Renal cell carcinoma-associated adult dermatomyositis treated laparoscopic nephrectomy
A 77-years-old Caucasian lady, with known rheumatoid arthritis and hypothyroidism, presented with a 4 months history of facial rash and progressive muscle weakness affecting the legs more than the arms. Examination revealed Gottron papules (erythematous papules on the dorsal surface of the phalangeal joints), heliotrope rash (oedematous erythema of the eyelids) and proximal muscle weakness []. Blood biochemistry revealed a creatine kinase (CK) level of >6000 U/L (normal 0-70 U/L) and positive ANA (Antinuclear Antibody). The electromyography (EMG) studies and a muscle biopsy were subsequently performed. The EMG showed signs of severe myopathic features. The needle EMG examination showed profuse spontaneous activities affecting the upper and lower limb muscles indicating an active stage of myositis. The muscle biopsy showed infiltration of the muscle fibres by lymphocytes and macrophages associated with muscles fibres necrosis. In view of these findings, a diagnosis of dermatomyositis was made. The patient was admitted due to the severe muscle weakness and was commenced on high dose steroids and intravenous immunoglobulins (IV-IG) without any significant benefit. Cancer screening was undertaken and a staging computed tomography (CT) of the chest, abdomen and pelvis revealed a 4 cm upper pole solid mass on the left kidney, suggestive of renal cell carcinoma. She was referred to urology and underwent a laparoscopic nephrectomy four weeks after presentation. Histopathology confirmed conventional (clear cell) renal cell carcinoma with focal nuclear changes accounting to Fulham grade 2. The carcinoma was confined to the kidney with no evidence of lympho-vascular invasion (stage pT1 a). Following the nephrectomy the patient was continued on steroids and had further monthly IV IG. Within four weeks of the nephrectomy her CK had improved to less than 200 U/L and there was a significant improvement in muscle strength []. She remained in hospital for 3 months during which she made a remarkable recovery with the disappearance of the rash, normalisation of CK levels and consistently improving muscle power. She was discharged on a reducing dose of prednisolone and within 6 months was back to normal life.