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Table of Contents
Definition / general | Epidemiology | Clinical features | Diagnosis | Prognostic factors | Case reports | Treatment | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Electron microscopy description | Differential diagnosisCite this page: Younes, S. Langerhans cell histiocytosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/thyroidlch.html. Accessed February 18th, 2019.
Definition / general
- Clonal proliferation of Langerhans cells
- Thyroid involvement is rare
- Isolated or part of disseminated disease
Epidemiology
- Adults more than children, reported in both
- No sex predilection
Clinical features
- Thyroid mass or enlargement, must rule out malignant lesions
- Thyroid function may be affected depending on degree of involvement
Diagnosis
- Combination of clinical, radiologic, cytologic and histopathologic features
- Immunohistochemistry and electron microscopy may play a role
Prognostic factors
- Poor prognosis if systemic disease and thyroid involvement (Mod Pathol 1996;9:145)
Case reports
- 5 month old girl with isolated Langerhans cell histiocytosis of thyroid (Eur J Pediatr 2007;166:1151)
- 19 year old woman with Langerhans cell histiocytosis involving thyroid and parathyroid glands (Mod Pathol 2001;14:111)
- 27 year old man (Int J Clin Exp Pathol 2014;7:1229)
- 28 year old women with masses simulating thyroid carcinoma (Jpn J Clin Oncol 1997;27:180, Hum Pathol 1988;19:239)
- 37 year old man with Langerhans cell histiocytosis of thyroid and multiple cervical lymph node involvement (Case Rep Pathol 2014;2014:184237)
- 54 year old woman (J Clin Endocrinol Metab 2015;100:15)
- Langerhans cell histiocytosis and papillary thyroid carcinoma (Cytopathology 2015;26:130, Rev Clin Esp (Barc) 2014;214:e19)
- Langerhans cell histiocytosis presenting as hypothyroid goiter (BMJ Case Rep 2014 Dec 2;2014)
Treatment
- Chemotherapy and surgery
Microscopic (histologic) description
- Patchy to extensive involvement of thyroid gland, with follicle invasion and destruction
- May show extrathyroid involvement
- Langerhans cells have moderate eosinophilic cytoplasm and oval nuclei with grooves and a thin nuclear membrane
- Also eosinophils, lymphocytes, multinucleated histiocytes
Microscopic (histologic) images
Images hosted on other servers:
Langerhans cell centrally located
High cellularity
Nests of tumor cells and eosinophils
Cytology description
- Smears contain numerous histiocytic cells with moderate to abundant pale eosinophilic cytoplasm, ovoid to rounded vesicular nuclei, nuclear grooves
- Moderate to marked pleomorphism is seen
- Also eosinophils, lymphocytes, giant cells
- Normal thyroid follicular cells and histiocytes are present (Acta Cytol 2004;48:278)
Positive stains
Negative stains
Electron microscopy description
- Birbeck granules: tennis racquet or zipper shaped structures, 33 nm wide with central serrated line
