Association between Pituitary Langerhans Cell Histiocytosis and Papillary Thyroid Carcinoma

Abstract

Here we report a case of panhypopituitarism caused by pituitary Langerhans cell hystocitosis (LCH) in a 22-year-old woman affected by papillary thyroid carcinoma (PTC). Although several cases of the coexistence of PTC and LCH within thyroid tissue have been described in relative literature, in this case, the patient presented a unique suprasellar retrochiasmatic histocytosis localization which, to the best of our knowledge, had never been described before in association with PTC. Even if this aspect is not addressed in the present case report, it is worth noting that about 50% of the patients affected either by LCH or PTC are characterized by activating mutations of the proto-oncogene BRAF. This, along with other clinical studies, may warrant further biomolecular large-scale case study investigations in order to evaluate a possible connection between the 2 conditions and shed light on the etiology of these diseases, which are still largely unknown.

Keywords: Langherans cell hystiocytosis; hypopituitarism; papillary thyroid carcinoma.

Figure 1

Magnetic resonance imaging of the suprasellar lesion. Sagittal plane showing the presence of oval-shaped lesion (diameters 17 mm × 8 mm) in the suprasellar region. The lesion presents intense enhancement with a central hypointense area.

Figure 2

Ultrasonograpgy (US) and US-elastography of the thyroid nodule. Baseline US showed a large hypoechoic nodule (diameters 42 mm × 33 mm × 25 mm) with fairly marginated margins (left side); at US-elastography (right side) the lesion appeared stiff with a high ECI index suggesting its malignant nature.

Figure 3

Histology of the thyroid nodule. Typical thyroid papillary carcinoma containing numerous branching and randomly oriented papillae, with a central fibrovascular core lined by a single epithelial cells showing ground glass nuclei and nuclear groove.