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Skull base surgery. 1999; 9(1): 51–56.
PMCID: PMC1656715

Multifocal Langerhans' Cell Histiocytosis Involving Bilateral Temporal Bones, Lungs, and Hypothalamus in an Adult


Multifocal Langerhans' cell histocytosis is a rare condition in adults. We present the case of a 31-year-old female who initially presented with a clinical appearance of acute mastoiditis. The patient ultimately had involvement of bilateral temporal bones, lungs, and hypothalamus. Treatment with methotrexate, steroids, and desmopressin acetate (DDAVP) resulted in initial clinical improvement though not resolution. The patient also underwent radiation therapy to the hypothalamic lesion. The literature is reviewed, focusing on the diagnostic challenge of this disease process in adults. The additional morbidity of surgery, specifically mastoidectomy, in the setting of Langerhan's cell histiocytosis is discussed.

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  • Lichtenstein L, Jeffe HL. Eosinophilic granuloma of bone: With report of a case. Am J Pathol. 1940 Sep;16(5):595–604.3. [PubMed]
  • LICHTENSTEIN L. Histiocytosis X; integration of eosinophilic granuloma of bone, Letterer-Siwe disease, and Schüller-Christian disease as related manifestations of a single nosologic entity. AMA Arch Pathol. 1953 Jul;56(1):84–102. [PubMed]
  • Schuknecht HF. Histiocytosis X. Otolaryngol Head Neck Surg (1979) 1980 Sep-Oct;88(5):544–547. [PubMed]
  • Lieberman PH, Jones CR, Dargeon HW, Begg CF. A reappraisal of eosinophilic granuloma of bone, Hand-Schüller-Christian syndrome and Letterer-Siwe syndrome. Medicine (Baltimore) 1969 Sep;48(5):375–400. [PubMed]
  • Wintemute Garen J, Teret Stephen P, Kraus Jess F. Drs Wintemute, Teret and Kraus Respond. West J Med. 1987 Aug;147(2):b208–209. [PMC free article]
  • Stull MA, Kransdorf MJ, Devaney KO. Langerhans cell histiocytosis of bone. Radiographics. 1992 Jul;12(4):801–823. [PubMed]
  • Alessi DM, Maceri D. Histiocytosis X of the head and neck in a pediatric population. Arch Otolaryngol Head Neck Surg. 1992 Sep;118(9):945–948. [PubMed]
  • Cinberg JZ. Eosinophilic granuloma in the head and neck: a five year review with report of an instructive case. Laryngoscope. 1978 Aug;88(8 Pt 1):1281–1289. [PubMed]
  • Irving RM, Broadbent V, Jones NS. Langerhans' cell histiocytosis in childhood: management of head and neck manifestations. Laryngoscope. 1994 Jan;104(1 Pt 1):64–70. [PubMed]
  • Schloss MD, Klein A, Black MJ. Histiocytosis X of the head and neck. J Otolaryngol. 1981 Jun;10(3):189–194. [PubMed]
  • Tos M. A survey of Hand-Schüller-Christian's disease in otolaryngology. Acta Otolaryngol. 1966 Sep;62(3):217–228. [PubMed]
  • Angeli SI, Alcalde J, Hoffman HT, Smith RJ. Langerhans' cell histiocytosis of the head and neck in children. Ann Otol Rhinol Laryngol. 1995 Mar;104(3):173–180. [PubMed]
  • Anonsen CK, Donaldson SS. Langerhans' cell histiocytosis of the head and neck. Laryngoscope. 1987 May;97(5):537–542. [PubMed]
  • Appling D, Jenkins HA, Patton GA. Eosinophilic granuloma in the temporal bone and skull. Otolaryngol Head Neck Surg. 1983 Aug;91(4):358–365. [PubMed]
  • Cunningham MJ, Curtin HD, Jaffe R, Stool SE. Otologic manifestations of Langerhans' cell histiocytosis. Arch Otolaryngol Head Neck Surg. 1989 Jul;115(7):807–813. [PubMed]
  • DiNardo LJ, Wetmore RF. Head and neck manifestations of histiocytosis-X in children. Laryngoscope. 1989 Jul;99(7 Pt 1):721–724. [PubMed]
  • Esterly NB, Maurer HS, Gonzalez-Crussi F. Histiocytosis X: a seven-year experience at a children's hospital. J Am Acad Dermatol. 1985 Sep;13(3):481–496. [PubMed]
  • Greenberger JS, Crocker AC, Vawter G, Jaffe N, Cassady JR. Results of treatment of 127 patients with systemic histiocytosis. Medicine (Baltimore) 1981 Sep;60(5):311–338. [PubMed]
  • Hudson WR, Kenan PD. Otologic manifestations of histiocytosis X. Laryngoscope. 1969 Apr;79(4):678–693. [PubMed]
  • Martinez-Perez D, Mulliken JB, Arceci RJ. Langerhans cell histiocytosis: an uncommon disease commonly manifesting in the craniofacial skeleton. Plast Reconstr Surg. 1996 Aug;98(2):211–216. [PubMed]
  • McCaffrey TV, McDonald TJ. Histiocytosis X of the ear and temporal bone: review of 22 cases. Laryngoscope. 1979 Nov;89(11):1735–1742. [PubMed]
  • Sessa S, Sommelet D, Lascombes P, Prévot J. Treatment of Langerhans-cell histiocytosis in children. Experience at the Children's Hospital of Nancy. J Bone Joint Surg Am. 1994 Oct;76(10):1513–1525. [PubMed]
  • Smith RJ, Evans JN. Head and neck manifestations of histiocytosis-X. Laryngoscope. 1984 Mar;94(3):395–399. [PubMed]
  • Sweet RM, Kornblut AD, Hyams VJ. Eosinophilic granuloma in the temporal bone. Laryngoscope. 1979 Oct;89(10 Pt 1):1545–1552. [PubMed]
  • Toohill RJ, Kidder TM, Eby LG. Eosinophilic granuloma of the temporal bone. Laryngoscope. 1973 Jun;83(6):877–889. [PubMed]
  • Green I, Behar AJ, Shanon E, Gorsky M. Multifocal extraosseous eosinophilic granuloma of the head and neck. Arch Otolaryngol Head Neck Surg. 1988 May;114(5):561–563. [PubMed]
  • Kaufman A, Bukberg PR, Werlin S, Young IS. Multifocal eosinophilic granuloma ("Hand-Schuller-Christian disease"). Report illustrating H-S-C chronicity and diagnostic challenge. Am J Med. 1976 Apr;60(4):541–548. [PubMed]
  • Kepes JJ, Kepes M. Predominantly cerebral forms of histiocytosis-X. A reappraisal of "Gagel's hypothalamic granuloma", "granuloma infiltrans of the hypothalamus" and "Ayala's disease" with a report of four cases. Acta Neuropathol. 1969;14(2):77–98. [PubMed]
  • Kimmelman CP, Nielsen E, Snow JB., Jr Histiocytosis X of the temporal bone. Otolaryngol Head Neck Surg. 1984 Oct;92(5):588–590. [PubMed]
  • Moghadam BK, Saedi S, Gier RE. Adult-onset multifocal histiocytosis X presenting as a periodontal problem. J Oral Maxillofac Surg. 1991 Apr;49(4):417–419. [PubMed]
  • Pressman BD, Waldron RL, 2nd, Wood EH. Histiocytosis-X of the hypothalamus. Br J Radiol. 1975 Mar;48(567):176–178. [PubMed]
  • Quesada P, Navarrete ML, Perelló E. Eosinophilic granuloma of the temporal bone. Eur Arch Otorhinolaryngol. 1990;247(3):194–196. [PubMed]
  • Gramatovici R, D'Angio GJ. Radiation therapy in soft-tissue lesions in histiocytosis X (Langerhans' cell histiocytosis). Med Pediatr Oncol. 1988;16(4):259–262. [PubMed]
  • Lieberman PH, Jones CR, Steinman RM, Erlandson RA, Smith J, Gee T, Huvos A, Garin-Chesa P, Filippa DA, Urmacher C, et al. Langerhans cell (eosinophilic) granulomatosis. A clinicopathologic study encompassing 50 years. Am J Surg Pathol. 1996 May;20(5):519–552. [PubMed]
  • Mackenzie WG, Morton KS. Eosinophilic granuloma of bone. Can J Surg. 1988 Jul;31(4):264–267. [PubMed]
  • Zinkham WH. Multifocal eosinophilic granuloma. Natural history, etiology and management. Am J Med. 1976 Apr;60(4):457–463. [PubMed]
  • Arcand P, Caouette H, Dufour JJ. Eosinophilic granuloma of the temporal bone: simultaneous bilateral involvement. J Otolaryngol. 1985 Dec;14(6):375–378. [PubMed]
  • Bass RM. Eosiophilic granuloma in the head and neck. J Otolaryngol. 1980 Jun;9(3):250–255. [PubMed]
  • DeMarino DP, Dutcher PO, Jr, Parkins CW, Hengerer AS. Histiocytosis X: otologic presentations. Int J Pediatr Otorhinolaryngol. 1985 Oct;10(1):91–100. [PubMed]
  • Fradis M, Podoshin L, Ben-David J, Grishkan A. Eosinophilic granuloma of the temporal bone. J Laryngol Otol. 1985 May;99(5):475–479. [PubMed]
  • Jones RO, Pillsbury HC. Histiocytosis X of the head and neck. Laryngoscope. 1984 Aug;94(8):1031–1035. [PubMed]
  • Lopez-Rios G, Benitez JT. Histiocytosis: histopathological study of the temporal bone. Ann Otol Rhinol Laryngol. 1968 Dec;77(6):1171–1180. [PubMed]
  • Martin TH. Solitary eosinophilic granuloma of the temporal bone. A case report. Laryngoscope. 1969 Dec;79(12):2165–2171. [PubMed]
  • Mehta DN, Ramani GV, Chatterjee AK, Subramanyam CS. Eosinophilic granuloma of the temporal bone. J Laryngol Otol. 1974 Feb;88(2):185–191. [PubMed]
  • Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 21-1991. A 13-year-old boy with a destructive lesion of the left mastoid bone. N Engl J Med. 1991 May 23;324(21):1489–1495. [PubMed]
  • Straka JA, Caparosa RJ. Eosinophilic granuloma of the temporal bone. Laryngoscope. 1972 Jan;82(1):41–44. [PubMed]
  • Raney RB, Jr, D'Angio GJ. Langerhans' cell histiocytosis (histiocytosis X): experience at the Children's Hospital of Philadelphia, 1970-1984. Med Pediatr Oncol. 1989;17(1):20–28. [PubMed]
  • Wester SM, Beabout JW, Unni KK, Dahlin DC. Langerhans' cell granulomatosis (histiocytosis X) of bone in adults. Am J Surg Pathol. 1982 Jul;6(5):413–426. [PubMed]
  • Tos M. Facial palsy in Hand-Schüller-Christian's disease. Arch Otolaryngol. 1969 Nov;90(5):563–567. [PubMed]
  • Levy R, Sarfaty SM, Schindel J. Eosinophilic granuloma of the temporal bone. Two cases with bilateral involvement. Arch Otolaryngol. 1980 Mar;106(3):167–171. [PubMed]
  • Angeli SI, Luxford WM, Lo WW. Magnetic resonance imaging in the evaluation of Langerhans' cell histiocytosis of the temporal bone: case report. Otolaryngol Head Neck Surg. 1996 Jan;114(1):120–124. [PubMed]

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