A 41-year-old female patient was referred to the head and neck surgery department because of a large midline submandibular mass that was diagnostic for functioning thyroid tissue in a totally ectopic location.

Papillary thyroid carcinoma of the pyramidal lobe is rare. We describe a case of a 77-year-old woman who corroborates current literature findings, namely greater presence of adverse prognostic factors. Upper Neck Papillary Thyroid Cancer is a new entity, proposed to group these tumors with potential clinical and therapeutic implications.

Background Papillary thyroid carcinoma (PTC) primarily located in the pyramidal lobe of the thyroid gland is extremely rare, so the clinical and pathological features are not well understood. Case The authors describe a case of multifocal PTC with larger pyramidal lobe tumour, in a 77-year-old female patient who underwent en bloc total thyroidectomy with pyramidal lobe, hyoid bone and cervical lymph node excision. Conclusion In line with the present case, current literature reports a greater presence of worse prognostic factors, namely extrathyroidal extension, advanced T stage or presence of cervical lymph node metastasis. Recently, a new classification has been suggested – Upper Neck Papillary Thyroid Cancer (UPTC) – which encompasses these carcinomas, Delphi ganglion metastases and thyroglossal duct cyst carcinomas, with potential clinical and therapeutic implications, particularly the need for orthotopic thyroidectomy. Also, the complete excision of the pyramidal lobe during thyroidectomy may influence the success of radioactive iodine therapy and the patient’s follow-up surveillance.