Aggressive tall cell variant of papillary thyroid cancer in a child with Omenn Syndrome

  • BACKGROUND: Thyroid cancer is a rare pediatric tumor, representing about 3% of all pediatrics carcinomas.

    METHODS: A case of tall cells thyroid carcinoma with a very aggressive behavior in a eight years old patient with Omenn Syndrome is described.

    RESULTS: The patient underwent Total Thyroidectomy+central neck dissection+left lateral neck dissection (levels II-V) in May 2017. The final histology showed a widespread presence of papillary carcinoma type with areas of tall cells papillary carcinoma (about 30%) and solid type (about 30%) reaching thyroid surface,affected adipose and muscular components with some aspects of vascular neoplastic infiltration. Nodes metastases were found  in 22/29 nodes (pT3b N1b Mx- Stage I). No mutation of TERT promoter and BRAF mutations. Patient underwent Radioiodine therapy with the evidence of metastases in bilateral cervical lymph nodes and secondary localization in the upper lobe of the left lung. A right lateral neck dissection (levels II-V)+completion of left lateral neck dissection (levels I-IIb) was performed. The definitive histological examination showed metastasis in 5/59 nodes removed. The patient underwent, in September 2017, a PET-CT with no uptake neither in the neck nor lung.

    DISCUSSION & CONCLUSION: Tall cells carcinoma is an adult and elderly pathology and it is very rare in pediatric patients. Guidelines don’t provide any information concerning its management. Our case had a very aggressive behavior at presentation with an excellent response to treatments despite the advanced and aggressive form. The extent of initial surgery appears to have a greatest impact on improving long-term disease free survival also in pediatric population, suggesting the necessity of dedicated guide-line for these patients.


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