Paraneoplastic Dermatomyositis Secondary to Hepatocellular Carcinoma in an Ectopic Liver: A Rare Case Report

Objective: To report a rare case of ectopic liver, complicated with hepatocellular carcinoma in a 75-year female who presented with dermatomyositis. Case Report: A 75-year old Saudi female presented with erythema, pruritus and scaling eruptions on the face, neck, trunk and the extremities assuming violaceous color. The condition started five years earlier and was diagnosed as photodermatosis, and was unsuccessfully treated with antihistamines and topical glucocorticoids. Three months prior to admission, she began to experience progressive proximal muscle weakness, dysphagia and severe weight loss. Neurological examination showed proximal, symmetric muscle weakness in upper and lower limbs. MRI showed inflammatory changes of the vastus lateralis muscle which was biopsied later to reveal histopathological changes consistent with dermatomyositis. Enhanced abdominal CT showed a well-defined rounded solid soft tissue mass in the subhepatic right lumbar region. A percutaneous biopsy and histopathology revealed that the patient had malignant tumor with hepatoid pattern of growth mostly in favor of hepatocellular carcinoma. The final diagnosis of our case was hepatocellular carcinoma originating from an ectopic liver tissue and associated with paraneoplastic dermatomyositis. Conservative palliative management was the only option since she was critically ill. The patient’s condition deteriorated rapidly. She developed fever with urinary tract infection and she died of septic shock three days later. Conclusions: Physicians awareness regarding the diagnosis of dermatomyositis should be raised. Screening for hidden malignancies is important in all cases of dermatomyositis.