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Thyroglossal duct cysts are the most common congenital disorder of the neck. One percent of cases may degenerate and give rise to a cancer, mainly arising in the pericystic thyroid tissue. Some 250 cases have been reported in the literature to date. We report here on a 39-year-old man with a midline mass in the neck measuring 4 cm max. The patient was examined preoperatively by ultrasonography of the neck and assay of thyroid hormones, which yielded a diagnosis of a thyroglossal duct cyst. On the basis of these findings, the patient underwent surgery to remove the mass and, after an extempore histopathological examination, was submitted to total thyroidectomy owing to the presence of papillary carcinoma of the thyroid arising on the thyroglossal duct cyst with multiple foci in the context of the thyroid gland. Most thyroid cancers at the time of surgery are confined to the thyroid gland, infiltrating the adjacent structures in approximately 20% of cases and the local-regional lymph nodes in 8 to 11.5%. Thyroid papillary adenocarcinoma is multifocal in 21% of cases. The multifocal nature of the cancer makes total thyroidectomy mandatory at the same time as surgery is performed to remove the cyst.

The aim of this study was to describe the clinico-pathological characteristics of malignant biliary papillomatosis and the diagnostic and therapeutic approach adopted in this case, which extended from the left hepatic biliary duct to the bifurcation of the 2nd and 3rd segments. The patient was studied with ultrasonography, endoscopic retrograde cholangiopancreatography (ERCP), CT scan and MRI cholangiography, which showed the morphological characteristics and the extent of the disease in the biliary tract. The malignancy of the disease was confirmed by preoperative cytological analysis of bile collected via a nasobiliary probe. The surgical treatment, preceded by intraoperative ultrasonography, consisted in a left hepatectomy, sparing the 1st segment, and a right intrahepatic jejunostomy. The final pathological analysis revealed a malignant biliary papillomatosis. Preoperative ERCP clearly documented the exact distribution of the biliary tract lesions and allowed both treatment of the cholestasis and diagnosis of the nature of the lesion through cytological analysis of the bile. Surgical treatment must be carried out and guided not only by the preoperative diagnosis, but also by perioperative ultrasonography. Finally, owing to the rarity of such cases and consequently our very limited knowledge of the disease, an intensive follow-up is recommended.