Carcinoma of unknown primary and paraneoplastic dermatomyositis

Abstract

Dermatomyositis is known to be associated with neoplastic disorders, however the presentation of carcinoma of unknown primary as dermatomyositis is rare. We describe a case index of 50-year-old female who presented with enlarged inguinal lymph nodes accompanied with symmetric proximal muscle weakness and erythematous plaques. Conventional basic work-up did not reveal the diagnosis, however, positron emission tomography-computed tomography and re-staining of the pathology specimen suggested the ovaries as the primary site. Chemotherapy including carboplatin paclitaxel and bevacizumab led to complete response of disease and improvement in the dermatomyositis. The present case emphasizes the importance of a thorough directed evaluation for the underlying cancer in patients with carcinoma of unknown primary presenting as dermatomyositis. We further provide an up-to-date detailed review of published data describing these clinical entities.

Keywords: Cancers of unknown primary; Dermatomyositis; Paraneoplastic; Positron emission tomography.

Figure 1

Positron emission tomography-computed tomography scan which demonstrated increased standardized uptake values uptake in the pelvis in an ovarian cyst and uptake in para-aortic and cervical lymph node (arrows).