%0 Journal Article
%T Multifocal Adult Rhabdomyoma of the Head and Neck Manifestation in 7 Locations and Review of the Literature
%A Lorraine A. de Trey
%A Stephan Schmid
%A Gerhard F. Huber
%J Case Reports in Otolaryngology
%D 2013
%I Hindawi Publishing Corporation
%R 10.1155/2013/758416
%X Background. Adult rhabdomyoma is a rare benign tumour with the differentiation of striated muscle tissue, which mainly occurs in the head and neck region. Twenty-six cases of multifocal adult rhabdomyoma are documented in the literature. Method. We report a 55-year-old male with simultaneous diagnosis of 7 adult rhabdomyomas and review the literature of multifocal adult rhabdomyoma. Result. Review of the literature revealed 26 cases of multifocal adult rhabdomyoma, of which only 7 presented with more than 2 lesions. Mean age at diagnosis was 65 years with a male to female ratio of 5.5£¿:£¿1. Common localizations were the parapharyngeal space (36%), larynx (15%), submandibular (14%), paratracheal region (12%), tongue (11%), and floor of mouth (9%). Besides the known radiological features of adult rhabdomyoma, our case showed FDG-uptake in (18) F-FDG PET/CT. Conclusion. This is the first case of multifocal adult rhabdomyoma published, with as many as 7 simultaneous adult rhabdomyomas of the head and neck. 1. Introduction Rhabdomyoma, named by Zenker [1] in 1864, is an exceedingly rare benign tumor that exhibits mature skeletal muscle differentiation. Although in general benign soft tissue neoplasms outnumber their malignant counterpart, this is not true for rhabdomyomas, which are considerably less common than rhabdomyosarcomas and account for no more than 2% of all striated muscle tumors. Topographically a distinction is made between the more common cardiac and the extracardiac localizations. Cardiac rhabdomyomas are rare tumors that occur chiefly in the heart of infants and small children [2]. They are considered to be hamartomatous lesions and are frequently associated with tuberous sclerosis [3]. According to Weiss and Goldblum extracardiac rhabdomyomas can be divided into adult, fetal, and genital types, the adult type being the most common [2]. Adult rhabdomyoma (ARM) predominantly occurs in individuals over 40 with a male to female ratio of 3£¿:£¿1 to 5£¿:£¿1 depending on the literature [2, 4, 5]. There is a predilection for the head and neck region. ARM mostly appears as a solitary lesion but may be multicentric in about 15% [6]. Fetal rhabdomyoma is even less common than ARM. It also mainly occurs in male patients in the head and neck but is often present at birth. Moreover, it differs from the adult type in its areas of predilection and histology. Two different subtypes are known, the myxoid and intermediate types. The genital rhabdomyoma is a rare tumor found in the vagina and vulva of middle-aged females. As a related lesion, Weiss further mentions
%U http://www.hindawi.com/journals/criot/2013/758416/