title: Langerhans cell sarcoma arising from Langerhans cell histiocytosis: a case report.
abstract: Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells that have overtly malignant cytologic features. It is a very rare disease and theoretically, it can present de novo or progress from an antecedent Langerhans cell histiocytosis (LCH). However, to our knowledge, LCS arising from an antecedent LCH has not been reported on. We present here a case of LCS arising from a pulmonary LCH. A 34 yr-old man who was a smoker, had a fever and a chronic cough. Computed tomographic (CT) scan revealed multiple tiny nodules in both lungs. The thoracoscopic lung biopsy revealed LCH. The patient quit smoking, but he received no other specific treatment. One year later, the follow up chest CT scan showed a 4 cm-sized mass in the left lower lobe of the lung. A lobectomy was then performed. Microscopic examination of the mass revealed an infiltrative proliferation of large cells that had malignant cytologic features. Immunohistochemical stains showed a strong reactivity for S-100 and CD68, and a focal reactivity for CD1a. We think this is the first case of LCS arising from LCH.
authors.affiliation: Department of Pathology, College of Medicine, Gyeongsang National University, Jinju, Korea.
authors.forename: Jong-Sil
authors.initials: JS
authors.lastname: Lee
authors.forename: Gyung Hyuck
authors.initials: GH
authors.lastname: Ko
authors.forename: Ho Cheol
authors.initials: HC
authors.lastname: Kim
authors.forename: In Seok
authors.initials: IS
authors.lastname: Jang
authors.forename: Kyung-Nyeo
authors.initials: KN
authors.lastname: Jeon
authors.forename: Jeong-Hee
authors.initials: JH
authors.lastname: Lee
mesh_terms: D000328:Adult; D015703:Antigens, CD / Q000096:biosynthesis; D018949:Antigens, CD1 / Q000096:biosynthesis; D015214:Antigens, Differentiation, Myelomonocytic / Q000096:biosynthesis; D015972:Gene Expression Regulation, Neoplastic; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007801:Langerhans Cells / Q000473:pathology*; D008297:Male; D010190:Pancreatic Neoplasms / Q000473:pathology*; D013902:Radiography, Thoracic; D009418:S100 Proteins / Q000096:biosynthesis; D012509:Sarcoma / Q000473:pathology*; D014057:Tomography, X-Ray Computed; D000097382:CD68 Molecule
chemical_list: D015703:Antigens, CD; D018949:Antigens, CD1; D015214:Antigens, Differentiation, Myelomonocytic; D009418:S100 Proteins; C419393:CD1a antigen; C067980:CD68 antigen, human; D000097382:CD68 Molecule