Recurrent Parapharyngeal Rhabdomyoma

Abstract

A 64-year-old Caucasian male with a left parapharyngeal mass had a past medical history that was significant for excision of a benign rhabdomyoma of the soft palate 30 years previously. Then 25 years ago, the tumor recurred in the palate and retropharyngeal space on the left and was reexcised. Histologic examination of all three excisions showed adult rhabdomyoma. Ultrastructural and histochemical studies of the second excision of this tumor have been published previously. The present study included histologic, ultrastructural, immunohistochemical, and cytogenetic analyses. The histologic and ultrastructural features of the tumor were identical to those reported 25 years ago. Immunohistochemical studies demonstrated that the tumor cells were desmin and myoglobin positive and vimentin negative. Focal positivity for CD56 was also present. Intracellular inclusions in the tumor cells were strongly positive for desmin. Cytogenetic examination of short-term cultures of the tumor cells demonstrated clonal chromosome abnormalities in 60% of metaphases. The majority of cells showed a reciprocal translocation between chromosomes 15 and 17 as the sole abnormality. A minor clone was characterized by abnormalities of the long arm of chromosome 10. The presence of clonal structural chromosome abnormalities in extracardiac adult rhabdomyoma lends strong support to the idea that these rare tumors are true neoplasms rather than hamartomatous or regenerative lesions.