An adult spindle cell rhabdomyosarcoma in the head and neck region with long-term survival: a case report

Abstract

IntroductionSpindle cell rhabdomyosarcoma of the head and neck is a very rare tumor in adults. We report on one case with long-term survival.Case presentationA 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, our patient remained disease-free until September 2010. The local recurrence was treated unsuccessfully with various chemotherapy regimens. In September 2011, our patient underwent surgical resection again, and a spindle cell rhabdomyosarcoma was diagnosed. To analyze the mismatch between 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, our patient suffered further recurrences that were surgically treated, and he is still alive with disease six years and 10 months after the initial diagnosis in June 2007.ConclusionsIn 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.

Nasal spindle cell tumor with rhabdomyoblastic features: A rare and diagnostically difficult case

“…This is a unique case report not only because nasal spindle cell rhabdomyosarcoma is very rare 3,4 but also because the tumor was initially diagnosed as desmoid-type fibromatosis. In the initial tumor, compared with the recurrent one, rhabdomyoblastic features were not clear.…”

Resilience Through Squamous Cell Carcinoma and Rhabdomyosarcoma of Oral Cavity: A Unique Case Report

“…Oral cavity lesions account for 10-12% cases of RMS in head and neck region [ 12 ]. Its occurrence is a rare entity in adults with head and neck as the most frequent site involved [ 13 ]. Atypical mesenchymal proliferation during embryonic tissue development is believed to form histological aspects of RMS.…”

Oral rhabdomyosarcoma in an adult male: A rare case report

“…Sarcomas of the head-and-neck region are found only in 1% of all head-and-neck malignancies. [ 1 ] Among the soft-tissue sarcomas, rhabdomyosarcoma predominantly occurs in children (60%) while is rarely found in the adults (2%–5%). [ 2 3 ] Rhabdomyosarcoma is a malignant neoplasm of mesenchymal cells, showing varying degrees of striated muscle cell differentiation.…”

“…[ 7 ] This variant of rhabdomyosarcoma was first reported in adults by Rubin et al . [ 1 ] These adult-type spindle cell lesions have several distinctive features, including a predilection for the head-and-neck region, a greater degree of cytologic atypia in the spindled cells, focal areas resembling pseudovascular sclerosing rhabdomyosarcoma and a more aggressive clinical course than pediatric lesions. [ 7 ]…”