Bone & joints
Other tumors
Langerhans cell histiocytosis

Topic Completed: 1 June 2005

Minor changes: 11 November 2019

Copyright: 2003-2019,, Inc.

PubMed search: langerhans cell histiocytosis [title] bone

Nat Pernick, M.D.
Page views in 2019: 12,415
Page views in 2020 to date: 9,353
Cite this page: Pernick N. Langerhans cell histiocytosis. website. Accessed October 29th, 2020.
Definition / general
  • Formerly called Histiocytosis X
  • Either solitary bone involvement, multiple bone involvement (variable skin involvement) or multiple organ involvement (bone, liver, spleen, other sites)
  • Ages 5 - 15 years, 60% male
  • Neoplastic, although cause unknown
  • Sites: skull, jaw, humerus, rib, femur; metaphysis or diaphysis
Radiology description
  • Lytic masses that may extend into soft tissue
Radiology images

Case of the Week #72

Involvement of both parietal bones with soft tissue extension #1 (plain film)

#2 (CT scan)

AFIP images

Eosinophilic granuloma

Contributed by Dr. Mark R. Wick

Skull xray

Case reports
Gross description
  • Sharply circumscribed
Microscopic (histologic) description
  • Infiltration by Langerhans cells (polygonal cells with eosinophilic cytoplasm, oval nuclei with longitudinal grooves resembling coffee beans)
  • Eosinophils, giant cells, neutrophils, foam cells, lymphocytes, plasma cells, fibrosis, necrosis; may have typical and atypical mitotic figures
Microscopic (histologic) images

AFIP images

Eosinophilic granuloma

Contributed by Dr. Mark R. Wick

Langerhans cell disease


Langerin stain


Images hosted on other servers:

(a) H&E;
(b) CD1a

Contributed by Dr. Saroona Haroon, The Aga Khan University Hospital (Pakistan)

Cytology description
  • Highly cellular with large, polygonal cells with ample cytoplasm, nuclei are round, oval or bean shaped with fine and even chromatin and prominent longitudinal grooves
  • Inconspicuous nucleoli, mild pleomorphism
  • No / minimal mitotic figures
  • Scattered eosinophils and neutrophils and multinucleated osteoclast-like giant cells
  • Necrosis common
Cytology images

Case of the Week #72

Pap stain of parietal bone

Contributed by Dr. Mark R. Wick

Touch preparation,
Diff-Quik stain

Images hosted on other servers:

B: Diff Quik; C: Pap (arrow at eosinophil); D: CD1a; E: H&E

Positive stains
Negative stains
Electron microscopy description
  • Birbeck granules (electron dense cross striations)
Electron microscopy images

Contributed by Dr. Mark R. Wick

Birbeck granules

Electron micrograph

Molecular / cytogenetics description
Additional references
Solitary bone involvement
Definition / general
  • Also called eosinophilic granuloma
  • Accounts for most cases
  • Usually young adults with localized pain
  • Lesions may spontaneously regress

  • Any bones but hands and feet; most common is cranial vault, jaw, humerus, rib, femur

Radiology description
  • Osteolytic lesion of metaphysis of long bones, with variable periosteal proliferation
  • May resemble metastatic carcinoma or Ewing / PNET
  • May extend into soft tissue if bone fracture
  • Skull lesions have area of lucency with "hole in hole" appearance due to different rates of destruction of two tables of bone in skull

Case reports
  • Excision but may recur; also small doses of radiation
  • Excellent prognosis
Multiple bone involvement
Definition / general
  • Also called multiple or polyostotic eosinophilic granuloma, Hand-Schüller-Christian disease
  • May cause proptosis, diabetes insipidus, chronic otitis media
  • Prolonged clinical course with regression and relapse
  • Usually favorable outcome
Multiple organ involvement
Definition / general
  • Lung and skin most commonly involved in addition to bone; also lymphadenopathy, diabetes insipidus

Prognostic factors
  • Poor prognosis if < 18 months old at diagnosis, hepatomegaly, anemia, bone marrow involvement, thrombocytopenia, hemorrhagic skin lesions
  • Histologic features not predictive of clinical course
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