected by exacerbation of 29. Oldroyd A, Sergeant JC, New P, McHugh NJ, Betteridge Z, Lamb JA, skin symptom in a patient with dermatomyositis accompanied by rectal et al. The temporal relationship between cancer and adult onset anti- cancer: a case report and literature review. Surgical Case Rep. 2017;3(1):3. transcriptional intermediary factor 1 antibody–positive dermatomyositis. 33. Chandiramani M, Joynson C, Panchal R, Symonds R, Brown L, Morgan B, Rheumatology. 2019;58(4):650–5. et al. Dermatomyositis as a paraneoplastic syndrome in carcinosarcoma 30. Patel MM, Stacy RC. Paraneoplastic dermatomyositis related to a of uterine origin. Clin Oncol. 2006;18(9):641–8. chondrosarcoma involving the cavernous sinus. J Neuroophthalmol. 2013;33(4):363–6. Publisher’s Note 31. Nagano Y, Inoue Y, Shimura T, Fujikawa H, Okugawa Y, Hiro J, et al. Exacer- Springer Nature remains neutral with regard to jurisdictional claims in pub- bation of dermatomyositis with recurrence of rectal cancer: a case report. lished maps and institutional affiliations. Case Rep Oncol. 2015;8(3):482–6. Ready to submit your research ? Choose BMC and benefit from: fast, convenient online submission thorough peer review by experienced researchers in your field rapid publication on acceptance support for research data, including large and complex data types • gold Open Access which fosters wider collaboration and increased citations maximum visibility for your research: over 100M website views per year At BMC, research is always in progress. Learn more biomedcentral.com/submissions http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Clinical Sarcoma Research Springer Journals

Paraneoplastic dermatomyositis associated with metastatic leiomyosarcoma of unknown primary

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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 pre- sented 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. 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 para- neoplastic 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 association between dermatomyositis and visceral malig- Dermatomyositis is a form of autoimmune inflammatory nancy in 1916, in a patient with gastric carcinoma [2]. myopathy, with characteristic cutaneous features and The underlying mechanism of paraneoplastic dermato - myositis-related weakness [1]. Stertz first described the myositis remains incompletely understood. A leading hypothesis is that there is cross-reactivity with tumour- directed autoantibodies attacking similar autoantigens within muscle and skin cells [3]. Paraneoplastic dermato- *Correspondence: charlotte.benson@rmh.nhs.uk Sarcoma Unit, The Royal Marsden Hospital NHS Foundation Trust, 203 myositis has been most commonly observed in ovarian, Fulham Road, London SW3 6JJ, UK lung, pancreatic, stomach and colorectal cancers [4, 5]. Full list of author information is available at the end of the article © The Author(s) 2020. This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creat iveco mmons .org/licen ses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creat iveco mmons .org/publi cdoma in/ zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. Merry et al. Clin Sarcoma Res (2020) 10:15 Page 2 of 7 Sarcomas are rare and heterogeneous cancers with over shawl-like distribution (upper chest, neck and arms), 100 different histologic subtypes [6] and are uncom - with flagellate erythema (Fig.  1) and associated perior- monly associated with paraneoplastic syndromes. A bital oedema (Fig.  2) and proximal limb weakness. The small number of cases of paraneoplastic dermatomyositis patient denied breathing or swallowing difficulty. On have been described in chondrosarcoma [7–10] and one examination periungual erythema and Gottron’s pap- case in a soft tissue neoplasm characterised only as ‘low- ules were noted, without visible dilated capillary nailfold grade mesenchymal neoplasm’ [11]. To our knowledge, loops. There was reduced proximal power in both upper the association between paraneoplastic dermatomyosi- and lower limb (3–4/5), but no neck or truncal weak- tis and leiomyosarcoma has not been reported. Here, we ness. Vital signs found she was tachycardic and febrile. report a case of a patient presenting with paraneoplastic Routine blood tests on admission showed c-reactive pro- dermatomyositis associated with metastatic leiomyosar- tein < 4 and mildly raised white cell count 11.4 (normal coma of unknown primary. range 4–11 × 10 L). Alanine aminotransferase (ALT) was mildly raised at 61 (normal 10–49 U/L) with unremark- able renal function. Blood cultures grew Staphylococcus Case report hominis and she was treated with intravenous antibiotics A 43-year-old, previously healthy Caucasian female pre- and appropriate medical management. sented to her general practitioner with a few-months his- Rheumatology and dermatology review led to the tory of left knee pain. She had also noticed a left-sided diagnosis of dermatomyositis, likely paraneoplastic, supraclavicular fossa (SCF) mass. She had no significant in the context of known metastatic leiomyosarcoma. medical history, never smoked, with moderate alcohol Investigation supporting this included; raised creati- intake. A maternal grandmother had endometrial cancer nine kinase (CK) and erythrocyte sediment rate at aged 65 years. 695 (normal range 25–200 U/L) and 32 (normal range An ultrasound-guided core biopsy of the suspicious SCF mass was urgently arranged. Review by an expert soft tissue pathologist revealed grade 2 leiomyosarcoma, with immunohistochemistry positive for desmin, smooth muscle actin and h-caldesmon. CT and PET-CT imaging showed extensive metastatic disease, with no clear pri- mary site. There were widespread cutaneous, subcutane - ous, soft tissue, lung, liver, right adrenal and peritoneal metastases. A lytic lesion involving the left femoral head was identified as at risk for pathological fracture and a likely cause of the patient’s longstanding left knee pain. She initially underwent urgent prophylactic stabilisation of the left femur, followed by radiotherapy. She commenced first-line palliative doxorubicin chem - otherapy. The patient was noted to have a mild erythe - matous maculopapular rash on her face and upper chest in sun-exposed areas at her pre-treatment consultation. This developed several days following femoral surgery and persisted for a few weeks. No drug, environmental or infective triggers were identified. The rash was treated with topical hydrocortisone 1% and emollient cream (diprobase), and resolved during cycle one of doxorubicin with a sustained remission throughout chemotherapy. She did not report any muscle weakness or pain during this time. Six cycles of doxorubicin were well tolerated, with one episode of febrile neutropenia requiring a 25% dose reduction. Imaging assessment at the end of chem- otherapy showed overall stable metastatic disease by RECIST 1.1 criteria [12], with a minor reduction in some Fig. 1 Clinical features of dermatomyositis with shawl-like rash metastatic deposits noted mid-treatment. affecting neck, chest and back. a Flagellate erythema upper back. b Two months later, the patient presented acutely ‘V’ neck distribution of erythema unwell to her local hospital with a new-onset rash in a M erry et al. Clin Sarcoma Res (2020) 10:15 Page 3 of 7 was not performed, given the potential risk of meta- static disease seeding. Treatment was commenced with a weaning course of prednisolone (initial dose 40  mg daily) and topi- cal clobetasol 0.05% with clinical improvement in both weakness and rash over several months. Diagnosis of paraneoplastic dermatomyositis triggered re-evaluation of the patient’s leiomyosarcoma with CT imaging, which showed multifocal progression. Second-line chemother- apy with trabectedin was commenced following recovery from the acute admission and once the dermatomyositis was controlled with steroid therapy. Systemic anti-cancer therapy was again associated with mild improvement in dermatomyositis symptoms. Discussion Paraneoplastic dermatomyositis was first described in 1916 and represents approximately 30% of dermatomy- ositis cases [2, 4].Population-based studies have sup- ported an increased risk of cancer in dermatomyositis patients, with standardised incidence ratios for cancer Fig. 2 Clinical features of dermatomyositis including facial heliotrope between 3.0 and 7.7 [4, 5, 13, 14]. Strongest associations rash and periorbital oedema have been seen with ovarian, lung, pancreatic, stomach and colorectal cancers [4, 5], and tumours with high prevalence in specific populations, namely nasopharyn - geal carcinoma in a southeast Asian population [13]. 0–27  mm/h) respectively. Autoantibody screen nega- Paraneoplastic dermatomyositis has been seen with tive for ENA, dsDNA, anti-CCP, with normal comple- chondrosarcoma (4 cases) [7–10] and an unspecified soft ment levels and weakly positive ANA (1:160). Myositis tissue sarcoma (1 case) [11]. Like other paraneoplastic antibody panel was positive for transcription interme- phenomena, the pathophysiology of paraneoplastic der- diary factor-1 gamma (TIF1γ) antibodies alone, whilst matomyositis is thought to relate to autoimmune cross- anti-Jo1 and anti-Mi2 antibody negative. In addition to reactivity between similar autoantigens within the cancer intramuscular metastatic disease, MRI femur showed tissue and normal tissue in muscle and skin. mild increased signal within the muscles compatible The diagnosis of paraneoplastic dermatomyositis is with myositis. An echocardiogram confirmed normal supported by both the clinical and serological features cardiac function. Electromyography (EMG) found myo- found in this case. Bohan and Peter proposed five diag - pathic changes of moderate degree in lower and upper nostic criteria (Table 1) for dermatomyositis in 1975 [15, limbs, worse in the lower limbs with proximal predomi- 16]. Our patient displayed four out of five of these criteria nance. The findings were in keeping with a diagnosis of with the absent criterion being a positive muscle biopsy, proximal myopathy. Right chest punch biopsy histology which was performed due to high risk for metastatic was consistent with dermatomyositis. Muscle biopsy Table 1 Bohan and Peter’s proposed diagnostic criteria for dermatomyositis Criterion I Symmetrical proximal muscle weakness ± dysphagia or respiratory muscle involvement II Increase of skeletal muscle enzymes; creatine kinase, aspartate aminotransferase, alanine aminotransferase and lactate dehydrogenase III Abnormal EMG characteristic of myopathy IV Abnormal muscle biopsy V Typical cutaneous features (e.g. Gottron’s sign, heliotrope rash with periorbital oedema) Definite diagnosis: Criterion V and at least 3 of criteria I–IV Probable diagnosis: Criterion V and 2 of criteria I–IV Possible diagnosis: Criterion V and 1 of criteria I–IV Merry et al. Clin Sarcoma Res (2020) 10:15 Page 4 of 7 spread. MRI did show signal changes consistent with muscle weakness, increase of skeletal muscle enzymes myositis (Fig. 3). MRI imaging has been used for diagno- (namely ALT and CK), abnormal EMG and typical skin sis and assessment of treatment response for inflamma - rash, for a definite diagnosis as seen in this case [15, 16, tory myositis [17]. The results of MRI imaging coupled 18, 19]. In addition, this patient also meets the definite with the patient’s clinical presentation fit diagnostic cri - criteria of the Paraneoplastic Neurological Syndrome teria without the need for biopsy. Euronetwork for paraneoplastic neurological syndrome, The Bohan and Peter diagnostic criteria requires pres - with dermatomyositis as the classical syndrome and a ence of characteristic skin manifestations alongside three cancer developing within 5  years of dermatomyositis out of the four remaining criteria, symmetrical proximal diagnosis [20]. While the Bohan and Peter classification Fig. 3 MRI signal change of the musculature of the lower limbs on (a) left sagittal (b) axial (c) coronal inversion recovery sequence in keeping with myositis M erry et al. Clin Sarcoma Res (2020) 10:15 Page 5 of 7 remains widely used for the diagnosis of dermatomyositis contrast, surgical excision of a humeral chondrosarcoma [21], updated diagnostic criteria combining clinical fea- did not lead to complete resolution of dermatomyositis tures and serological testing, including myositis-specific but control of the cutaneous features improved [10]. In a and myositis-associated antibodies have been proposed case of paraneoplastic dermatomyositis related to a cav- [21, 22]. The goal of updates to the classification is to ernous sinus chondrosarcoma, complete surgical excision ensure robust, reliable criteria for clinical trial enrolment was not possible and the skin lesions persisted despite and understand the distinct clinical behaviour of differ - appropriate steroid and immunosuppressive therapy [30]. ent inflammatory myopathies. However, recent EULAR/ These limited cases suggest that even with optimal medi - ACR consensus criteria lack incorporation of MRI find - cal treatment for dermatomyositis, in patients with unre- ings and non anti-Jo1 antibodies [23], leaving room for sectable or metastatic disease, dermatomyositis does not improvement of this contemporary diagnostic criteria. fully resolve. In our case, serological results support paraneoplastic The severity of the skin lesions appeared to wax and dermatomyositis, with an isolated TIF1γ antibody posi- wane, with the metastatic disease suggesting a temporal tive test. The presence of the TIF1γ antibody indicates relationship between cutaneous features and leiomyo- increased risk of cancer-associated dermatomyositis sarcoma disease status. In patients with paraneoplastic with high specificity and moderate sensitivity [24, 25]. dermatomyositis and advanced cancer, similar temporal A meta-analysis assessed the clinical value of a posi- relationships have been described [31, 32]. The devel - tive TIF1γ antibody test to predict cancer-associated opment of erythema in the ‘V’ neck distribution, in dermatomyositis, and concluded a positive predictive hindsight, may have been the first early sign of dermato - value of 58%, and a negative predictive value of 95% [26]. myositis and mild progression of metastatic disease. The TIF1γ antibody-positive dermatomyositis and cancer has subsequent severe, acute presentation with severe der- been more commonly seen in older patients (age over matomyositis, complicated by septicaemia, was a dra- 39 years). It has a different phenotypic expression in chil - matic, and alarming red flag for cancer progression, as dren or young adults, with skin ulceration and chronic confirmed on imaging. Severe dermatomyositis compli - disease, without a cancer association [27, 28]. The only cated by septicaemia was also seen in uterine carcino- other reported association between TIF1γ antibody-pos- sarcoma and highlights the potential seriousness of this itive dermatomyositis and sarcoma has been described in paraneoplastic condition if untreated [33]. Now that this a Caucasian woman of similar age with chondrosarcoma pattern has been identified, the clinical severity of der - of the humerus [10]. Importantly, the absence of other matomyositis may predict response to anti-cancer ther- myositis antibodies in this patient, including anti-Jo1 and apy for metastatic leiomyosarcoma. Unfortunately, given anti-Mi2, is also supportive of paraneoplastic dermato- the metastatic nature of the leiomyosarcoma, treatment myositis [25]. In a cross-sectional study of UK Caucasian intent is palliative and it is unlikely that the dermatomy- adults with dermatomyositis, patients without myositis- ositis will resolve completely. However, good control has specific autoantibodies, like anti-Jo1 and anti-Mi2, had been achieved with steroid therapy without the need for a significantly increased risk of cancer-associated der - alternative immunosuppressive therapy. Understanding matomyositis [25]. They concluded that a negative rou - the relationship between the dual pathologies alongside tine myositis antibody panel is highly sensitive for cancer multidisciplinary management between rheumatology associated dermatomyositis. and oncology teams is key to providing optimal treat- The diagnosis of cancer has been reported to occur ment for paraneoplastic dermatomyositis. before, at the same time or after dermatomyositis onset [14, 29]. Most cancers are detected within the first year Conclusion of diagnosis of dermatomyositis and the risk remains This is, to our knowledge, the first description of paraneo - high for up to 5 years [25]. A cohort study focusing spe- plastic dermatomyositis associated with leiomyosarcoma. cifically on TIF1γ antibody-positive dermatomyositis Given leiomyosarcoma is a cancer of smooth muscle, it patients found that malignancy occurred between 3 years seems feasible that like other tumour types, the tumour prior and 2.5 years after dermatomyositis diagnosis [29]. cells may harbour autoantigens that mimic antigen within In this case the diagnosis of metastatic leiomyosarcoma regenerating normal muscle allowing for autoimmune of unknown primary preceded the TIF1γ-positive der- cross-reactivity and resultant myositis [3]. The temporal matomyositis diagnosis by several months. Resolution of relationship between dermatomyositis and cancer diagno- dermatomyositis has been variably associated with cura- sis varies. Cancer risk is significantly elevated in patients tive-intent surgical excision and optimal immunosup- with dermatomyositis, with the risk for cancer diagno- pressive treatment [11]. Symptoms have been reported to sis highest in the first year [13]. Given the poor prognosis resolve in a case of resected soft tissue sarcoma [11]. In associated with metastatic leiomyosarcoma, and soft tissue Merry et al. Clin Sarcoma Res (2020) 10:15 Page 6 of 7 2. G S. Polymyositis Berl Klin Wochenschr. 1916;53:489. sarcomas in general, it is important for physicians to be 3. Casciola-Rosen L, Nagaraju K, Plotz P, Wang K, Levine S, Gabrielson E, aware in the work-up of dermatomyositis, that there may et al. Enhanced autoantigen expression in regenerating muscle cells in be an underlying rare cancer. Early diagnosis of a sarcoma idiopathic inflammatory myopathy. J Exp Med. 2005;201(4):591–601. 4. Hill CL, Zhang Y, Sigurgeirsson B, Pukkala E, Mellemkjaer L, Airio A, et al. may provide the opportunity for curative surgical resec- Frequency of specific cancer types in dermatomyositis and polymyositis: tion, and possible resolution of the dermatomyositis that a population-based study. 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Nagano Y, Inoue Y, Shimura T, Fujikawa H, Okugawa Y, Hiro J, et al. Exacer- Springer Nature remains neutral with regard to jurisdictional claims in pub- bation of dermatomyositis with recurrence of rectal cancer: a case report. lished maps and institutional affiliations. Case Rep Oncol. 2015;8(3):482–6. Ready to submit your research ? Choose BMC and benefit from: fast, convenient online submission thorough peer review by experienced researchers in your field rapid publication on acceptance support for research data, including large and complex data types • gold Open Access which fosters wider collaboration and increased citations maximum visibility for your research: over 100M website views per year At BMC, research is always in progress. Learn more biomedcentral.com/submissions

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