CASE REPORT Paraneoplastic dermatomyositis as presentation of thymic carcinoma Andrea Dell’Amore • Nizar Asadi • Guido Caroli • Giampiero Dolci • Alessandro Bini • Franco Stella Received: 15 August 2011 / Accepted: 8 August 2012 / Published online: 21 August 2012 Ó The Japanese Association for Thoracic Surgery 2012 Abstract Thymic carcinomas are very rare and hetero- geneous groups of anterior mediastinum neoformations with an extremely aggressive behavior. Often, the diag- nosis is made in the advanced stages. Paraneoplastic syn- dromes associated with thymic carcinoma are extremely rare. We report a case of a 64-year-old man presenting with early stage thymic carcinoma which was discovered because of associated paraneoplastic dermatomyositis. The dermatomyositis disappeared completely after radical resection of the tumor. After 20-month follow-up, the patient is in good clinical condition without recidivism of disease. Keywords Dermatomyositis Á Thymic carcinoma Á Paraneoplastic syndrome Á Thymus Introduction Thymic carcinomas (TCs) are uncommon and heteroge- neous groups of neoformations with an incidence ranging from 0.8 to 36 % of all thymic tumors [1]. TCs include different histological types classified recently by the WHO classification as malignant epithelial thymic tumors [2]. TCs have an extremely aggressive behavior and the diag- nosis is often made in the advanced stages. Paraneoplastic dermatomyositis (DM) associated with TCs is extremely rare [1, 3]. Case report A 64-year-old male was admitted to the dermatologic department because of pruritus, erythema and progressive weakness of the arms and legs without significant medical history. Physical examination revealed a scaly erythematous rash on his face, chest and hands. Gottoron’s papules were discovered over the knuckles of the fingers (Fig. 1). The neurological evaluation confirmed muscle weakness of grade 3–4/5 power involving both shoulders and hip girdles. Electromyography examination demonstrated typical myo- pathic changes in the left deltoid and quadriceps indicating myositis rather than myasthenia gravis (MG). Laboratory tests showed remarkably elevated lactate-dehydrogenase (LDH) and creatine-phosphokinase (CPK). Tumor markers were normal except for slightly elevated carbohydrate anti- gen 19-9 (CA19-9). Rheumatologic analysis showed only a mild increase of anti-nucleus antibodies. A skin and left biceps muscle biopsy specimen showed a histological pat- tern compatible with an inflammatory myositis. The patient was treated with steroids (40 mg/die-prednisolone) and azathioprine (100 mg/die) achieving the remission of the dermatological alteration and muscle weakness. With the suspicion of paraneoplastic syndrome, chest radiography and computed tomography (CT scan) were performed. The total-body CT scan showed a 60 9 28 mm mass with irregular margins of the upper anterior mediastinum (Fig. 2a). No further neoformations were reported. The mass had an [ 18 F]fluorodeoxyglucose activity of 9.1 at positron emission tomography (PET) (Fig. 2b). The CT-guided fine needle aspiration biopsy and cytological analysis revealed malignant epithelial cells suggesting a thymic carcinoma. We opted for surgery with radical intent. The clinical con- ditions of the patient were improved after medical therapy and no further comorbidities were present. A. Dell’Amore (&) Á N. Asadi Á G. Caroli Á G. Dolci Á A. Bini Á F. Stella Department of Thoracic Surgery, S. Orsola Malpighi Hospital, University of Bologna, Via Massarenti 9, Bologna, Italy e-mail: dellamore76@libero.it 123 Gen Thorac Cardiovasc Surg (2013) 61:422–425 DOI 10.1007/s11748-012-0144-x