Apical Schwannoma Presenting as Harlequin Syndrome

Abstract

Fig 3. Aa 12-month history of unilateral facial flushing when she exercised in the gym; only the left side of her face became red (Fig 1). Physical examination at rest was normal. There was no detectable difference in the sweat pattern on her hands or face and no signs of Horner’s syndrome. These symptoms and signs are consistent with Harlequin syndrome. First reported in 1988 by Lance and colleagues [1], this syndrome occurs because of autonomic neuropathy to the side that lacks flushing. Varying causes have been reported. In this patient, a chest roentgenogram revealed a well-defined mass in the right apex medially (Fig 2). Magnetic resonance imaging of the thorax showed a right upper thoracic paravertebral mass. Thoracoscopic excision and histologic examination revealed a World Health Organization grade I schwannoma (Fig 3; hematoxylin and eosin stain, 20 magnification). The patient has recovered well and is considering left-sided thoracoscopic sympathectomy to restore facial symmetry.