Join us in Philadelphia this September for the DTRF "Together We Will" Weekend: - Register today!

The Desmoid Tumor Research Foundation

May 2018

Case Report Papillary thyroid carcinoma with nodular fasciitis-like stroma and beta-catenin gene mutations:report of a recurrent case

Author Information

L Zhou(1), L Shi(1), Z Jiang(2), L Xie(1)

1)Dept of Head and Neck Surgery, Institute of Micro-Invasive Surgery of Zhejiang University, Sir Run Run Shaw Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang Province, China; 2)Dept of Pathology, Sir Run Run Shaw Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang Province, China.

Publication

Int J Clin Exp Pathol

Summary

Papillary thyroid carcinoma with nodular fasciitis-like stroma (PTC-NFS) is a rare lesion with dual pathological features, that behaves more aggressively if positive for beta-catenin gene (CTNNB1) mutations. A 48-year-old female presented with a recurrent enlarging tumor with a diagnosis of desmoid-type fibromatosis 6 months after her initial operation for PTC-NFS. Over a 2-year observation period, she underwent remedial surgery to resect the recurrent tumor. Local invasion, beta-catenin aberrant staining, and CTNNB1 mutations were detected both in the primary tumor stroma and the recurrent tumor. This case indicates that CTNNB1 mutation screening and beta-catenin staining should be performed in PTC-NFS cases as a predictor for recurrence. If either of these are positive, a wider surgical resection should be adopted to achieve negative margins. Otherwise, radiotherapy should be considered. Furthermore, the lesion might be better known as papillary thyroid carcinoma with desmoid-type fibromatosis in such situations to reflect its local aggressive nature.

View Abstract