Langerhans Cell Histiocytosis Of Thyroid Research Articles

A rare case of multisystemic Langerhans cell histiocytosis in an adult affecting the thyroid gland, skeletal system, and pituitary gland: A case report.

Langerhans cell histiocytosis (LCH) is a rare disorder affecting usually children and rarely adults. The most common site affected is bone. The involvement of the thyroid gland in LCH is extremely rare and it is usually observed in an adult as a multisystemic disease. Adult males presented with anterior neck swelling, and fine needle aspiration cytology (FNAC) diagnosed it as suspicious for malignancy. On frozen sectioning, differential diagnosis was given as non-Hodgkin lymphoma and poorly differentiated malignancy. On immunohistochemistry (IHC), tumor cells were positive for CD1a and S100p. Thus, the final diagnosis offered was LCH-thyroid. The pathogenesis is unclear and believed to be related to immune dysfunction, cytokine mediators, viral infection, smoking, and genetic factors. Due to its rarity, thyroid LCH is often misinterpreted as other common disorders of the thyroid and results in delayed diagnosis. This case highlights the importance of histomorphology and the use of ancillary techniques such as IHC to arrive at a diagnosis in time and hence the treatment.

Langerhans cell histiocytosis of the thyroid mimicking thyroiditis in a boy: a case report and literature review

BackgroundLangerhans cell histiocytosis affecting the thyroid commonly presents with nonspecific clinical and radiological manifestations. Thyroid Langerhans cell histiocytosis is typically characterized by non-enhancing hypodense lesions with an enlarged thyroid on computed tomography medical images. Thyroid involvement in LCH is uncommon and typically encountered in adults, as is salivary gland involvement. Therefore, we present a unique pediatric case featuring simultaneous salivary and thyroid involvement in LCH.Case presentationA 3-year-old boy with complaints of an anterior neck mass persisting for 1 to 2 months, accompanied by mild pain, dysphagia, and hoarseness. A physical examination revealed a 2.5 cm firm and tender mass in the left anterior neck. Laboratory examinations revealed normal thyroid function test levels. Ultrasonography revealed multiple heterogeneous hypoechoic nodules with unclear and irregular margins in both lobes of the thyroid. Contrast-enhanced neck computed tomography revealed an enlarged thyroid gland and bilateral submandibular glands with non-enhancing hypointense nodular lesions, and multiple confluent thin-walled small (< 1.5 cm) cysts scattered bilaterally in the lungs. Subsequently, a left thyroid excisional biopsy was performed, leading to a histopathological diagnosis of LCH. Immunohistochemical analysis of the specimen demonstrated diffuse positivity for S-100, CD1a, and Langerin and focal positivity for CD68. The patient received standard therapy with vinblastine and steroid, and showed disease regression during regular follow-up of neck ultrasonography.ConclusionsInvolvement of the thyroid and submandibular gland as initial diagnosis of Langerhans cell histiocytosis is extremely rare. It is important to investigate the involvement of affected systems. A comprehensive survey and biopsy are required to establish a definitive diagnosis.

Isolated Langerhans Cell Histiocytosis of Thyroid-Masquerading as a Thyroid Neoplasm - A Rare Case Report

Langerhans Cell Histiocytosis (LCH) is a rare neoplastic disease of unknown etiology characterized by oligoclonal proliferation of langerhans cells with a varying incidence rate of 4-5.4 per 1 million individuals. LCH occurs in localised and multifocal forms. LCH lesions were first described in the year 1893, they are composed of large histiocytes with abundant cytoplasm intermixed with lymphocytes and eosinophils. Diabetes insipidus is the most common endocrinological abnormality of LCH. Other manifestations such as hypothalamic/pituitary axis disturbance and anterior pituitary deficiency can occur resulting in secondary or tertiary hypothyroidism. Cases of LCH with isolated thyroid involvement are very rare. We present a case of LCH, in a 42-year-old female who had a nodular thyroid swelling since one and a half year with euthyroid status. Total thyroidectomy specimen was received with a clinical diagnosis of thyroid neoplasm. Histopathology revealed langerhans histiocytes both mononucleated and multinucleated with prominent nuclear grooves, accompanied by good number of eosinophils and lymphocytes. Histopathology diagnosis of LCH was given supported by IHC S-100 and CD 1a positivity. Since, it’s a rare entity it needs to be distinguished from its closer mimics non Hodgkin’s lymphoma and Rosai Dorfman’s disease. Treatment of choice is surgical resection, but there is no evidence of improved outcome on usage of adjuvant chemoradiotherapy for primary thyroid LCH post resection. So, more case reports and articles are recommended to acess treatment regimen for better outcome.

Thyroid Langerhans cell histiocytosis and papillary thyroid carcinoma

A 27-year-old female, married with two children, presented to our clinic with a 1-year history of thyroid swelling and pressure symptoms on lying backward and bilateral cervical lymphadenopathy. The patient was a known case of panhypopituitarism for 5 years. Comprehensive patient evaluation including FNAC with papillary thyroid cancer result then she underwent total thyroidectomy and bilateral neck dissection and final histologic examination confirmed papillary thyroid carcinoma in the background of lymphocytic thyroiditis, associated with Langerhans cell histiocytosis (LCH). The draining cervical lymph nodes were also involved by LCH and metastatic papillary thyroid carcinoma. Although the association of LCH with papillary thyroid carcinoma in the thyroid has been reported, their co-existence with LCH in the draining lymph nodes is very uncommon.

CT imaging findings of thymus or thyroid gland involved in Langerhans cell histiocytosis of pediatric patients

Objective To investigate the CT imaging findings of thymus and thyroid gland involved in Langerhans cell histiocytosis(LCH) of pediatric patients. Methods CT image findings in pediatric patients with LCH confirmed by pathology and clinical diagnosis from January 2006 to February 2015 were retrospectively analyzed. By radiography, 38 cases' thymus gland were involved and 8 cases' thyroids gland were involved. Results Plain CT scan for thymus gland involved patients revealed 9 cases were shown as hyperplasia, 17 cases were shown as calcification, 9 cases were shown as hyperplasia with calcification, 1 case was shown as cavity, 1 case was shown as hyperplasia with calcification and cavity and 1 case was shown as hyperplasia with calcification and low density. Enhanced CT scan for thyroid gland involved patients showed bilateral or unilateral enlargement of thyroid gland with heterogeneous low density and unobvious enhancement. Conclusions Finely calcifications were the most common CT findings on thymus gland involved in LCH and might without hyperplasia. CT imaging of thyroid LCH exhibited an enlarged or normal size and abnormal density in the thyroid gland with adjacent soft tissues involved. Key words: Child; Histiocytosis, Langerhans-cell; Tomography, X-ray computed; Thymus gland; Thyroid gland

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

Goiter is a very common clinical problem; however, Langerhans cell histiocytosis (LCH) with thyroid involvement that presents as a goiter is very rare. In this article, we report one case of thyroid LCH. An 18-year-old male patient presented with goiter, polyuria, polydipsia, and lymphadenectasis of the neck, and LCH was confirmed by a lymph node biopsy and pathological investigation. Without a thyroidectomy, the goiter shrank after nine cycles of chemotherapy. In addition, we summarize the reported thyroid LCH cases in the literature from the last 10 years. LCH usually involves other organs, such as the lungs, bones, skin, pituitary gland, and lymph nodes. Thyroid LCH is more common in adults than in children, and it may coexist with a thyroid carcinoma. Without any unique thyroid manifestations, either clinically or by imaging, it is difficult to distinguish thyroid LCH from other thyroid diseases. Pathology is the gold standard for the diagnosis of LCH. A fine needle aspiration biopsy (FNAB) may help to diagnosis LCH, although sometimes it leads to misdiagnosis. Chemotherapy is recommended for multi-system LCH. Younger patients with widespread disease or who are non-responsive to chemotherapy have poor outcomes.

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

Comments on “Langerhans cell histiocytosis of the lung and thyroid, co-existing with papillary thyroid cancer” Jamaati HR, Shadmehr MB, Saidi B, Khosravi A, Arab M, Mohammadi F. Endocr Pathol 20:133–136, 2009 and “Incidental langerhans cell histiocytosis of thyroid: case report and review of the literature” Foulet-Roge A, Josselin N, Guyetant S, Gardet JJ, Besancon A, Saint-Andre JP, Fabiani B. Endocr Pathol 13:227–233, 2002. We read with great interest the contributions of Jamaati et al. and Foulet-Roge et al. respectively entitled “Langerhans cell histiocytosis of the lung and thyroid, co-existing with papillary thyroid cancer” and “Incidental langerhans cell histiocytosis of thyroid: case report and review of the literature” [1, 2]. We have had a similar case of coexistent thyroid Langerhans cell histiocytosis (LCH) and papillary thyroid carcinoma (PTC). A 29-year-old male patient presented with a huge neck mass, immobile, and compressive, involving the trachea, the pharynx, the left jugular vein and the left carotid sheath. Bone (mandible), lung, skin and hypothalamo-pituitary LCH lesions had been documented for 6 years, and clinical stability had been obtained with corticosteroids. The transpharyngeal biopsies performed during a panendoscopy revealed a PTC with positive immunostaining for TTF1, HBME1 and CK19. Associated LCH involvement was diagnosed by the presence of Langerhans cells reactive to CD1a and S100 protein (Fig. 1). The cytological examination of a blind transcutaneous thyroid fine needle aspiration biopsy and other surgical biopsies isolated only the LCH section and a lymphocytic thyroiditis. A surgical resection of this advanced disease could not be considered and a multidisciplinary panel proposed a medical treatment (Prednisone 40 mg/day). Given that there was no response 6 months later, chemotherapy with Vinblastine (Velbe® 6 mg/m every 2 weeks) produced a stabilization over 14 months. Then, due to a severe increase in size and the extension from the oropharynx to the mediastinum in a vertical axis and from the cervical rachis to the subcutaneous tissues in a horizontal axis, a treatment with Cladribine was initiated (Leustatin® 0.1 mg/kg/day over 5 days every 2 months). After 7 months without a response, the treatment was stopped and changed to Imatinib (Gleevec® 100 mg/day for 1 month and then 400 mg/day). Three months after the beginning of this targeted therapy and 3 years after the appearance of the cervical mass, a dramatic dyspnea caused by the compression and the infiltration of the trachea occurred. A tracheostomy under local anaesthesia was carried out in difficult technical conditions. Despite this attempt, acute pulmonary distress resulted in the death of the patient. The family refused an autopsy. S. Vergez (*) Department of Head and Neck Surgery, University-Hospital Rangueil-Larrey, 24 chemin de Pouvourville, 31059 Toulouse Cedex 9, France e-mail: vergez.s@chu-toulouse.fr