: "Spindle cell rhabdomyosarcoma 😷 of the head and neck is a very rare tumor in adults. This is a case with long-term survival. A 41-year-old nonsmoking Caucasian man presented in June 2007 with a painless swelling under his tongue. A diagnosis of a soft tissue sarcoma, and a myofibrosarcoma in particular, was made via biopsy. After multimodal treatment, including local and systemic therapy, the patient remained disease-free until September 2010.
The local recurrence was treated unsuccessfully with various chemotherapy regimens. By August 2011, the tumor had reached a size of 11cm in its greatest dimension (figure). In September 2011, the patient underwent surgical resection again, and a spindle cell rhabdomyosarcoma was diagnosed (figure). To analyze the mismatchbetween the original diagnosis of a myofibrosarcoma and the second diagnosis, the two specimens were reassessed, and a final diagnosis of a spindle cell rhabdomyosarcoma was made. In 2012 and 2013, the patient suffered further recurrences that were surgically treated, and he is still alive (May 2014) with disease six years and 10 months after the initial diagnosis in June 2007.
In adults, the spindle cell rhabdomyosarcoma tumor is very rare in the head and neck region. In contrast
to childhood tumors, spindle cell rhabdomyosarcoma in adulthood is often associated with a poor prognosis. In the present case, the radical surgical treatment might have helped to prolong the patient’s overall survival, which has lasted more than six years. To our knowledge, this is the longest overall survival reported so far for this tumor entity in the head and neck region.
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