Primary squamous cell carcinoma of the thyroid: Case report and systematic review of the literature.

BACKGROUND: Primary squamous cell cancer (PSCC) of thyroid is a rare malignancy with poor prognosis. It is mandatory to exclude secondary involvement of the thyroid by panendoscopy, CT-scan and immunohistochemical analysis. As treatment surgery, radiation and rarely chemotherapy is employed. METHODS: A systematic review of the literature was conducted searching medline and embase database using the medical subject headings "primary squamous cell carcinoma of thyroid" and "primary squamous cell cancer of thyroid", for articles published until April 2016 (n=1733). Of interest were the used treatment modalities and survival outcomes. RESULTS: A total of 35 publications reporting on 50 cases including ours were finally analyzed. A curative treatment approach was described in 24 patients (48%). Additional radiotherapy, chemotherapy or radiochemotherapy was applied in 17, 7 and 7 patients respectively. Median overall survival was 6 months [range 0-48] for 47 patients. Disease free survival was only achieved in 8 patients with disease limited to the thyroid gland, complete surgical resection and additional radiotherapy or radiochemotherapy [reported median 20 months; range 12-48]. CONCLUSION: Reported disease free survival of PSCC of the thyroid was only achieved in patients with complete surgical resection in combination with adjuvant radio- and/or chemotherapy. However long term survival has not been reported in the literature yet.