{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T00:52:16Z","timestamp":1760057536602,"version":"build-2065373602"},"reference-count":61,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2025,2,14]],"date-time":"2025-02-14T00:00:00Z","timestamp":1739491200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Nasopharyngeal carcinoma (NPC) with paraneoplastic dermatomyositis (DM) is an exceptionally rare clinical phenomenon, particularly among European populations. This case report details a 46-year-old woman initially diagnosed with DM, later confirmed to have NPC. Such an association is more frequently documented in Asian populations, highlighting its unique presentation in this case. The patient first developed symptoms in December 2016, which progressed significantly by spring 2017, manifesting as progressive proximal muscle weakness, characteristic skin changes, and elevated muscle enzyme levels. Diagnostic workup, including electromyography and biopsy, confirmed DM. Persistent symptoms and secondary DM suspicion prompted further malignancy screening, which identified undifferentiated NPC with strong Epstein\u2013Barr virus RNA positivity. Multimodal treatment comprising corticosteroids, hydroxychloroquine, chemotherapy, and radiotherapy led to temporary symptomatic improvement. Despite initial success, the patient\u2019s condition deteriorated, and she passed away by the end of 2018. This case underscores the importance of comprehensive malignancy screening in DM patients, considering rarer cancers like NPC even in non-endemic regions. It emphasizes the role of multidisciplinary care and adherence to international guidelines for managing such complex cases. Recognizing NPC-associated DM remains critical for early intervention and tailored therapeutic approaches to improve clinical outcomes and survival.<\/jats:p>","DOI":"10.3390\/medicina61020334","type":"journal-article","created":{"date-parts":[[2025,2,14]],"date-time":"2025-02-14T03:20:52Z","timestamp":1739503252000},"page":"334","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Dermatomyositis as the First Manifestation of Nasopharyngeal Carcinoma\u2014A Rare Case Report"],"prefix":"10.3390","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-5477-7883","authenticated-orcid":false,"given":"Bojana","family":"Kne\u017eevi\u0107","sequence":"first","affiliation":[{"name":"Military Medical Academy, Faculty of Medicine, University of Defense, Crnotravska 17, 11000 Belgrade, Serbia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tatjana","family":"Radevi\u0107","sequence":"additional","affiliation":[{"name":"Military Medical Academy, Faculty of Medicine, University of Defense, Crnotravska 17, 11000 Belgrade, Serbia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Milan","family":"Petronijevi\u0107","sequence":"additional","affiliation":[{"name":"Military Medical Academy, Faculty of Medicine, University of Defense, Crnotravska 17, 11000 Belgrade, Serbia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,2,14]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2041","DOI":"10.1016\/S0140-6736(05)66698-6","article-title":"Nasopharyngeal carcinoma","volume":"365","author":"Wei","year":"2005","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"E. A. R., E.N.S., Irekeola, A.A., and Yean Yean, C. (2020). Diagnostic and Prognostic Indications of Nasopharyngeal Carcinoma. Diagnostics, 10.","DOI":"10.3390\/diagnostics10090611"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1012","DOI":"10.1016\/S0140-6736(15)00055-0","article-title":"Nasopharyngeal carcinoma","volume":"387","author":"Chua","year":"2016","journal-title":"Lancet"},{"key":"ref_4","unstructured":"EMA (2024, November 12). Committee for Orphan Medicinal Products (COMP). Available online: https:\/\/www.ema.europa.eu\/en\/committees\/committee-orphan-medicinal-products-comp#:~:text=In%20the%20European%20Union%20(EU,medicine%20based%20on%20COMP%27s%20opinion."},{"key":"ref_5","unstructured":"WHO (2024, November 12). WHO-Cancer Today. Available online: https:\/\/gco.iarc.fr\/today\/en\/dataviz\/tables?mode=population&cancers=4&populations=100_112_191_196_203_208_233_246_250_276_300_348_352_372_380_40_428_440_442_470_498_499_528_56_578_616_620_642_643_688_70_703_705_724_752_756_8_804_807_826&multiple_populations=1."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"394","DOI":"10.3322\/caac.21492","article-title":"Global cancer statistics 2018: GLOBOCAN estimates of incidence and mortality worldwide for 36 cancers in 185 countries","volume":"68","author":"Bray","year":"2018","journal-title":"CA Cancer J. Clin."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1016\/S0140-6736(19)30956-0","article-title":"Nasopharyngeal carcinoma","volume":"394","author":"Chen","year":"2019","journal-title":"Lancet"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"115","DOI":"10.3322\/caac.21338","article-title":"Cancer statistics in China 2015","volume":"66","author":"Chen","year":"2016","journal-title":"CA Cancer J. Clin."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"53","DOI":"10.4103\/0971-9903.178107","article-title":"Dermatomyositis: A case report","volume":"21","author":"Kalyan","year":"2016","journal-title":"J. Mahatma Gandhi Inst. Med. Sci."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"375","DOI":"10.4103\/0019-5154.100486","article-title":"Clinical presentation and evaluation of dermatomyositis","volume":"57","author":"Marvi","year":"2012","journal-title":"Indian J. Dermatol."},{"key":"ref_11","unstructured":"Bader-Meunier, B., and Benveniste, O. (2016, July 10). National Diagnostic and Care Protocol (PNDS) Child and Adult Dermatomyositis. Available online: www.has-sante.fr."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1016\/S0140-6736(00)03540-6","article-title":"Frequency of specific cancer types in dermatomyositis and polymyositis: A population-based study","volume":"357","author":"Hill","year":"2001","journal-title":"Lancet"},{"key":"ref_13","unstructured":"WHO (2024). Head and Neck Tumours: WHO Classification of Tumours, WHO. [5th ed.]. Available online: https:\/\/publications.iarc.fr\/Book-And-Report-Series\/Who-Classification-Of-Tumours\/Head-And-Neck-Tumours-2024."},{"key":"ref_14","first-page":"3687","article-title":"Nasopharyngeal carcinoma: A review of current updates","volume":"15","author":"Wu","year":"2018","journal-title":"Exp. Ther. Med."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1016\/j.canlet.2016.01.040","article-title":"Global trends in incidence and mortality of nasopharyngeal carcinoma","volume":"374","author":"Tang","year":"2016","journal-title":"Cancer Lett."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1056\/NEJM197502202920807","article-title":"Polymyositis and dermatomyositis (second of two parts)","volume":"292","author":"Bohan","year":"1975","journal-title":"N. Engl. J. Med."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1186\/s13256-016-0816-8","article-title":"A mild form of dermatomyositis as a prodromal sign of lung adenocarcinoma: A case report","volume":"10","author":"Papakonstantinou","year":"2016","journal-title":"J. Med. Case Rep."},{"key":"ref_18","first-page":"471","article-title":"The paraneoplastic syndrome in nasopharynx cancer. 32 cases","volume":"14","author":"Maalej","year":"1985","journal-title":"Presse Medicale"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1955","DOI":"10.1136\/annrheumdis-2017-211468","article-title":"2017 European League Against Rheumatism\/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups","volume":"76","author":"Lundberg","year":"2017","journal-title":"Ann. Rheum. Dis."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"452","DOI":"10.1016\/j.annonc.2020.12.007","article-title":"Nasopharyngeal carcinoma: ESMO-EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up","volume":"32","author":"Bossi","year":"2021","journal-title":"Ann. Oncol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1195","DOI":"10.1002\/cac2.12218","article-title":"The Chinese Society of Clinical Oncology (CSCO) clinical guidelines for the diagnosis and treatment of nasopharyngeal carcinoma","volume":"41","author":"Tang","year":"2021","journal-title":"Cancer Commun."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1016\/j.rpor.2014.02.005","article-title":"Nasopharyngeal carcinoma in dermatomyositis patients: A 10-year retrospective review in Hospital Selayang, Malaysia","volume":"19","author":"Teoh","year":"2014","journal-title":"Rep. Pract. Oncol. Radiother."},{"key":"ref_23","first-page":"29","article-title":"Paraneoplastic dermatomyositis revealing an undifferentiated nasopharyngeal carcinoma: About a case","volume":"24","author":"Ziani","year":"2016","journal-title":"Pan Afr. Med. J."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1016\/j.anl.2008.10.006","article-title":"Paraneoplastic syndromes in patients with nasopharyngeal cancer","volume":"36","author":"Toro","year":"2009","journal-title":"Auris Nasus Larynx"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"544","DOI":"10.1111\/j.1365-2133.1969.tb16031.x","article-title":"Dermatomyositis: A clinical investigation of twenty-three cases in Hong Kong","volume":"81","author":"Wong","year":"1969","journal-title":"Br. J. Dermatol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1111\/j.1365-4362.1985.tb05816.x","article-title":"Dermatomyositis and cancer in Singapore","volume":"24","author":"Chan","year":"1985","journal-title":"Int. J. Dermatol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1097\/00000421-199602000-00008","article-title":"Study of 45 cases of nasopharyngeal carcinoma with dermatomyositis","volume":"19","author":"Hu","year":"1996","journal-title":"Am. J. Clin. Oncol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"282","DOI":"10.3899\/jrheum.140566","article-title":"Polymyositis\/dermatomyositis and malignancy risk: A meta-analysis study","volume":"42","author":"Yang","year":"2015","journal-title":"J. Rheumatol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1212\/WNL.0000000000002863","article-title":"Anti-TIF1-\u03b3 antibody and cancer-associated myositis: A clinicohistopathologic study","volume":"87","author":"Hida","year":"2016","journal-title":"Neurology"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1007\/s40674-019-00128-8","article-title":"Cancer and Idiopathic Inflammatory Myositis","volume":"5","author":"Baig","year":"2019","journal-title":"Curr. Treat. Options Rheumatol."},{"key":"ref_31","doi-asserted-by":"crossref","unstructured":"Requena, C., S\u00e1nchez-Romero, E., Nagore, E., and Sanmart\u00edn, O. (2024). Paraneoplastic Dermatomyositis: A 25-Patient Series From a Cancer Center. Actas Dermosifiliogr., S0001-7310.","DOI":"10.1016\/j.ad.2025.02.012"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1016\/j.rmed.2016.07.009","article-title":"Clinical features and outcomes of interstitial lung disease in anti-Jo-1 positive antisynthetase syndrome","volume":"118","author":"Zamora","year":"2016","journal-title":"Respir. Med."},{"key":"ref_33","first-page":"67","article-title":"Anti-transcriptional intermediary factor 1 gamma antibodies in cancer-associated myositis: A longitudinal study","volume":"38","author":"Dani","year":"2020","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_34","first-page":"164","article-title":"Anti-TIF1-G-Paraneoplastic Dermatomyositis: A Novel Association with Mantle Cell Lymphoma","volume":"113","author":"Cheung","year":"2020","journal-title":"Ir. Med. J."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1309","DOI":"10.1007\/s00405-018-4956-x","article-title":"Prognostic values of hematological biomarkers in nasopharyngeal carcinoma patients treated with intensity-modulated radiotherapy","volume":"275","author":"Ye","year":"2018","journal-title":"Eur. Arch. Otorhinolaryngol."},{"key":"ref_36","doi-asserted-by":"crossref","unstructured":"Cocuzza, S., Parisi, F.M., Spatola, C., La Mantia, I., Lechien, J.R., Chiesa-Estomba, C., Ferlito, S., Albanese, G., Lentini, M., and Mayo-Yanez, M. (2024). Neutrophil-to-Lymphocyte and Platelet-to-Lymphocyte Ratios as Predictors of Dysphagia Severity and Quality of Life in Nasopharyngeal Cancer Patients after Intensity Modulated Radiotherapy (IMRT). J. Clin. Med., 13.","DOI":"10.3390\/jcm13164821"},{"key":"ref_37","first-page":"1004","article-title":"Remission of Dermatomyositis Following Antitumor Combination Therapy in EBV-Associated Nasopharyngeal Carcinoma","volume":"1","author":"Chen","year":"2024","journal-title":"J. Phys. Ther. Rehabil."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1016\/j.jcv.2010.08.015","article-title":"Polymyositis\/dermatomyositis and nasopharyngeal carcinoma: The Epstein-Barr virus connection?","volume":"49","author":"Chen","year":"2010","journal-title":"J. Clin. Virol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"e197","DOI":"10.1093\/rheumatology\/keaa832","article-title":"Anti-TIF1\u03b3 antibody-positive dermatomyositis in a 15-year-old boy with Epstein-Barr virus-related nasopharyngeal cancer receiving nivolumab","volume":"60","author":"Asano","year":"2021","journal-title":"Rheumatology"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"824","DOI":"10.3109\/00016489.2014.913312","article-title":"Paired study of 172 cases of nasopharyngeal carcinoma with or without dermatomyositis","volume":"134","author":"Huang","year":"2014","journal-title":"Acta Otolaryngol."},{"key":"ref_41","doi-asserted-by":"crossref","unstructured":"Irekeola, A.A., Shueb, R.H., E. A. R., E.N.S., Wada, Y., Abdul Rahman, Z., Ahmad, S., Mohamud, R., Mat Lazim, N., and Yean, C.Y. (2021). Prevalence of Nasopharyngeal Carcinoma in Patients with Dermatomyositis: A Systematic Review and Meta-Analysis. Cancers, 13.","DOI":"10.3390\/cancers13081886"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1765","DOI":"10.1158\/1055-9965.EPI-06-0353","article-title":"The enigmatic epidemiology of nasopharyngeal carcinoma","volume":"15","author":"Chang","year":"2006","journal-title":"Cancer Epidemiol. Biomark. Prev."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1001\/archotol.129.1.101","article-title":"Connective tissue disease coincident with nasopharyngeal carcinoma: Two sporadic cases in a Western population","volume":"129","author":"Ferris","year":"2003","journal-title":"Arch. Otolaryngol. Head Neck Surg."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"2704","DOI":"10.3899\/jrheum.090549","article-title":"Malignancy is associated with dermatomyositis but not polymyositis in Northern New England, USA","volume":"36","author":"Antiochos","year":"2009","journal-title":"J. Rheumatol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1054\/bjoc.2001.1699","article-title":"Risk of cancer in patients with dermatomyositis or polymyositis, and follow-up implications: A Scottish population-based cohort study","volume":"85","author":"Stockton","year":"2001","journal-title":"Br. J. Cancer"},{"key":"ref_46","first-page":"48","article-title":"Rhinopharyngeal carcinoma and dermatomyositis: Description of a clinical case","volume":"54","author":"Botsios","year":"2002","journal-title":"Reumatismo"},{"key":"ref_47","first-page":"AB78","article-title":"Dermatomyositis associated with nasopharyngeal carcinoma in a Caucasian patient: The first report in the United Kingdom P705","volume":"54","author":"Mitra","year":"2006","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/j.anorl.2010.10.007","article-title":"Paraneoplastic dermatomyositis accompanying nasopharyngeal carcinoma: Diagnosis, treatment and prognosis","volume":"128","author":"Chakroun","year":"2011","journal-title":"Eur. Ann. Otorhinolaryngol. Head Neck Dis."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"854","DOI":"10.1111\/j.1365-2133.2009.09274.x","article-title":"Malignancies associated with dermatomyositis and polymyositis in Taiwan: A nationwide population-based study","volume":"161","author":"Huang","year":"2009","journal-title":"Br. J. Dermatol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1097\/BOR.0b013e328359e780","article-title":"Paraneoplastic rheumatologic syndromes","volume":"25","author":"Azar","year":"2013","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1016\/j.rdc.2020.04.002","article-title":"Paraneoplastic Musculoskeletal Syndromes","volume":"46","author":"Khan","year":"2020","journal-title":"Rheum. Dis. Clin. N. Am."},{"key":"ref_52","first-page":"615","article-title":"Incidence and predictive factors for malignancies with dermatomyositis: A cohort from southern China","volume":"32","author":"Chen","year":"2014","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"1273","DOI":"10.1111\/j.1365-2133.2011.10595.x","article-title":"Incidence, cancer risk and mortality of dermatomyositis and polymyositis in Taiwan: A nationwide population study","volume":"165","author":"Kuo","year":"2011","journal-title":"Br. J. Dermatol."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"904","DOI":"10.1046\/j.1365-4362.1997.00190.x","article-title":"Malignancy in adult dermatomyositis","volume":"36","author":"Leow","year":"1997","journal-title":"Int. J. Dermatol."},{"key":"ref_55","first-page":"26","article-title":"Dermatomyositis in the elderly: A study of 4 cases in southern Tunisia","volume":"13","author":"Frikha","year":"2012","journal-title":"Pan Afr. Med. J."},{"key":"ref_56","first-page":"9","article-title":"Current therapy for polymyositis and dermatomyositis","volume":"29","year":"2008","journal-title":"Rev. Med. Interne"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1016\/j.autrev.2011.06.007","article-title":"Therapy of polymyositis and dermatomyositis","volume":"11","author":"Marie","year":"2011","journal-title":"Autoimmun. Rev."},{"key":"ref_58","doi-asserted-by":"crossref","unstructured":"Sabat\u00e9-Ortega, J., Bujons-Buscarons, E., Fina-Planas, C., Vilanova-Anducas, N., Vidal-Sarr\u00f3, N., and Sala-Gonz\u00e1lez, N. (2023). Paraneoplastic dermatomyositis associated with urothelial cancer: Report of a case and systematic review of the literature. Front. Oncol., 13.","DOI":"10.3389\/fonc.2023.1223627"},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1007\/s11845-017-1716-7","article-title":"Dermatomyositis and colorectal cancer: A systematic review","volume":"187","author":"Gkegkes","year":"2018","journal-title":"Ir. J. Med. Sci."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1046\/j.1365-4362.2002.01648.x","article-title":"Polymyositis and dermatomyositis associated with malignancy: A 30-year retrospective study","volume":"41","author":"Wakata","year":"2002","journal-title":"Int. J. Dermatol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"e21733","DOI":"10.1097\/MD.0000000000021733","article-title":"Malignancy in dermatomyositis: A retrospective paired case-control study of 202 patients from Central China","volume":"99","author":"Chang","year":"2020","journal-title":"Medicine"}],"container-title":["Medicina"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1648-9144\/61\/2\/334\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T16:34:02Z","timestamp":1760027642000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1648-9144\/61\/2\/334"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,2,14]]},"references-count":61,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2025,2]]}},"alternative-id":["medicina61020334"],"URL":"https:\/\/doi.org\/10.3390\/medicina61020334","relation":{},"ISSN":["1648-9144"],"issn-type":[{"type":"electronic","value":"1648-9144"}],"subject":[],"published":{"date-parts":[[2025,2,14]]}}}