{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T09:54:33Z","timestamp":1785405273059,"version":"3.56.0"},"reference-count":15,"publisher":"Department of Medicine, Warren Alpert Medical School at Brown University","issue":"3","license":[{"start":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T00:00:00Z","timestamp":1782864000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:p>Langerhans cell histiocytosis (LCH) is a rare hematologic neoplasm predominantly affecting children, with adult-onset cases being exceedingly uncommon. Neurological presentations, including spinal cord compression and cranial neuropathies, represent atypical manifestations that often lead to diagnostic delays. We present a 20-year-old previously healthy female who presented with progressive back pain, bilateral lower extremity weakness, and left-sided hearing loss. Imaging revealed a thoracic epidural mass causing severe spinal stenosis with additional diffuse osteolytic lesions. Initial biopsies showed nonspecific lymphoplasmacytic infiltrates. The patient subsequently developed cranial nerve palsies and dysphagia over several months. Definitive diagnosis required three biopsies, with the final petrous bone specimen demonstrating characteristic LCH histology and positive immunostaining for CD1a, S100, and Langerin. Treatment with zoledronic acid failed; subsequent therapy with cytarabine followed by cladribine plus hydroxyurea achieved partial radiographic and clinical improvement, though complicated by treatment-related sepsis. This case highlights the diagnostic and therapeutic challenges of adult LCH, given its rarity, variable presentation, and limited treatment guidelines. Multiple tissue samples with appropriate immunohistochemical staining may be required for diagnosis. Adult LCH treatment remains empirical, often extrapolated from pediatric data, with systemic therapies carrying significant toxicity risks. Greater awareness and adult-specific clinical trials are urgently needed.<\/jats:p>","DOI":"10.56305\/001c.162643","type":"journal-article","created":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T12:47:46Z","timestamp":1782910066000},"source":"Crossref","is-referenced-by-count":0,"title":["Enigmatic Case of Adult-Onset Langerhans Cell Histiocytosis with Aggressive Bony Involvement"],"prefix":"10.56305","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5944-9505","authenticated-orcid":true,"given":"Zhan","family":"Rong","sequence":"first","affiliation":[{"id":[{"id":"https:\/\/ror.org\/05wyq9e07","id-type":"ROR","asserted-by":"publisher"}],"name":"Stony Brook University Hospital","department":["Department of Internal Medicine"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan","family":"Abboud","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/05wyq9e07","id-type":"ROR","asserted-by":"publisher"}],"name":"Stony Brook University Hospital","department":["Department of Internal Medicine"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan","family":"Pendleton","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/05wyq9e07","id-type":"ROR","asserted-by":"publisher"}],"name":"Stony Brook University Hospital","department":["Department of Internal Medicine"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sashank","family":"Cherukuri","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/03mtd9a03","id-type":"ROR","asserted-by":"publisher"}],"name":"Stanford Medicine","department":["Department of Pathology"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"35509","published-online":{"date-parts":[[2026,7,1]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"108991","DOI":"10.1016\/j.clineuro.2025.108991","article-title":"Langerhans cell histiocytosis of the spine in adults: A rare and controversial disorder: A systematic review","volume":"255","author":"E. Abdulla","year":"2025","journal-title":"Clin Neurol Neurosurg"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"589","DOI":"10.2214\/AJR.07.3573","article-title":"Craniofacial and intracranial manifestations of langerhans cell histiocytosis: report of findings in 100 patients","volume":"191","author":"N. D'Ambrosio","year":"2008","journal-title":"AJR Am J Roentgenol"},{"issue":"1","key":"ref3","doi-asserted-by":"publisher","first-page":"28","DOI":"10.1097\/IOP.0000000000000402","article-title":"Langerhans Cell Histiocytosis of the Orbit: Spectrum of Disease and Risk of Central Nervous System Sequelae in Unifocal Cases","volume":"32","author":"N. Esmaili","year":"2016","journal-title":"Ophthalmic Plast Reconstr Surg"},{"issue":"11","key":"ref4","doi-asserted-by":"publisher","first-page":"1305","DOI":"10.6004\/jnccn.2021.7098","article-title":"Challenges in the Histopathologic Diagnosis of Histiocytic Neoplasms","volume":"19","author":"K. L. Rech","year":"2021","journal-title":"J Natl Compr Canc Netw"},{"issue":"12","key":"ref5","doi-asserted-by":"publisher","first-page":"1415","DOI":"10.1182\/blood-2015-03-635151","article-title":"Cladribine and cytarabine in refractory multisystem Langerhans cell histiocytosis: results of an international phase 2 study","volume":"126","author":"J. Donadieu","year":"2015","journal-title":"Blood"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"726","DOI":"10.1056\/NEJMoa1502309","article-title":"Vemurafenib in multiple nonmelanoma cancers with BRAF V600 mutations","volume":"373","author":"D. M. Hyman","year":"2015","journal-title":"N Engl J Med"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"856","DOI":"10.1056\/NEJMra1607548","article-title":"Langerhans-cell histiocytosis","volume":"379","author":"C. E. Allen","year":"2018","journal-title":"N Engl J Med"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"6","DOI":"10.5114\/pdia.2017.67095","article-title":"Langerhans cell histiocytosis in children - a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment","volume":"35","author":"M. Jezierska","year":"2018","journal-title":"Postepy Dermatol Alergol"},{"issue":"5","key":"ref9","doi-asserted-by":"publisher","first-page":"728","DOI":"10.1111\/bjh.18459","article-title":"Incidence, prevalence and survival in patients with Langerhans cell histiocytosis: A national registry study from England, 2013-2019","volume":"199","author":"H. Liu","year":"2022","journal-title":"Br J Haematol"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"2601","DOI":"10.1182\/blood.2021014343","article-title":"International expert consensus recommendations for the diagnosis and treatment of Langerhans cell histiocytosis in adults","volume":"139","author":"G. Goyal","year":"2022","journal-title":"Blood"},{"issue":"2","key":"ref11","doi-asserted-by":"publisher","first-page":"185","DOI":"10.1007\/s12185-020-02892-z","article-title":"Clinical features and outcomes of adult Langerhans cell histiocytosis: a single-center experience","volume":"112","author":"M. Kobayashi","year":"2020","journal-title":"Int J Hematol"},{"key":"ref12","doi-asserted-by":"publisher","first-page":"2054","DOI":"10.1016\/j.mayocp.2019.02.023","article-title":"The Mayo Clinic histiocytosis working group consensus statement for the diagnosis and evaluation of adult patients with histiocytic neoplasms: Erdheim-Chester disease, Langerhans cell histiocytosis, and Rosai-Dorfman disease","volume":"94","author":"G. Goyal","year":"2019","journal-title":"Mayo Clin Proc"},{"issue":"5","key":"ref13","doi-asserted-by":"publisher","first-page":"650","DOI":"10.5858\/arpa.2018-0349-RS","article-title":"Histiocytic Sarcoma","volume":"144","author":"Y. P. Hung","year":"2020","journal-title":"Arch Pathol Lab Med"},{"issue":"1","key":"ref14","doi-asserted-by":"publisher","first-page":"594","DOI":"10.1182\/hematology.2024000584","article-title":"IgG4-related disease for the hematologist","volume":"2024","author":"L. Y. C. Chen","year":"2024","journal-title":"Hematology Am Soc Hematol Educ Program"},{"issue":"1","key":"ref15","doi-asserted-by":"publisher","first-page":"e2016033","DOI":"10.4084\/MJHID.2016.033","article-title":"Bisphosphonates in Langerhans Cell Histiocytosis: An International Retrospective Case Series","volume":"8","author":"D. Chellapandian","year":"2016","journal-title":"Mediterr J Hematol Infect Dis"}],"container-title":["Journal of Brown Hospital Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/bhm.scholasticahq.com\/article\/162643-enigmatic-case-of-adult-onset-langerhans-cell-histiocytosis-with-aggressive-bony-involvement","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T12:55:01Z","timestamp":1782910501000},"score":1,"resource":{"primary":{"URL":"https:\/\/bhm.scholasticahq.com\/article\/162643-enigmatic-case-of-adult-onset-langerhans-cell-histiocytosis-with-aggressive-bony-involvement"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7,1]]},"references-count":15,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2026]]}},"URL":"https:\/\/doi.org\/10.56305\/001c.162643","relation":{},"ISSN":["2994-5593"],"issn-type":[{"value":"2994-5593","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7,1]]}}}