Neurofibroma and pectoralis muscle hypoplasia: A mild degree of poland's syndrome

Abstract

Background: Poland's Syndrome (PS) is a rare congenital syndrome characterized by the unilateral partial or complete absence of the sternocostal head of the pectoralis major muscle and ipsilateral brachysyndactyly or syndactyly of the fingers. It has been reported to be associated with other diseases, but PS accompanied by neurofibroma has rarely been reported. Case Report: We report a rare case of a 16-year-old man with neurofibroma of the left breast accompanied by PS. Physical examination showed a mass in the left breast with skin hyperpigmentation and increased body hair. Intraoperative exploration showed a mass measuring 3-4 cm between the dysplastic pectoralis major and minor muscles. Pathologic examination of the resected mass showed cutaneous diffuse neurofibroma. A simple left mastectomy was performed. The postoperative course was uneventful with no evidence of recurrence at 4 months. Conclusion: Neurofibroma may also be accompanied by PS and should be differentiated from gynecomastia when a tumor grows in the breast. © 2012 S. Karger GmbH, Freiburg.