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S Villarin-Ayala, R Farrell, S Lou Asa, E Barksdale, S Wilhelm, A Kim Mackow
Case Western Reserve University School of Medicine

An 8 year old female was initially evaluated by pediatric endocrinology in 2015 (at the age of 2) for an asymptomatic right neck mass. Neck ultrasound showed heterogeneous enlargement of the right thyroid lobe with several nodules. FNA in 2015 revealed benign follicular tissue; the patient was biochemically euthyroid. In 2016, she underwent right thyroid lobectomy with isthmusectomy for definitive diagnosis at another hospital. Pathology was reported as multinodular goiter with lymphocytic thyroiditis.

Later in 2016, she was found to have significant left eye visual loss with photophobia on preschool screening. Ophthalmologic evaluation at yet another hospital revealed a choroidal amelanotic mass touching the optic disc, with extensive retinal detachment. Favoring a choroid hemangioma, this was managed with plaque radiation. In 2018, she developed symptomatic neovascular glaucoma requiring left eye enucleation. Pathology was read as benign ectopic thyroid tissue. No further workup was carried out at the outside hospital.

After re-establishing care with her initial pediatric endocrinologist two years later, she was found to be biochemically euthyroid on a lower-than-expected dose of levothyroxine. She underwent a 123I whole-body scan on thyrogen and low-iodine diet; it demonstrated uptake in the left thyroid as well as significant uptake concerning for osseous metastases in the right acetabular roof and the right posterior laminar process of S1. Review of both previous pathology specimens revealed a lobulated angioinvasive follicular thyroid carcinoma (FTC) replacing the right thyroid and compressing the surrounding gland that exhibited chronic thyroiditis, and metastatic FTC in the left eye. The patient was referred to our pediatric endocrine surgery team for a completion left thyroid lobectomy to facilitate radioactive iodine ablation therapy for her bone metastases. Completion thyroidectomy pathology showed a 0.25cm focus of minimally invasive FTC in a thyroid with chronic lymphocytic thyroiditis.

This case represents a delay in diagnosis of a rare FTC choroidal metastasis, with contribution from social issues leading to care at multiple institutions. This reveals the need for a high index of suspicion when ectopic thyroid tissue is found, especially with a history of previous thyroid surgery.

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