Langerhans Cell Histiocytosis of the Lung and Thyroid, Co-Existing with Papillary Thyroid Cancer

Abstract

A 24-year-old man presented to our center with a huge goiter compressing his airway. He had a previous diagnosis of Langerhans cell histiocytosis (LCH) of the lung. Core needle biopsy was consistent with histiocytosis. Thyroidectomy was performed. A very invasive mass was encountered at the time of surgery. Histopathology result was consistent with an invasive papillary cancer of thyroid co-occurring with LCH. Although association of LCH with different malignancies has been reported, co-existing invasive papillary thyroid cancer and LCH is a rare combination.

Langerhans Cell Histiocytosis of the Thyroid is a Rare Entity, but an Association with a Papillary Thyroid Carcinoma is Often Described

“…For an LCH strictly limited to the thyroid, surgical removal seems to be the gold standard, whereas aggressive systemic therapy is reserved for cases exhibiting disseminated disease [1]. It is interesting to note that among the eight patients with LCH and PTC, five were disease-free after surgery with or without medical treatment, but our patient had a fatal outcome despite several medical therapies without surgery to the thyroid.…”

“…To our knowledge, the current case is the eighth reported patient with co-existing thyroid LCH and PTC (Table 1) [1][2][3][4][5][6][7]. A frequent association of LCH and malignant neoplasms such as lymphoma, leukaemia and lung cancer [8] has been described.…”

“…For one patient, a marked infiltration of mixed inflammatory cells composed of lymphocytes, eosinophils and plasmocytes was observed [1]. The incidence of PTC arising in chronic inflammatory conditions of the thyroid (such as Hashimoto's thyroiditis) is increased compared with that of the general population [3,5].…”

A rare cause of goiter: Langerhans cell histiocytosis of the thyroid

“…The goiter may occur before or after the symptoms of other organ involvement. Thyroid function may be euthyroid or hypothyroid depending on the degree of thyroid involvement [10]. The enlarged thyroid presents as a hypoechoic mass on an ultrasound and as a "cold nodule" or radioisotope defect in a thyroid isotope scan.…”

“…Thyroid LCH cases that were misdiagnosed as thyroid carcinomas were reported in the literature recently [21]. Additionally, LCH may coexist with a thyroid carcinoma [6,10,16,29]. It is possible that chronic inflammatory conditions of the thyroid induced by LCH lead to an increased risk for the development of a thyroid carcinoma [6].…”