{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T14:17:49Z","timestamp":1787840269444,"version":"build-2784847793"},"reference-count":23,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,1]]},"abstract":"<jats:p>Langerhans cell sarcoma (LCS) is a rare malignant tumor of Langerhans cells and uncommonly involves head and neck regions. Unlike Langerhans cell histiocytosis (LCH), it has an aggressive clinical course with malignant cytological features. Till now, a handful of cases have been reported and the common anatomical sites involved are skin, lymph node, and bone in loco \u2013 regional cases and lymph node, lung, liver, spleen, and bone in disseminated disease. Due to its rarity, standard protocols of treatment for these patients are not yet well established. Herein, we report such a case in a 25-year-old male presenting with a bilateral submandibular swelling, which was diagnosed as LCH on Fine Needle Aspiration Cytology (FNAC) and later confirmed to be a case of LCS in histopathological examination and immunohistochemistry. The authors are aware of only a single similar case being reported in the English literature.<\/jats:p>","DOI":"10.4103\/ijc.ijc_819_20","type":"journal-article","created":{"date-parts":[[2024,4,10]],"date-time":"2024-04-10T06:28:11Z","timestamp":1712730491000},"page":"123-127","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":4,"title":["Unusual presentation of Langerhans cell sarcoma as bilateral submandibular gland swelling \u2013 A rare case report"],"prefix":"10.4103","volume":"59","author":[{"given":"Pranita","family":"Mohanty","sequence":"first","affiliation":[{"name":"Department of Pathology, Institute of Medical Sciences and SUM Hospital, Bhubaneswar, Odisha, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nibedita","family":"Sahoo","sequence":"additional","affiliation":[{"name":"Department of Pathology, Institute of Medical Sciences and SUM Hospital, Bhubaneswar, Odisha, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anasuya","family":"Lenka","sequence":"additional","affiliation":[{"name":"Department of Pathology, Institute of Medical Sciences and SUM Hospital, Bhubaneswar, Odisha, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suvodip","family":"Bhattacharyya","sequence":"additional","affiliation":[{"name":"Department of Pathology, Institute of Medical Sciences and SUM Hospital, Bhubaneswar, Odisha, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2022,5,19]]},"reference":[{"key":"R2-20250603","doi-asserted-by":"crossref","first-page":"4557","DOI":"10.4049\/jimmunol.0902336","article-title":"Cell-specific gene expression in Langerhans cell histiocytosis lesions reveals a distinct profile compared with epidermal Langerhans cells","volume":"184","author":"Allen","year":"2010","journal-title":"J Immunol"},{"key":"R3-20250603","doi-asserted-by":"crossref","first-page":"320","DOI":"10.1016\/j.ctrv.2015.02.011","article-title":"Langerhans cell sarcoma: A systematic review","volume":"41","author":"Howard","year":"2015","journal-title":"Cancer Treat Rev"},{"key":"R4-20250603","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1001\/archotol.1988.01860170091026","article-title":"Multifocal extraosseous eosinophilic granuloma of the head and neck","volume":"114","author":"Green","year":"1988","journal-title":"Arch Otolaryngol Head Neck Surg"},{"key":"R5-20250603","first-page":"1808","article-title":"111In-pentetreotide scintigraphy in patients with Langerhans' cell histiocytosis","volume":"41","author":"Weinmann","year":"2000","journal-title":"J Nucl Med"},{"key":"R6-20250603","first-page":"201","article-title":"Langerhans' cell histiocytosis in the parotid gland","volume":"32","author":"Darvishian","year":"2002","journal-title":"Ann Clin Lab Sci"},{"key":"R7-20250603","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1097\/00043426-200405000-00003","article-title":"Langerhans cell histiocytosis presenting as a painless bilateral swelling of the parotid glands","volume":"26","author":"Iqbal","year":"2004","journal-title":"J Pediatr Hematol Oncol"},{"key":"R8-20250603","doi-asserted-by":"crossref","first-page":"630","DOI":"10.1111\/j.1600-0714.2006.00436.x","article-title":"Incidental Langerhans cell histiocytosis of the parotid gland resembling marginal zone B-cell lymphoma: A case report","volume":"35","author":"Kojima","year":"2006","journal-title":"J Oral Pathol Med"},{"key":"R9-20250603","doi-asserted-by":"crossref","first-page":"970","DOI":"10.1016\/j.oraloncology.2007.12.009","article-title":"Nonlymphoid mesenchymal tumors of the parotid gland","volume":"44","author":"Takahama","year":"2008","journal-title":"Oral Oncol"},{"key":"R10-20250603","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1002\/pbc.22066","article-title":"A case of multisystem Langerhans cell histiocytosis with primary hypothyroidism followed by type 1 diabetes mellitus","volume":"53","author":"Shima","year":"2009","journal-title":"Pediatr Blood Cancer"},{"key":"R11-20250603","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1016\/j.mjafi.2012.09.004","article-title":"Histiocytosis","volume":"71","author":"Vashist","year":"2012","journal-title":"Med J Armed Forces India"},{"key":"R12-20250603","first-page":"546","article-title":"Adult Langerhans' cell histiocytosis: A rare cause of parotid gland enlargement","volume":"18","author":"Babacan","year":"2013","journal-title":"J BUON"},{"key":"R13-20250603","article-title":"Swelling of bilateral parotid glands: An unusual symptom of multisystem Langerhans cell histiocytosis","volume":"2","author":"Yuan","year":"2014","journal-title":"SAGE Open Med Case Rep"},{"key":"R14-20250603","first-page":"13647","article-title":"Isolated Langerhans cell histiocytosis of the sublingual gland in an adult","volume":"8","author":"Yang","year":"2015","journal-title":"Int J Clin Exp Pathol"},{"key":"R15-20250603","doi-asserted-by":"crossref","first-page":"520","DOI":"10.3342\/kjorl-hns.2018.00661","article-title":"A case of langerhans cell sarcoma presenting as submandibular gland mass","volume":"62","author":"Lee","year":"2019","journal-title":"Korean J Otorhinolaryngol-Head Neck Surg"},{"key":"R16-20250603","doi-asserted-by":"crossref","first-page":"e00603","DOI":"10.1002\/rcr2.603","article-title":"Multisystem Langerhans cell histiocytosis in an adult non-smoker treated with steroid therapy","volume":"8","author":"Ito","year":"2020","journal-title":"Respirol Case Rep"},{"key":"R17-20250603","doi-asserted-by":"crossref","first-page":"4966","DOI":"10.12998\/wjcc.v8.i20.4966","article-title":"Multisystem involvement Langerhans cell histiocytosis in an adult: A case report","volume":"8","author":"Wang","year":"2020","journal-title":"World J Clin Cases"},{"key":"R18-20250603","doi-asserted-by":"crossref","first-page":"1919","DOI":"10.1182\/blood-2010-04-279083","article-title":"Recurrent BRAF mutations in Langerhans cell histiocytosis","volume":"116","author":"Badalian-Very","year":"2010","journal-title":"Blood"},{"key":"R19-20250603","doi-asserted-by":"crossref","first-page":"e28","DOI":"10.1182\/blood-2012-06-429597","article-title":"BRAFV600E mutant protein is expressed in cells of variable maturation in Langerhans cell histiocytosis","volume":"120","author":"Sahm","year":"2012","journal-title":"Blood"},{"key":"R20-20250603","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1097\/PAS.0000000000000989","article-title":"CDKN2A\/B deletion and double-hit mutations of the MAPK pathway underlie the aggressive behavior of Langerhans cell tumors","volume":"42","author":"Xerri","year":"2018","journal-title":"Am J Surg Pathol"},{"key":"R21-20250603","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1186\/s13256-020-02460-3","article-title":"Adult Langerhans cell histiocytosis presenting with multisystem involvement and sarcomatoid features: A case report","volume":"14","author":"Aguirre","year":"2020","journal-title":"J Med Case Rep"},{"key":"R22-20250603","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1186\/1750-9378-9-15","article-title":"High viral loads of Merkel cell polyomavirus DNA sequences in Langerhans cell sarcoma tissues","volume":"9","author":"Kurakami","year":"2014","journal-title":"Infect Agents Cancer"},{"key":"R23-20250603","doi-asserted-by":"crossref","first-page":"577","DOI":"10.3346\/jkms.2006.21.3.577","article-title":"Langerhans cell sarcoma arising from Langerhans cell histiocytosis: A case report","volume":"21","author":"Lee","year":"2006","journal-title":"J Korean Med Sci"},{"key":"R24-20250603","doi-asserted-by":"crossref","first-page":"e14531","DOI":"10.1097\/MD.0000000000014531","article-title":"Langerhans cell sarcoma arising from antecedent langerhans cell histiocytosis: A case report","volume":"98","author":"Yi","year":"2019","journal-title":"Medicine (Baltimore)"}],"container-title":["Indian Journal of Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/ijc.IJC_819_20","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T13:19:00Z","timestamp":1787836740000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/ijc.IJC_819_20"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,1]]},"references-count":23,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2022]]}},"URL":"https:\/\/doi.org\/10.4103\/ijc.ijc_819_20","relation":{},"ISSN":["0019-509X","1998-4774"],"issn-type":[{"value":"0019-509X","type":"print"},{"value":"1998-4774","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,1]]},"assertion":[{"value":"2020-07-23","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-01-21","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}